United States
Environmental Protection
Agency
EPA-600/8-83-002
March 1983
Research and Development
Short-Term Tests for
Environmentally Induced
Chronic Health Effects

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                               EPA-600/ 8-83-002
                               March 1983
       Short-Term Tests for
Environmentally Induced Chronic
           Health Effects
           Avril D. Woodhead
                 Editor
      Brookhaven National Laboratory
          Upton, New York 11973
            Michael D. Waters
             Technical Editor
     Health Effects Research Laboratory
 Research Triangle Park, North Carolina 27711
  Office of Research and Development
 U.S. Environmental Protection Agency
        Washington, D.C. 20460

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                         DISCLAIMER

Mention of trade names or commercial products does not constitute endorsement or
recommendation for use.

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                             FOREWORD

  The formation of the U.S. Environmental Protection Agency in 1970 ushered in
the first decade of environmental awareness as a total national phenomenon. It was a
decade punctuated by major Congressional mandates to restore the nation's waters,
to reduce air pollution, and to find a comprehensive approach to other environ-
mental problems—those associated with pesticide use, hazardous waste disposal and
toxic substances. It was a decade underscored by the demand for new technology and
better  science to answer  environmental questions  and to solve environmental
problems.
  As  the scientific and technical arm of the Agency, The Office of Research and
Development is responsible for advancing the state of knowledge about the environ-
ment  such that critical issues and questions can  be  addressed and answered
effectively, based on the application of state-of-the-art science and technology. In the
years  since 1970, The Office of Research and Development has produced manifold
increases in the data base from which environmental decisions are made and in the
sophistication of the understanding which has provided the basis for decisions.
  This volume represents our effort to take stock of scientific advances in the use of
short-term tests for environmentally induced  chronic health  effects since the in-
ception of the Agency and to gauge what progress has been made and what remains
to be accomplished. The essays in this volume present a range of perspectives on the
subject, from the vantage points of the scientific and technical disciplines which have
been carrying out relevant research. The points of view represented are varied and
sometimes conflicting. But scientific progress depends on just such diversity. The
authors at times have speculated about emerging problems and research needs. Such
attempts require  extrapolation  based  upon  informed  scientific judgment. The
outcome of that process must, in the final analysis, be recognized as opinion and not
fact.

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                          EDITOR'S NOTE

  This book, "Short-Term Tests for Environmentally Induced Chronic Health Ef-
fects," is a part of the Environmental Protection Agency, Office of Research and
Development's "Decade Document" Program. The main objective was to provide
an assessment of the potential of short-term bioassays as tools in predicting chronic
health effects resulting from exposure to environmental agents. It was also intended
to guide future developments in these test systems, particularly in areas relevant to
the needs of the EPA.
  With these guidelines in hand, I approached my distinguished authors for their
contribution. Their response was mixed. All were enthusiastic about the prospect of
writing for the book, but several pointed out that there were so few short-term tests
available in their fields that it would be difficult to write a full chapter. I assured them
that the value of the document would lie not only in highlighting the spectacular
development of short-term tests in some areas over the past ten years, but equally in
drawing attention to the many large gaps in our knowledge, and to areas where there
is a great need for the validation of short-term assays. The chapters are therefore
somewhat diverse in their content. In fields in which short-term tests  exist, the
authors have critically reviewed their status, they have discussed how the tests might
be more precisely defined and have pointed the way to future programs of research.
In areas in which there are  very few short-term tests, the contributors have provided
excellent evaluations of the problems that hamper development of such  tests; this
may encourage others to  investigate any possible biological screening  methods.
Each chapter was reviewed by 3-4 reviewers drawn from EPA staff and other experts.
I am grateful to the authors for their forebearance, and for their readiness to accede
to my suggestions for making deletions and for modifying their chapters to ensure
some continuity of format. In several cases they allowed me to take out sections that
were near and dear to their hearts.
  I want to pay special tribute to Michael Waters, the EPA Project Officer on this
volume, who has  cheerfully and wholeheartedly devoted a great deal  of time and
effort to its  production. He was instrumental in developing close and successful
working relationships between myself, the  EPA staff, the reviewers, and the authors.
  Richard Setlow, Chairman of the Biology Department at Brookhaven National
Laboratory, and Alexander Hollaender, A.U.I. Washington, have given  me much
sound  advice, active aid,  and  encouragement at all  stages during preparation.
I also owe particular thanks to the volume editor, Dr. Jessica Barren, who did a mas-
terful job guiding  the book through the later stages of preparation and production.
Finally, my especial thanks to  two members of the Biology Department, Virginia
Pond and Kathy Kissel, for their hard work and outstanding support. Virginia Pond
did a tremendous task of carefully and painstakingly editing the  manuscript and
preparing the index.  Kathy Kissel typed,  and retyped, the manuscript with speed,
accuracy and unfailing good humor.
                                                     A.D. Woodhead

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                                PREFACE

                              Roger  Cortesi
INTRODUCTION
  The purpose of this preface is to give the reader a general understanding of the
regulatory process as it pertains to health issues, and to discuss the role that health
data play in that process. I hope to make clear at which stage data from short-term
tests  are considered  and to  put into perspective the  benefits  as well  as the
shortcomings of their use.

WHAT HEALTH DATA CAN PROVIDE
  The purpose of health-effects data in the regulatory process is to determine the
levels of risk that are associated with exposure to different concentrations of a pollu-
tant. This requires a knowledge of the dose-response curve and the level and extent
of individual and population exposures. The outputs  of a risk assessment can be
manifold, but two have been shown to be generally useful, namely: (1) an estimate
of the increased probability of disease or death of an individual as a function of
increased exposure; (2) an estimate of the morbidity or mortality in a population for
any given exposure. Ideally, what would be provided  by health data is a dose-re-
sponse curve at levels of concern. For the Environmental Protection Agency (EPA),
dose-response curves are needed at low levels of exposure (indeed, by toxicologic
standards, very low levels) and for chronic as well as for acute exposures.
  This situation,  while simple and understandable  in theory, is very much more
complicated in practice. The derivation of a precise dose-reponse curve at low levels
requires a clear definition of the health endpoint, the ability to extrapolate the data
and the obtaining of sufficient data.

Health Endpoint  Uncertainty
  Uncertainties in the health endpoint arise from questions as to what sector of the
population is to be protected, and from uncertainties and variability in the  clinical
manifestation of the disease.
  Determination  of the part of the population at risk from exposure to a particular
pollutant is far from straightforward, as consideration must range from its effects
upon the germ cells to those upon the aged. Obviously, the critical response to a pol-
lutant can vary with the target population. Symptoms  of chronic disease can differ
markedly from person to person, making evaluation of the response far from precise.
Also, the assessment of the dose that an individual has received is difficult, since ex-
posure estimates in retrospective epidemiological studies are usually not much better
than an order of  magnitude.
The Author: Roger Cortesi is Acting Director of the Office of Health Research, United States Environmen-
tal Protection Agency, Washington, D.C.

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Extrapolation of Data


 ' For the EPA's purpose, extrapolation of data is virtually always needed in two
areas. The first is extrapolation from relatively high doses where direct cause-effect
relations can be  observed to the much lower doses typical of environmental
exposures. The second is from the test system or animal to man. This is, of course,
the well-known "mouse to man" problem. In short-term testing we  would like to
make even more extreme projections, from cell to man or from biochemical marker
to man. Extrapolation is an inaccurate process, even when the kinetics of the rela-
tionship are known. When the functional form of the relationship is unknown, as in
much of the dose-response data, the estimation  of the dose-response curve at
environmental levels in humans from data obtained at higher doses or in nonhuman
systems is an educated guess at best.

Generation of Data


   It is very difficult in many cases to obtain data even if one is willing to accept the
 obstacles of endpoint definition and extrapolations. The problem arises even for the
 effects of acute  exposures and is worse for chronic exposures. Short-term high lev-
 els of air pollution can be clinically tested on healthy subjects but not on the very
 young nor on  the elderly or infirm, where  the most serious effects are to  be
 anticipated. Clinical testing for chronic exposures is not permissible, and therefore
 data for the dose-reponse curve must be extracted from epidemiological studies or
 from nonhuman test systems.
   Epidemiology has successfully highlighted causal relationships between particular
 enironmental pollutants and human cancer, but when it comes to estimating dose-
 response relationships it has serious and fundamental drawbacks. The major diffi-
 culty is that the method can rarely detect reliably a 50 percent increase in risk even in
 a large population because the intrinsic biases of epidemiological studies, in them-
 selves, are about this value. Yet a relative risk of this level or smaller is important
 when the effect is common, such as cardiovascular disease or kidney failure.
   The problems of epidemiology, particularly with respect to chronic effects, are
 many. They include the insidious nature of the diseases; the determination of expo-
 sure levels; the  control of confounding variables; and the large number of people
 needed to detect a minor effect. These problems and others are treated extensively in
 the literature, so they need not be expanded upon here. They make the derivation of
 a dose-response relationship possible only in a general and imprecise sense at best. In
 most cases, it is extremely fortunate if a robust association  can be made between a
 pollutant and a chronic disease.
   With epidemiology of limited use and clinical studies for chronic effects not allow-
 able, we have to try to get the most we can from derived  data from nonhuman test
 systems. Besides the problems of extrapolation to humans and from high to low ex-
 posure, which I have discussed above, there are a number of other factors making it
 difficult to obtain a dose-response curve. For example, the appropriate animal
 models may not be available and, when they are, then  a  lifetime exposure is often
 needed. In many cases, the exposure regime is not easy to define; for instance, should
 it be a series of spiked exposures or a continuous low-level  one? Finally, often it is
 impossible to reproduce the ambient conditions of the pollutant.
   First, lifetime tests on animals are time-consuming and costly procedures. More-
 over, the cumbersomeness and expense of such tests militate against their use when
 widespread testing is desirable, such as trying to find, by use of effects testing, the op-
 timal operating conditions of an engine so that the exhaust is least toxic. Lifetime
 assays on animals do not readily lend themselves to routine testing because quality
 assurance is difficult over a two- or three-year period  or more.
                                     VI

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DISCUSSION
  The purpose of what has been said above is not to make the task of health research
for standard-setting* appear hopeless. The purpose is to point out that the concept of
a "scientific" assessment of the health risks imposed by exposure to pollutants at am-
bient levels is generally not possible because we cannot produce dose-response data.
This being the case, one might think that trying to improve the situation by turning
to short-term tests is misdirection.  We are hopeful, however, that short-term tests
will be able to produce data that are quantitatively useful in the regulatory process.
We believe that  short-term tests must be pursued for several reasons.
  Further, in many regulatory situations, if health data are to be used at all, they
must be available quickly. These objections are overcome by the use of short-term
assays.
  We think that in the process of developing  and validating short-term tests,
mechanisms of toxicity will become much better defined, thereby greatly improving
the precision of the dose-response curves and hence the risk assessments. Similarly,
short-term tests  may be developed that can be used in large-scale surveys of human
populations, such as rapid and accurate measures of enzyme activity or of the mor-
phology and motility of spermatozoa, thereby again making epidemiology a more
powerful and useful source  of data.

THE REGULATORY PROCESS
   I have discussed above why the scientific component for setting exposure stan-
dards is uncertain. In this part, I will describe the regulatory process itself and try to
extract some key factors in deciding upon health-based exposure levels.
  It is important to understand that the standard-setting process is profoundly polit-
ical (in the nonpejorative sense). The question of where to set a limit cannot be con-
sidered separately from such issues as what it costs, who benefits, and who pays.
(The fact that it  cannot be considered apart from these factors does not, of course,
mean that one can always accurately define such things.) This consideration implies,
among other things, that a health standard can be set at two different levels in two
different places  without either standard being "wrong." Not  only is the standard-
setting process not entirely  a scientific one, it is also not entirely an economic one
either, in that one cannot balance cost versus benefit in any rigorous way. This is, of
course, to be expected from the imprecision of the health data, but it is driven further
in this direction by the imprecision of the economic data as well. We also have to take
into consideration major differences in philosophy, as to what is "right" and "rea-
sonable," and what will be the consequences of failure to pursue a version of right
and  reasonable.
  I will now describe a way of looking at the standard setting and regulatory process
that I believe to  be instructive even though many will profoundly disagree. I believe
there is enough  truth in  what I describe to provide insight into why the process is
indeed political.
  Obviously, it is very difficult for regulations to be set less stringently than the regu-
latees are essentially willing to accept. This means there is a tendency for regulations
to be as stringent as possible considering the political pressure that increasing strin-
gency generates. That  the process should proceed in this way can be repugnant to
many with a strong belief in order and method, be that belief scientific, economic, or
philosophic. The situation is not, however, as purely political as it appears. Scientif-
ic, economic, and philosophic considerations are  certainly taken into account, but
such data are not used  mechanically to regulate. If, for  instance, a  substance is
"Standard is not used in its physical or chemical sense, that is, as an acknowledged measure for quantitative
 or qualitative value. The standard may be set at different levels, as I will describe further in this section.
                                      Vll

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known to be very harmful and to cause especially damaging effects, regulations can
be very stringent before political pressure stops the process. Conversely, if the threat
is accompanied by widely perceived benefits, the political pressure will become un-
bearable with muchless stringency. A clear example of this is the loss of life weallow
from such things as automobiles, cigarettes, and alcohol. Alternatively, a very strin-
gent standard that adds only a small percentage to the unit cost may be tolerable,
even though the overall added cost greatly exceeds any perceived benefits. While
such a procedure does, in some sense, lack intellectual tidiness, it effectively copes
with the competing interests of the most seriously concerned parties.
  One more important inference can be made from the regulatory process described
above. Namely, some kind of assessment of risk is always  made,  whether it is
admitted or not. This assessment must be done consciously or unconsciously, openly
or covertly, individually or collectively, in deciding just how tight a standard to set.
  This procedure, as you  might expect, does not produce consistent results in such
narrow terms as incremental lifetime risk, or in terms of dollars per life saved. The
level of a pollutant that will be tolerated is very much influenced by what can be done
about it. If the benefits from the production  of a pollutant all lie in the future, strict
standards are  more likely to be set. When the benefits are large and immediate, less
stringent standards are likely. The range of increased lifetime risk to  an individual
from various standard-setting procedures has extended from more than one part in a
hundred down to less than one part in a million.
   Setting exposure limits  is a legal process and not a scientific one. Science can only
specify what the limit should reflect,  and only then to the extent that a consensus ex-
ists in the scientific community. When there is no agreement in the interpretation of
the scientific data, then the regulatory agency  must decide what to do in the face of
this scientific uncertainty. In order to do this in an acceptable way, the decision has
to be made according to precepts of fairness embodied in the law. These precepts bas-
ically define what information must be considered, and the steps to be taken  in doing
so. This means, in essence, that every submission impacting upon the standard-set-
ting process is made public, so that people who disagree are free to attempt to dis-
credit them. Submissions  such as scientific papers or data which have major  implica-
tions for setting a limit are subjected to  a great deal of scrutiny, usually under the
direction of lawyers. If a paper has implications that could cost a lot, it is going to be
reviewed line  by line.
   The significance of all this for short-term tests is as follows:   short-term tests need
a high probability of identifying a potentially harmful effect (that is, a high detection
probability or, equivalently, a low fraction of false negatives) and a low probability
of falsely detecting a harmful effect (that is, a low false-alarm probability or, equiva-
lently, a low fraction of false positives).* Finally, they should yield results that can be
expressed quantitatively,  giving a dose-response curve that is  applicable to humans.
The certainty  with which  one must be able to assert that a test meets these three cri-
teria is not clear at present. Recent vigorous challenges in court of assertions of the
carcinogenicity of a substance  based upon cancers or tumors induced in laboratory
animals indicate that short-term tests will have to meet the above conditions at least
as well  as currently used  long-term  animal assays.

PROBLEMS ASSOCIATED WITH DATA RELEASE
   Situations occur where the specter of health damage to people and  their  children
arises; Love Canal and Three M ile Island are just two examples. When this happens,
there is a great desire to know, virtually day by day, the results of any tests that would
*lt is worth observing in passing that high detection and low false-alarm probabilities are not independently
 achieved. A high detection probability can be achieved at the cost of a high false-alarm probability and vice
 versa. For example, a test which calls all substances toxicants will have a detection probability of one, but
 also have unacceplably high false-alarm probability.

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be useful for taking vigorous or drastic remedial actions. In fact, if there are relevant
tests in the processes of development, there will be very strong pressure to try the
tests before they have been properly validated. In such a situation, there are several
specific problems.  First, there is pressure, often successful, to release the results of
tests before they  have  met adequate  peer review. Second, the  caveats accom-
panying the results are often ignored. And third, there is a marked propensity for
fallacious reasoning—a post hoc ergo propter hoc—thinking by both  public and
press, resulting in  conclusions  being reached about cause and effect which may be
completely wrong.
  Such an atmosphere is clearly not one which fosters objective and disinterested as-
sessment. In such  an atmosphere, statements about what the results "mean" will
have to be made sooner and with less review and discussion than is normal for the
scientific process. Any delay in announcing results causes great problems in terms of
public demand that something be done in that delay itself is often perceived as bu-
reaucratic "cover-up."
  The successful development and application of short-term tests will certainly face
this dilemma. I believe that the conflict is an intrinsic one and hence can only be mol-
lified, but not resolved. The question of how to mollify this conflict is not one we
treat here.

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                             CONTENTS

                                                                    Page

Foreword 	   iii
Editor's Note  	   iv
Preface 	   v
Figures 	  xii
Tables  	  xvi
Quantitative Assessment of Risks, Richard B. Setlow 	   1
Short-Term Tests for Genetic Toxicity, Herman E.  Brockman
  and Frederick J.  de Serres 	   11
Short-Term In Vitro Tests for Identifying Carcinogens:
  Transformation of Mammalian Cells in Culture, Arthur C. Upton  	   38
Short-Term Tests for Identifying Teratogens, Thomas H. Shepard  	   69
Short-Term Tests for Renal Toxicity, David S. Miller and Anthony
  M. Guarino 	   89
Early Warning Systems for Behavioral Toxicology, Bernard  Weiss  	  108
The  Role of the Environment in Chronic Cardiovascular Disease,
  C. Richard Minick and Carl  G. Becker  	  136
Frontiers in  Short-Term Testing of Pneumotoxicants, D.B. Menzel,
  E.D. Smolko, D.E. Gardner, and J.A. Graham  	  175
Index  	  211
                                   XI

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                               FIGURES


Number                                                               Page

Setlow

  1  -  Linear (L), linear quadratic (LQ), and quadratic (Q) dose-response
        curves for mortality from leukemia or bone cancer induced by
        gamma radiation  	  3
  2  -  Two ethylation products of deoxyguanosine in DNA  	  6
  3  -  Two possible relations between the dose of an environmental
        agent and the relative level of specific DNA adducts formed in
        cells   	  6
  4  -  Hypothetical dose-response curves relating a particular biological
        effect with concentration of specific DNA adducts  	  8

Brockman and de Serres

  1  -  The long latent period of cancer illustrated by the association
        between cigarette smoking and  lung cancer  	  17
  2  -  DNA damage test using the bacterium Escherichia coli  	  24
  3  -  The mouse spot test for detection of a forward mutation at
        one of the heterozygous loci determining coat color  	  25
  4  -  The detection of chromosomal  aberrations by the formation of
        micronuclei  	  26
  5  -  Sister chromatid exchange (SCE) test in human lymphocytes
        in culture  	  27
  6  -  The use pf the higher plant Tradescantia as an in situ
        environmental monitor  	  33

Upton

  \  -  Neoplastic transformation of mouse cells in culture  	  40
  2  -  Outline of procedure  of standard colony assay for in vitro
        transformation of Golden Syrian hamster embryo cells  	  42
  3  -  Schematic illustration of the microscopic appearance of
        transformed fibroblasts as compared with normal fibroblasts  	  43
  4  -  Outline of procedure  of mass culture assay for in vitro
        transformation of Rauscher leukemia virus-infected Fischer
        rat embryo cells  	•	  47
  5  -  Variation in the probability that a compound will be correctly
        identified as a carcinogen (true  positive result) by the combined use
        of the  in vitro transformation test and the Salmonella mutagenicity
        test, in relation to the percentage of carcinogens among the
        samples being tested  	  51

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 Number                                                             Page

  6  -   Variation in the probability of true positive and false positive
         results provided by the combined use of discordant results, from
         the in vitro transformation test and the Salmonella mutagenicity
         test, in relation to the percentage of carcinogens among the
         samples being tested  	 52


Shepard

  1  -   Curve illustrating the susceptibility to teratogenesis from
         fertilization throughout intrauterine development  	 72
  2  -   Diagram of the factors that influence embryonic dose of a
         foreign chemical present in the maternal blood stream  	 72
  3  -   Diagram showing that each drug tested has a dose spectrum
         ranging from no effect to one of maternal lethality  	 73
  4  -   Diagram giving an example of an  in vitro test of a teratogen,
         using a growing rat embryo  	 76
  5  -   Diagram showing the relationship between cause, mechanism
         and manifestation of a teratogenic agent   	 77
  6  -   Perspective of our major defenses  against  teratogenic agents 	 79
  7  -   Diagram of a scheme whereby three data  bases (individual,
         agents and clinical syndromes) might  be used to determine the
         mechanisms which cause congenital defects and their
         prevention  	 82
  8  -   Events between the introduction of a  new agent causing
         widespread disease and the ending of the epidemic  	 83

Miller and Guarino

  1  -   Diagrammatic representation of the relationships between  the
         component parts of the nephron and  associated blood
         vessels  	 91
  2  -   Simplified diagram of the glomerular filtration apparatus  	 92
  3  -   Simplified summary of the movement of water and solutes in
         the different regions of a model  nephron  	 93
  4  -   Concentration of total solutes in tubular fluid as a function of
         segment of the nephron   	 95

 Weiss

  \  -   Procedure for plotting visual fields in monkeys   	  113
  2  -   The response of a patient with Minamata  Disease  and a
         normal control  	  115
  3      Mice being tested  on a rotarod  	  116
  4      A system designed  to measure fine motor  control  	  117
  5      Conduction velocities, for two worker groups, of the slower
         motor fibers (CVSF) of the ulnar  nerve 	  119
  6      Scheme for testing copulatory performance in male rats  	  120

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Number                                                               Pa8e

  7  -   System for evaluating the effect of microwave exposure on
         thermo-regulatory behavior  .................................  '"
  8  -   Irritant potency of airborne substances is measured
         behaviorally with a response that terminates irritant flow  .......  124

Minick and Becker

  1  -   Blood vessels in the muscularis of the gallbladder of a dog
         injected intravenously with ellagic acid  .......................  147
  2      Section of lung treated in a similar fashion to that
         illustrated in Fig. 1   ........................................  147
  3      Atherosclerosis of the right coronary artery of a 35-year-old
         man with marked coronary artherosclerosis, old and recent
         myocardial infarcts, and aortic and mitral stenosis  .............  149
  4  -   Atherosclerosis of the right coronary artery of rabbit that
         received semisynthetic lipid-rich diet and seven  injections of
         horse serum over a period of nine months  ....................  149
  5  -   Atherosclerosis of the left  coronary artery of a  72-year-old
         man who died with disseminated rheumatoid disease  ...........  150
  6      Atherosclerosis of  mesenteric artery of rabbit that received
         semisynthetic, lipid-rich diet and six intravenous injections of
         bovine serum albumin  over a period  of one year  ...............  150
  7      Lumen of rabbit mesenteric artery is narrowed  by musculoelastic
         intimal thickening  (bar) resembling diffuse intimal thickening
         in man  [[[  151
  8      Atherosclerosis of  rabbit artery representative of that induced
         in rabbits repeatedly injected with foreign serum protein and
         subsequently fed a cholesterol-supplemented diet 40 to 80 days
         after last injection   .........................................  151
  9      Cardiac allograft and recipient's  heart and aorta from rabbit
         fed a cholesterol-supplemented diet and treated with immuno-
         suppressives ...............................................  152
 10      Proximal left main coronary artery of cardiac allograft  that
         functioned for approximately two weeks  ......................  152
 1 1      Atheromatous change in gastric artery of normocholesterolemic
         chicken infected  with Marek's disease herpesvirus and fed a diet
         low in cholesterol  ..........................................  153
 12      Higher magnification of artery shown in Fig. 11  ...............  153
 13  -   Cross-section of  heart of a chicken infected with Marek's
         disease herpesvirus and fed cholesterol-supplemented diet  for
         1 5  weeks  .................................................  154
 14      Major coronary artery  of chicken infected with Marek's disease
         herpesvirus and fed a cholesterol-supplemented diet for
         1 5  weeks  .................................................  154
 15      Photomicrograph of endothelial  surface of rabbit that received
         one injection  of horse serum and was  sacrificed 15 days  later ....  [55
 16      Luminal surface  of large coronary artery of transplanted

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Number                                                             Page

Menzel, Smolko, Gardner, and Graham

  1     Three major regions of the respiratory tract  	  178
  2  -  Some common lung diseases in man   	  180
  3  -  Spirometric measurements of lung volumes  	  185
  4  -  Examples of forced expiratory volume (FEV), forced vital
        capacity (FVC), and maximal midexpiratory flow rates (MMFR)
        in normal individuals and patients with chronic obstructive and
        restrictive lung diseases during forced expiration into a
        spirometer  	  188
  5  -  Flow-volume curves illustrating the different patterns existing
        in patients with obstructive and restrictive diseases 	  188
  6  -  Comparison of normal lung volumes to those observed in
        restrictive and obstructive disease states   	  189

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                                TABLES

Number                                                               Pa8e

Brockman and de Serres

  1  -  An outline of twenty-four short-term tests for genetic toxicity
        selected for systematic review and evaluation in Phase 1 of the
        EPA's GENE-TOX Program  	  20
  2  -  Possible use of tests from Table 1 for hazard identification,
        hazard evaluation and risk estimation  	  29

Upton

  1  -  Some properties of transformed cells 	  41
  2  -  Comparative effects of different classes of chemicals with
        respect to transformation of hamster embryo cells in vitro,
        mutagenicity in Salmonella, and carcinogenicity in vivo 	  44
  3  -  Results with the in vitro transformation assay (colony formation
        in soft agar), as compared with five  other short-term tests for
        carcinogenicity  	  45
  4  -  Cell culture systems investigated for assaying the  carcinogenic
        potential of chemicals  	  48
  5  -  Biological properties  of promoting agents, as compared with
        initiating agents  	  49
  6  -  Comparative results of short-term tests when used alone or in
        combinations   	  50
  7  -  Comparative reliability of results from in vitro transformation
        test and Salmonella mutation test, used alone or  in  combination,
        on various classes of  chemicals  	  53

Miller and Guarino

  1  -  Nephrotoxic metallic  compounds  	  98
  2  -  Nephrotoxic organic  compounds  	  98
  3  -  Portions of the nephron that can be studied by the  use of available
        in vitro procedures 	  106

Weiss

  1  -  Adverse neurobehavioral responses  ascribed to metals  	  109
  2  -  Developmental progress  in mice 	   125
  3  -  Comparison of children with high and low tooth  lead
        concentrations  on a teacher rating scale  	   J2g
  4     Intelligence test scores of children with high and low tooth lead
        concentrations   	

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Number                                                           Page

Menzel, Smolko, Gardner, and Graham

  1   -   Inflammatory and immune related protein constituents of normal
        human alveolar epithelial fluid  	  194
  2   -   Indicators of injury in lung lavage fluids 	  195

  3   -   Properties and functions of normal human alveolar
        macrophages  	  196
                                   xvn

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        QUANTITATIVE ASSESSMENT OF RISKS*

                          Richard B.  Setlow


INTRODUCTION
  Assessment of risks involves an estimation of the extent of the biological effects
that result from exposure to particular levels of environmental agents. I use the word
"effects" in a general sense and, even though most of the examples cited are in the
field of carcinogenesis,  the description should be applicable to most biological
endpoints.  The difficulties  in  making such estimates led  one National Research
Council Committee (National Research Council, 1980a) to recommend that the "Of-
fice of Pesticide Programs of the Environmental Protection Agency (EPA) should
abandon its attempts to produce numerical estimates  of the effects of the use of pes-
ticides on human mortality and morbidity except when reliable human epidemiolog-
ical data are  available." But Harris (1981) states:
    A robust epidemiological study of the effect of an environmental agent on
    the risk of human cancer should have the following characteristics: Well-
    defined  groups  of exposed subjects and comparable control subjects
    should be identified. The magnitude and duration of individual exposures
    should be  measured. Possible significant confounding factors should be
    evaluated.  Potential biases caused by the nonrandom selection or follow-
    up of subjects should be avoided. The duration of the follow-up should be
    sufficient to observe a significant increase in the incidence of the suspected
    cancer. The number of persons or person-years at risk should be sufficient
    to detect a statistically significant difference  in cancer rates between ex-
    posed and control subjects.
  Clearly, few studies can satisfy these criteria.
  At low doses of environmental agents, the  effects of concern are either small ones,
such as minor headaches, that  affect large numbers of people or large ones, such as
cancer, that affect small numbers of people. The degree of risk is estimated by ex-
trapolation from  animal tests  or from accidental exposure of individuals to high
doses. But before we can distinguish the extent of the risk,  we need to be able to
measure the background level of the effect in the human population in the absence of
any known exposure. The fact  that background levels vary from one population to
another is taken as an indication of the role of the environment in ill health. Never-
theless, there are few detailed explanations for the  magnitude of background levels.
However, if one could show (which one cannot at present) that the cause of the back-
ground effect was the same  as from an environmental agent, one could safely infer
that no threshold dose existed.  The dose-response curve would have a finite slope at
low doses because any presumptive threshold dose will have been exceeded by the
cause of the background effect (Hoel, 1980). If the background is high, a relatively
large increased incidence is  needed to be detectable, implying that individuals must
•Research carried out under the auspices of the United States Department of Energy.

The Author: Richard B. Setlow is Chairman of the Department of Biology at Brookhaven National Labora-
tory, Upton, Long Island, New York 11973.

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have been exposed to relatively high doses. On the other hand, if the background
level is low, relatively small changes in incidence are readily observable and hence
may be associated easily with the responsible environmental agent(s).  Mesothe-
liomas of the lung arising from exposure to asbestos and adenocarcinomas of the va-
gina in young women  resulting from exposure  in utero to diethylstilbestrol are
examples of such cause-and-effect association (Herbst et al., 1977). Of course, a pre-
cise estimate of risk from such epidemiologic studies requires an adequate knowl-
edge of the exposure levels.
  The assessment should in principle use human data but fortunately not many such
data exist.  When they do, uncertainties in exposure levels  and in the number of
people affected mask the shape of the dose-response curve at low doses. A typical
example is the effects arising from exposure to ionizing radiation. The lifetime risk
to males of mortality from leukemia or bone cancer is approximately 100 per 104
individuals (Fig. 1). The'acute radiation dose that would double this risk is between
150 and 200 rads (National Research Council, 1980b). Judging from animal experi-
ments, the doubling dose would be appreciably higher at low dose rates. Hence, the
background frequency of mortality from leukemia cannot be ascribed to the back-
ground of ionizing radiation—natural plus manmade—of approximately 0.1 rad per
year. The problem in extrapolating dose-response data from high doses to low doses
is illustrated by the curves shown in Fig. 1. Human data obtained at relatively high
doses (50 to 300 rads), as a result of the atomic bombs dropped on Japan, have been
analyzed extensively and three possible dose-response models have been described
for such data.* These models, discussed in a National Research Council report (Na-
tional Research Council, 1980b), are called the linear (L), linear quadratic (LQ) and
quadratic (Q) models. The curves drawn in Fig. 1 are based on the equations derived
from these data. Because of the high frequency of background mortality, any sample
of 10,000 individuals, unirradiated or irradiated with a low dose, will show a large
statistical uncertainty about the average mortality figure of approximately 100. The
confidence interval shown is twice the standard deviation, the latter being the square
root of the average number of events.  Obviously, data obtained from the exposure
of 104 males to 30 rads cannot be used to distinguish among the three  models. Pop-
ulations appreciably greater than 104 would have to be used. Clearly, as the dose goes
down,  the population examined must  increase. Thus, the shape of a dose-response
curve cannot  be determined at low  doses unless one  uses tremendous numbers of
humans or experimental animals. Even if one could do such an experiment on peo-
ple, the uncertainty in the doses and  the possibility that the responses of individuals
may vary greatly make such experiments more or less useless for risk assessment for
the average person. Therefore, the emphasis in this volume is on short-term tests.

RISK ASSESSMENT FROM SHORT-TERM TESTS
  The  problem is how to extrapolate quantitatively from short-term tests to assess-
ments of risk to humans. An intermediate step in this process is to perform chronic
experiments with animals. Such experiments also  have their shortcomings because
they use limited numbers of animals and, to obtain observable results, they require
high exposures. Thus, the interpreter of such experiments is also faced with the prob-
lem of extrapolating to low exposure levels. A knowledge of the dose-response
curve is needed, but, as we saw in Fig. 1, the correct shape of the curve at low doses
cannot be derived from data at high doses. Hence, the extrapolation  is made using
mathematical models of one type or another that are felt to give reasonable upper
limits to the risk and therefore to give safe environmental standards. It is not our
aim to examine these many mathematical possibilities. They are discussed well and
extensively elsewhere (Cornfield etal., 1980; Mantel, 1980;Peto, 1977; Preussmann,
1980).
•The accuracy of the dosimetry is in question (Marshall, 1981).

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    250
 c
 
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  Extrapolations  from short-term  tests to human risks involve three major un-
resolved difficulties: (1) the projection from acute, short-term to chronic, long-term
exposures;  (2) the complicated interconnected kinetics of metabolic activation, de-
activation and repair; and (3) the fact that a number of important biological end-
points require multiple environmental insults. (For example, the effects of tumor
promoters may be all important at low dose levels.) We take up these difficulties be-
low.

Acute Versus Chronic Exposure
  It is possible to determine dose-response relation for acute exposures as the many
short-term  tests described in this volume illustrate. How  should one extrapolate
such data to chronic exposures? There is no single answer to this question without a
knowledge  of the pharmacokinetics of the various processes involved (Anderson et
al.,  1980). Unfortunately,  in many instances the details of the  processes are not
known and therefore an elucidation of the kinetics may not  be possible. It is known,
however, that chronic  exposure  to a  number of  chemicals, not necessarily
carcinogens, can change the level of enzyme systems that convert procarcinogens to
ultimate carcinogens and degrade the ultimate carcinogens to unreactive, excretable
compounds. Moreover, low chronic exposure of animals to alkylating agents renders
them better able to remove DNA damage from a large challenge dose (Montesanoet
al.,  1979). Thus, how one  should extrapolate from acute to  chronic exposures  or
from high to low rates of exposure depends upon the rates of various reactions, the
lifetimes of reaction products,  and  the biological effects of such products.
Metabolic Pathways
  The  analysis of the biological effects  of environmental agents is  complicated
because many agents classified as hazardous  do not react  directly with cell
macromolecules such as protein, RNA and DNA or with membranes. They often
must be metabolically activated to form reactive intermediates. Such intermediates
may affect  crucial structures in cells or, before they do so, may be inactivated  to
innocuous compounds. Hence, the ability of an agent to damage a particular tissue
depends critically  on the activation and deactivation pathways in the organism. An
additional complication, hidden in  the above analysis, is the implication that one
knows which are the important activated compounds, and which are the important
reaction products in macromolecules. In this context, the shape of the dose-response
curve depends not on the concentration of the ingested agent but on the internal level
of reactive compounds. For example, Bailey et al. (1981) have reported that analysis
of the in vivo production of methylcysteine in rat hemoglobin shows a linear dose-re-
sponse curve after injection of various concentrations of methyl methanesulfonate;
on  the other hand, after injection  of dimethylnitrosamine a curvilinear dose-re-
sponse curve was obtained. In this system, a high background level of methylcysteine
was observed.
  Not only is it essential to know which products are the deleterious ones in order to
measure their kinetics of appearance and disappearance, but one must have accurate
knowledge  of the relative rates of removal of the  macromolecular adducts, not just
their rates of formation. The rate of repair or removal and the chemical instability of
particular adducts determine their half-life, and this value,  when measured against
the  rates of  replication,  transcription,  and translation of genetic material, will
determine the extent of the harmful biological effects. The  half-lives of some prod-
ucts also depend on the tissue  in which the product resides.
  One of the better examples to illustrate these repair considerations involves the
products resulting from treatment of cells with alkylating agents (Singer, 1979). Sup-
pose that the only important products are those that affect DNA. Even at this level of
investigation, there are many products formed. One of the more numerous reactions
is the one with guanine. But there are a number of different guanine adducts. For ex-
ample, 7-alkylguanine is the most plentiful and 06-alkylguanine is formed in a much

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smaller amount (Fig. 2). However, the latter product is felt to be the more important
biologically, and there are reasonable indications that it is the one that is associated
with mutagenesis and carcinogenesis. In any event, 06-alklguanine is removed more
rapidly from many tissues than is 7-alkylguanine (Rajewsky et al., 1977). Such tis-
sues are less likely to be the targets for carcinogenesis. For example, 06-ethylguanine
has a very long half-life in the neural tissue but a very short half-life in the liver of
newborn rats,  and the rats develop neural, not liver,  tumors.
  A complication in this simple description is that the cellular machinery that re-
moves 06-alkylguanine may become saturated by high levels of the product(Medcalf
and Lawley, 1981; Pegg and Hui, 1978). As a result, the level of DNA adducts at high
doses and over short periods of time would be expected to increase faster than a
linear power of the dose. (See Fig. 3.) If a low concentration (Fig. 3B) is adminis-
tered for a long time, one would expect that particular repair systems might be able
to keep up with the production of DNA adducts and hence their level would be less
than those observed after treating cells at high concentrations as illustrated in Fig.
3 A. Moreover, there are  good indications in  prokaryotic systems, and some in
eukaryotic  ones, that low chronic doses of alkylating agents may stimulate the
production of a repair system to remove 06-alkylguanine. Clearly a knowledge of the
saturation level of enzymic repair systems and of the properties of any inducible re-
pair systems as a function of dose is necessary for an extrapolation from high to low
doses and from acute to chronic ones. Likewise, comparable kinetic data are needed
for human tissues before any valid extrapolation can be developed from cells to ani-
mals to humans. One of the most important future lines of investigation is in the
direction of determining the  concentration of DNA adducts in target tissue. This is
not an easy task at low chronic exposure levels but is being approached for a number
of specific products by ultrasensitive enzymic radioimmunoassays that are capable
of detecting close to 10~15 moles of the product (Hsu et al., 1981; Hsu et al., 1980;
Muller and  Rajewsky, 1980).
  One well-documented case of the quantitative role of DNA repair in carcinogenesis
is the effects of ultraviolet radiation in the induction of skin cancer in humans (Scott
and Straf, 1977). The most common photoproducts in cellular DNA are intrastrand
pyrimidine dimers. They are responsible for killing and mutagenesis in microorga-
nisms and for tumor production in a model fish system. Because pyrimidine dimers
are known to have adverse biological consequences, they have been extensively stud-
ied for their repairability (Setlow, 1980). Individuals with the disease xeroderma pig-
mentosum have a cancer risk from exposure to sunlight that is 103-to 104-fold greater
than that of the average population because these individuals have a defect in one or
more DNA repair pathways. Despite the high rate of dimer formation by sunlight
(~105 dimers per hr per exposed skin cell), normal DNA repair reduces the effect of
the ultraviolet dose to the skin by an approximate factor of 7 to 20 (Setlow, 1980).
The big difference in the skin cancer prevalence between individuals with xeroderma
pigmentosum and normal individuals is observed at the high levels of DNA damage,
damage from sunlight exposure that results in the formation of more than 104 dimers
per skin cell. What would be  the effect of DNA repair at lower UV doses and at very
low dose rates?  The effect would be less because, on the average, individuals with
xeroderma pigmentosum are not absolutely deficient in DNA repair so that at lower
dose rates their repair system might not be saturated so readily. This question of sat-
uration of repair and dose-rate levels is an important one to answer if we are to ad-
dress the problem of risk assessment. Cairns  (1980) has extrapolated from the
observation that people with xeroderma pigmentosum get no more internal cancers
than expected for the average population to suggest that U V-mimetic chemicals, such
as polycyclic aromatic hydrocarbons, are not important in environmental carcino-
genesis. Such carcinogens in the environment probably produce much less DNA
damage per exposed  cell than does a short sunlight exposure. Hence  it is important
to know how the relative sensitivities of xeroderma pigmentosum and normal cells
depend on dose and on dose rate. If the relative sensitivities are more or  less

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  H2N
                                                HN
                           H,N '    " N
                  deoxyribose
                                           deoxyribose
   06-ethylguanine
                                         7-ethylguanine
Figure 2.  Two ethylation products of deoxyguanosine in DNA. Ethylation at the O6-
          position leads to mispairing more often than ethylation at the 7-position.
        (A)
HighC
Low C
                                      1       2       3

                           Dose (concentration x time)
 Figure 3.  Each panel shows two possible relations between the dose of an
           environmental agent and the relative level of specific DNA adducts
           formed in cells. If adducts are unstable, are repaired, or are diluted by
           cell division, one would expect steeper dose-response curves at high
           concentrations, A, than at low concentrations,  B. The background level
           of adducts may be greater than zero, as shown, as a result of other,
           not measured, chemical reactions.

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independent of dose and dose rate, an extrapolation such as Cairns made would
have high credibility. If,  instead, the sensitivity of xeroderma pigmentosum cells
decreased to normal values at very low dose rates, the extrapolation would not be
valid. The dose-response curves at high dose rates for killing or mutagenesis of xero-
derma pigmentosum cells by polycyclic hydrocarbon derivatives indicate that they
are much more sensitive  than normal cells even at low total doses (Maher et al.,
1977). Nevertheless, the question remains an open one because these data have not
been obtained directly at the dose rates that would be equivalent to environmental
exposures; furthermore, ultraviolet carcinogenesis is  more than just the result of a
single exposure to light.

Multiple Insults

   Although changes in DN A can result in mutation and may induce transformation
or tumorigenesis  in a number of simple model  systems, the effects observed in
animals usually depend on a repetitive stimulus by a carcinogen or on the effects of a
carcinogen followed by a promoter (Slaga et al., 1978). Also, individuals are usually
exposed to a variety of agents at the same time or sequentially and such agents might
act synergistically so that what could be a threshold in experiments dealing with one
compound disappears in  the real world.
   What is needed, to extend short-term animal data to low doses and then to ap-
ply these data to humans is not only extrapolation rules (Rail, 1977) but also a basic
understanding of the relationship between  macromolecular  damage and biological
effects (Bridges, 1980; Campbell, 1980; Comptroller General's Report to Congress,
1981; McElheny and Abrahamson, 1979). For example, if damage to DN A were the
important initiating effect in carcinogenesis, the data  in Fig. 3 could be used to pre-
dict the possible levels of biological effects  from the level of DNA adducts (Fig. 4).
The background levels of adducts or biological effects  in both graphs could result
from the action of chemicals other than the one under consideration or from the nor-
mal metabolic wear and tear in biological systems in the absence of specific environ-
mental chemical insults. Of course, Fig. 4 is a great oversimplification since the bio-
logical effect would depend not only on the level of particular damage but on how
such damage changes with the time scale  of the biological system. Other dimensions
should be included in Fig. 4 indicating the effects of changing the rate of production
of adducts and  of other environmental agents, such as promoters or protectors. In
the absence of such data, extrapolation from animal experiments at high dose rates
to low dose rates necessitates somewhat arbitrary dose-response relations (Cornfield
et al., 1980; Mantel, 1980); moreover, the linear extrapolation usually cannot be
excluded (Guess et al., 1977). Thus, a rational approach requires a constant interplay
between fundamental studies at the molecular  level  and animal  data, and  an
interplay  between  animal  pharmacokinetics  and  human pharmacokinetics
(Anderson et al., 1980; Bridges, 1980; Campbell, 1980). A basic part of these interac-
tions is  a knowledge of which macromolecular changes are important in producing
biological effects and of the experimental techniques that are required for measuring
such changes in animals and humans. It  is the  rare instance, such as nonmelanoma
skin cancer, in which there are crude epidemiologic data that are sufficient to predict
approximately the increase in the biological effect that would result from an increase
in solar ultraviolet. The  extrapolation  is relatively easy  because almost all non-
melanoma skin cancer seems to arise from sunlight exposure and because basic bio-
logical data indicate which range of solar  wavelengths is carcinogenic (Scott and
Straf, 1977).
  It is not sufficient to know that some particular agent is bad.  It is necessary to
know how bad it is and to whom, and what  is the extent of the risk to one segment of
the population against the benefit to another. We are also faced with the fact that dif-
ferent segments of the human population react differently to environmental agents.
Thus, enhanced ultraviolet radiation is not a real problem for highly pigmented indi-
viduals and is a much more serious problem for fair-skinned individuals with a Celtic

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                      1            2            3

                                   [DNA adducts]
Figure 4.  Hypothetical dose-response curves relating a particular biological effect with
          concentration of specific DNA adducts. The animal data represent the typical
          ones obtained at high doses and, by themselves, give no indication of the
          possible curves joining them  to the point at zero adducts. The background
          biological effect could arise from causes other than the specific adducts
          under consideration.

mcestry. Moreover, there exist small, seemingly sensitive subsets of the population,
subsets that are defective in one or more mechanisms of DNA repair (Setlow, 1978).
In fact, initial experiments  indicate that there may be a relatively wide variance in
DNA repair capacity even among the seemingly normal population. Certainly there
ire many  indications  that  the  ability  to  metabolize potentially  hazardous
:nvironmental chemicals may vary  between individuals as a function of age and as a
function of other agents in the environment or in the diet (Harris et al.,  1980).
  Without a rational approach based on fundamental knowledge, we are in danger
of being led to absurdities of regulation, especially because the sensitivity of analyt-
ical techniques to detect deleterious agents is increasing rapidly. For example, there
is little doubt that high concentrations of diethylstilbestrol are dangerous, but to ban
such a compound from meat at the present sensitivity of detection seems to suggest
that the benefits at this level do not outweigh the estimated one death  per United
States population per 100 years (Jukes,  1977). A ban implies that there are no natu-
rally occurring biologically active analogs that might have effects orders of magni-
tude greater.

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PERSPECTIVE
  Risk assessment should involve three parameters: background, threshold, and ex-
trapolation. Background refers to the existence of an environmental level of a potent-
ially deleterious agent independent of the activities of humans and includes a level of
biological effects ascribable to unknown causes or to causes by  some agent other
than a chemical under consideration. Even in the absence of threshold, a knowledge
of the shape of the dose-response relation at low dose levels is crucial for predicting
the relative risk. Complications  arise because the environment and its interaction
with biological systems are made up of nonindependent terms although the labora-
tory analyses usually assumes they are so. Most of the factors involved in risk assess-
ment are not determinable directly by experiment but must be  extrapolated on the
basis of theory  or  of  fundamental  biological knowledge of the systems under
investigation. That knowledge is imperfect, but, just as an assessment of risk at low
doses of ionizing radiation can come about only from a fundamental understanding
of the interaction between  radiation and cells in  animals, so an assessment of risk
from low levels of  chemicals can come about only from a fundamental under-
standing of the interactions of such agents with cells and with tissues of animals and
humans. The direct experiment on humans is not possible and, if it were, its existence
would indicate the failure of present risk assessment procedures.
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Setlow,  R.B. Repair deficient human disorders and cancer. Nature 277:713-717, 1978.
Setlow, R.B. Different basic mechanisms in DNA repair. Arch. Toxicol. Suppl. 3:217-228, 1980.
Singer, B. W-Nitroso alkylating agents: Formation and persistence of alkyl derivatives  in mam-
   malian nucleic acids as contributing factors in carcinogenesis. J. Nail. Cancer Inst. 62:1329-
   1339, 1979.
Slaga, T.J., A. Sivak, and R.K. Boutwell (eds.) Carcinogenesis. Vol. 2,  Mechanisms of Tumor
   Promotion and Cocarcinogenesis, Raven  Press, New York, 558p., 1978.
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    SHORT-TERM  TESTS  FOR GENETIC TOXICITY

       Herman E. Brockman  and  Frederick J. de Serres


INTRODUCTION
  The genetic material of the human species and of all other species is our most pre-
cious heritage from long and still ongoing evolutionary processes. The great respon-
sibility of safeguarding this legacy is thrust upon each generation. The discipline of
genetic toxicology deals most closely with this awesome responsibility of ensuring
that the genetic material is transmitted to future generations in a form that will not
lead to increased incidences of ill health. This responsibility is difficult because of the
increasing number and diversity of man-made (synthetic) chemicals that are entering
our environment. The growth of the chemical industry burgeoned after World War
II, particularly the petrochemical industries. Presently, over 50,000 synthetic chemi-
cals are rather commonly used, and about 1,000 new chemicals or derivatives of ex-
isting ones enter the market place each year.  Most of these chemicals eventually
become part of our environment, and yet many of them have not been tested for their
genetic toxicity before being released  for general use.
  Currently,  there is great concern that toxic substances being introduced into our
environment  are contributing factors  in various classes  of  human  ill  health,
including genetic diseases, cancer, birth defects, heart attacks, strokes, and aging.
We have come to realize that toxic substances often  are in the air we breathe, the
food we eat, and the water we drink. Furthermore, we are often exposed to toxic sub-
stances in our occupations and homes.
  In the last  decade, a major concept about the mechanism by which many toxic
substances cause certain adverse health effects has gained increasing acceptance.
This concept is that the primary or initial cause of certain diseases is a mutation in
the genetic material of a cell. The development of this concept has resulted in the
field  of genetic  toxicology, a  hybrid  discipline that draws  upon the traditional
sciences  of genetics and toxicology. Genetics pursues an understanding of how
biological traits are inherited, how information is encoded in the genetic material,
and how this  information is used in cells. Toxicology deals with the identification of
toxic substances that cause illness and death, and with the mechanisms of their ac-
tion. Genetic toxicology is concerned  with toxic effects that are due to an agent's
ability to alter the information encoded in the genetic material of an organism.
  In this review, we will discuss the new field  of genetic toxicology. First, we will
provide a brief historical perspective (1900-1970), introducing and defining many of
the terms and concepts that we will use; then we will present the major advances that
have taken place in the last ten years. In this connection, we will discuss the federal
legislation and actions that have accompanied the development of this field, and fi-
nally we  will  speculate on future emphases in  genetic toxicology.
The Authors: Herman E. Brockman works in the Department of Biological Sciences, Illinois State Univer-
sity, Normal, Illinois 61761. Frederick J. de Serres works in the Mutagenesis Branch of the National Insti-
tute of Environmental Health Sciences, Research Triangle Park, North Carolina 27711.

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HISTORICAL PERSPECTIVE
  Genetics is one of the oldest of all sciences, and at the same time it is a relatively
young science. When humans started to cultivate plants and domesticate animals
about 10,000 years ago, they also began to practice artificial selection—that is, they
selected for breeding those members of a species that had desirable characteristics.
Thus, early humans chose certain phenotypes (what the individuals looked like) and,
simultaneously, the  underlying  genotypes (the individuals' genetic constitutions).
However, a  sound experimental basis for genetics was not established until 1866,
when the Augustinian monk Gregor Mendel published his results and interpreta-
tions from crosses between varieties  of the common garden pea Pisum  sativum
(Mendel, 1866). The significance of this publication was not appreciated until 1900,
when the  same kinds  of results and interpretations were published by others, at
which time genetics became recognized as a formal science.
  Mendel and others concluded that  in diploid organisms, such as humans, each
gene is present in one copy in the gametes (eggs and sperm) but is present in two
copies in all other cells in the body. Furthermore, a gene can occur in two different
states (alleles): a dominant allele and a recessive allele.  For example, in the garden
pea, purple and white are two different phenotypes of one trait, flower color. The
allele /"(purple) is dominant to  the allelep(white)—therefore, the genotype Pp, as
well as the genotype PP, is purple, whereas the genotype pp is white. Today, we
know that there can be many different alleles, some of which can have intermediate
states of dominance.

Some Major Discoveries Concerning Mutation
  One of the  rediscoverers of Mendel's results, de Vries, coined  the terms muta-
tion and mutant (de Vries, 1901). A mutation is an abrupt change in phenotype that
is inherited, and a mutant is the individual resulting from such a change.  In 1910,
Morgan reported that a single white-eyed male had appeared in a culture of red-eyed
fruit flies (Drosophila melanogaster) that had reproduced for many generations in
the laboratory (Morgan, 1910).  The red-eyed flies  had  been collected originally in
nature (in the  "wild"); hence, they were called wild type. Morgan showed that the
mutant allele for white eyes is recessive to the wild-type allele for red eyes and is lo-
cated on one of the sex chromosomes referred to as the X chromosome. By  1915,
many mutants of Drosophila had been recognized by their different phenotypes, and
each mutation had been mapped at a specific place (locus) on a particular chromo-
some by Morgan and his collaborators. Because these mutants had arisen abruptly
in cultures of  wild-type flies, they were referred to as spontaneous mutants.
  Many investigators in the early 1900s considered whether mutations could be in-
duced by certain physical and chemical agents. Using Drosophila, Muller( 1927) first
devised a test to detect induced mutation. This test is referred to as the sex(X)-linked
recessive lethal test because the mutations being measured are located on the X chro-
mosome,  are  recessive, and cause death. The test is unambiguous, quantitative,
sensitive, and rather simple. Muller showed that the physical agent X ray, at the dose
used, caused a 140-fold increase over the spontaneous frequency  of sex-linked re-
cessive lethal mutations. Muller had discovered the first mutagen, and we also can
credit him with devising the first short-term test for mutagenicity.  Muller's paper is
the real  beginning  of mutation research within the science of genetics and led
eventually to the field of genetic  toxicology. His paper also marks the origin of envi-
ronmental mutagenesis, which is the study of mutagens  that are known or suspected
to be in our environment. Muller also clearly stated that the environmental mutagen
X ray is a potential hazard to the genetic material of  humans. He indicated that
their indiscriminate use in medical practice might result  in an increased frequency of
mutations having lethal or detrimental effects in future generations. Unfortunately,
his message was largely ignored until much later.
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  The induced mutations studied by Muller and others were mainly sex-linked reces-
sive lethals or morphological (visible) mutations. Beadle and Tatum became inter-
ested in the underlying biochemistry of these mutations, and in 1941 they reported
the first induction of biochemical mutants (Beadle and Tatum, 1941). They used the
pink bread mold Neurospora crassa, which is a simpler organism than the garden
pea or fruit fly. Wild-type Neurospora grows on a very simple, mainly inorganic,
medium; therefore, it must synthesize a large array of organic compounds and mac-
romolecules needed for its growth and reproduction. They reasoned correctly that
they could induce mutations with X ray or ultraviolet light (UV) in genes determining
the synthesis of enzymes that catalyze biosynthetic steps. Their techniques were ex-
tended in the 1940s and 1950s to isolate biochemical mutants in several other micro-
organisms, including bacteria.
  In the 1940s, certain chemicals were shown to be mutagenic. Using the sex-linked
recessive lethal test in Drosophila,  Auerbach and Robson (1946) demonstrated that
mustard gas is mutagenic. Research on the mutagenicity of chemicals has expanded
tremendously since that pioneering study.
  Certain biochemical mutants of Neurospora isolated by the methods of Beadle
and Tatum were used to devise the reverse-mutation test in Neurospora (Jensen et
al., 1951). Mutations in the direction of wild type—mutant are called forward muta-
tions, whereas mutations in the direction of mutant—wild type are referred to as re-
verse mutations. The reverse-mutation test in microbes is much simpler and less time
consuming  than  most  forward-mutation  tests,  because  a selective (minimal)
medium is used on which only the revertant colonies grow. Reverse-mutation tests
became a popular way to test forthe mutagenicity of large numbers of chemicals. As
data from mutagenicity tests  accumulated in the 1950s and  1960s, mutagens were
discovered among numerous classes of chemicals such as food additives, medicinals,
pesticides, cosmetics, and industrial chemicals. Furthermore, it became known that
mutagens are found as highly complex mixtures in our environment.
  In experiments where two  or more doses of a mutagen are used,  dose-response
curves for percent survival and for mutation frequency can be constructed. For most
mutagens, these two activities go together—that is, as the dose of an agent is increas-
ed, the percent survival decreases and the mutation frequency increases. These re-
sults led many people to assume that mutagenic chemicals would be detected by clas-
sical toxicologic tests, because concentrations that caused mutation also caused kill-
ing. But, in the 1950s and 1960s, rather startling examples of chemicals termed "su-
permutagens" were discovered. These chemicals cause high frequencies of mutations
at doses that cause little or no killing. An early example was  TV-methyl-N'-nilro-N-
nitrosoguanidine in the bacterium Escherichia coli. The discovery of these mutagens
focused attention on the fact that  because existing classical toxicologic tests would
not detect certain chemical mutagens there was a  need for short-term tests for
mutagenicity as part of a standard set of toxicologic tests.
  A major concern in the area of chemical mutagenesis has been whether chemical
carcinogens also are mutagens. Almost all carcinogens have been identified from
studies in human epidemiology or from tests  with laboratory animals, usually mice
or rats. It was noted in the 1950s and 1960s that many chemicals that are carcinogens
in man and laboratory animals are not mutagenic in microbial tests. This discrep-
ancy was found to be due to the fact that these chemicals are not carcinogenic per se;
rather, they are metabolized in the animal to one or more carcinogenic intermediates.
The original noncarcinogenic chemical is termed a procarcinogen, and the  carcino-
genic intermediate is termed an ultimate carcinogen. If the conversion is due to en-
zymes, the process is called metabolic activation, and the enzymes are termed acti-
vating enzymes. Similarly, we also refer to promutagens and ultimate mutagens. The
earlier results in microbial tests were due to microbes not having nearly as extensive
a repertoire of activating enzymes as cells of  animals, especially those in the major
detoxifying organ, the liver. In order to make the microbial mutagenicity tests more
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"animal-like," various approaches were used to combine a promutagen, indicator
cells  (for  example,  bacteria),  and  activating  enzymes  from  animal  cells,
usually from rat or mouse liver. Using this important modification, studies in the
1970s suggested that most carcinogens are mutagenic and conversely, that most non-
carcinogens are nonmutagenic.
  The Environmental Mutagen Society (U.S.) was organized in 1969. This society
serves as a focal point for scientists from many fields who are concerned about the
adverse effects of environmental mutagens on the health  of  humans  and other
species.

Some Major Discoveries Concerning the Molecular Biology of Mutation
  Geneticists soon realized that an understanding of the mutational process was
impossible without answers to the questions: What is the molecular nature of the ge-
netic material? What  is the structure of this  molecule? How does this molecule
replicate? The answers to these questions emerged dramatically. Avery, MacLeod,
and McCarty (1944) provided the first direct evidence that the genetic material is de-
oxyribonucleic acid (DNA). The basic building blocks  of DNA are nucleotides,
which are covalently bonded together in a specific way to form a polynucleotide
chain. Watson and Crick (1953a) proposed a three-dimensional model for the struc-
ture of DNA in which two polynucleotide chains are wound around each other and
around an imaginary central axis in a regular way to give a helical structure called
the double helix. They stressed that the novel feature of their model is what later was
called the complementarity of the polynucleotide chains. This complementarity is
due to the fact that, among the four nucleotide bases in DNA, there are only two le-
gitimate base pairs: the purine adenine (A) with the pyrimidine thymine (T), and the
purine guanine (G) with the pyrimidine cytosine (C). The two polynucleotide chains
are held together along their length by hydrogen bonds between these bases.
  In the same year, Watson and Crick proposed models for the replication and spon-
taneous mutation of DNA, both of which are dependent on the complementarity of
the polynucleotide chains (Watson and Crick, 19536). Their model for replication is
disarmingly simple: the double helix unwinds, and each polynucleotide chain acts
as a template for the synthesis of a complementary daughter chain according to the
rules of complementarity. Their model for spontaneous mutation,  which was  the
first one based upon their model for the molecular  structure  of DNA, basically
says that the pairing of bases is not a perfect process. Occasionally, illegitimate base
pairing (other than A with T and G with C) occurs during DNA replication, and after
one or more rounds of replication a base-pair substitution mutation occurs in the
DNA molecule.
  Two other closely related discoveries about the molecular biology of mutation are
important to our discussion. First, the molecular nature of the initial lesions in DNA
caused by certain mutagens has been elucidated. Second, it has been discovered that
cells possess complex and amazingly effective enzymatic mechanisms for removing
or bypassing certain of these lesions. For example, a major lesion induced by UV is
the pyrimidine dimer, in which two adjacent pyrimidines in the same polynucleotide
chain are covalently bonded together. Because a dimer blocks  DNA replication, it
acts as a lethal lesion. Setlow and Carrier (1964) and  Boyce and Howard-Flanders
(1964) showed that a dimer and some adjacent nucleotides are excised, and that a
correct sequence of nucleotides is laid down in the resulting gap, using the comple-
mentary chain as a template. The steps are catalyzed by enzymes that constitute a
nucleotide excision-repair pathway. This repair pathway is relatively error free, but
there are other repair pathways that are referred to as error prone, because during
the process of bypassing the dimer an altered (mutated) sequence of bases often is
synthesized. The lethal effect of the lesion is circumvented, but a mutation occurs
during the  process. Therefore, error (mutation)-prone DNA repair pathways are
very important mechanisms for  mutation.
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Two Major Principles about Mutation
  One  principle is based on the fact that higher organisms are composed of two
kinds of cells: somatic cells and germ cells. Only the germ cells may transmit genetic
material to future generations; therefore, the consequence of mutation in the two cell
types is much different. Mutations in somatic cells are thought to be the initiating
event in certain diseases, such as cancer, whereas mutations in germ cells may result
in genetic diseases, such as Down's syndrome and sickle cell anemia, in future gener-
ations.
  A second principle is that there are many ways that the genetic material can be al-
tered, resulting in mutations. We  will discuss  three major types of alterations:
changes in the number of chromosomes, changes in the  architecture of chromo-
somes (chromosomal aberrations), and changes within a gene (gene mutations). The
first two types, collectively referred  to as chromosome abnormalities, generally are
detected by  microscopic examination. Gene mutations usually are too small to be
seen with the light microscope, but they  can be detected by genetic tests.
  In humans with a normal chromosome complement, each somatic (diploid) cell
contains two of each of 23 kinds of chromosomes. In germinal tissue, diploid cells
undergo meiosis, a division process that produces the gametes. A key event in meio-
sis is that the two members of a pair of chromosomes first pair, and then disjoin, one
member of each pair becoming incorporated into a gamete. Two gametes fuse at fer-
tilization,  and the diploid chromosome number is restored.
  One major type of alteration in the genetic material is a change in the number of
chromosomes per somatic cell to give other than 46 chromosomes. If the number of
chromosomes is an integral  multiple of 23, the condition is called euploidy (for ex-
ample, triploid  -  69). If the number is not an integral multiple of 23, the condition is
called  aneuploidy (for example,  monosomy = 45, trisomy   47).   In humans,
euploidy (other than the diploid condition) generally is lethal before birth, whereas
some kinds of aneuploidy are compatible with life but usually cause severe mental
and physical defects. Aneuploidy arises when the members of a pair of chromosomes
do not disj oin normally, so that one gamete may receive both members of the pair of
chromosomes and the other gamete may  receive no members of that pair. When a
gamete that contains less or more than 23 chromosomes combines with a normal
gamete, an aneuploid individual is  produced.
  Loss of a chromosome in humans  usually is incompatible with life, except for
monosomy (2N -  1) for the X chromosome (45,X). Most 45,X cases are aborted
spontaneously, early in pregnancy;  those that live are sterile females characterized
by short stature and failure to develop female secondary sex characteristics (Turner's
syndrome). Most kinds of trisomy (2N+ 1 = 47) are lethal. About one-half of detected
spontaneous abortions that are chromosomally abnormal are trisomics; individuals
that live often have severe physical and mental defects. The best-known example of
trisomy is of chromosome 21, which results in Down's syndrome.
  Another major type of alteration  in  the genetic material is a change in the
architecture  of the chromosomes but without the loss or gain of a complete chromo-
some. There are four kinds  of chromosomal aberrations: deficiencies and duplica-
tion of less than one entire chromosome,  and translocations and inversions, which
are rearrangements of less than one entire chromosome. In translocations, a section
of one chromosome is moved to a new location in the genome, whereas in inversions,
a section of a chromosome is inverted 180°. Chromosomal aberrations are caused by
agents  that break chromosomes (clastogenic agents). A third major type of altera-
tion in the genetic material is called gene mutations, because no more than one gene
is affected. Intermediate between gene mutations and the deficiency class of chromo-
some aberrations  are multilocus deletions,  which are  larger than one gene but
smaller than a deficiency  normally discernible through the light microscope. All
these types of alterations in the genetic material can cause ill health and death.
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Short-Term Tests for Genetic Toxicity
  Traditionally, carcinogens have been identified from epidemiologic studies in hu-
mans and by cancer tests in mice and rats. A major difficulty in identifying carcinogens
in epidemiologic studies in  humans is the long latent period (usually 20-30 years)
between first exposure of the person to a carcinogen and diagnosis of cancer. This is
demonstrated dramatically for the association between cigarette smoking and lung
cancer (Fig. 1). Although the incidence of cigarette smoking in males increased
steadily from 1900 to about  1940, the incidence of lung cancer did not show a similar
increase until 25 to 30 years later. The same latent period can be seen emerging in the
curves for cigarette smoking and for lung cancer in females. Epidemiologic studies
also often are limited by inadequate sample sizes and incomplete information on the
subjects. Even when these studies identify a chemical as carcinogenic, a portion of
the human population, which can be very large if the chemical is distributed widely,
will have been exposed to the chemical before it is identified as a carcinogen. The
other traditional method of identifying carcinogens is in laboratory tests with ani-
mals, but these tests unfortunately also have serious limitations: they are expensive
(at least 1/4 million dollars per chemical), lengthy  (about three years), and are rel-
atively insensitive (the low number of animals used limits the detection of weak car-
cinogens). Therefore, although both human epidemiologic studies and cancer tests in
laboratory animals will continue to be useful for detecting certain carcinogens, they
cannot meet the urgent need to test a large number of chemicals. Short-term tests for
genetic toxicity are needed as indicators of mutagenicity and as predictors  of carci-
nogenicity. These tests, as indicated earlier, are considered valid because mutation in
somatic cells is thought to be the initiating event in certain somatic cell diseases such
as cancer and because mutation in germ cells could cause an increase in genetic dis-
eases in future generations.
  Short-term tests for genetic toxicity are to various degrees relatively inexpensive,
rapid, simple,  and sensitive. There are some problems in interpretation and of ex-
trapolation to humans, as we will discuss,  but the great advantage of these tests is
that  they permit us to test chemicals and chemical mixtures before they are released
into  our environment.

MAJOR  ADVANCES  IN  THE  DEVELOPMENT  AND  USE  OF
SHORT-TERM TESTS FOR  GENETIC TOXICITY

Further Development Since About 1970
  Three main  problems in the development and use of short-term tests for genetic
toxicity have been the need to develop: (1)  a capability to test chemical metabolites
as well as the chemical itself; (2) strains that are highly sensitive to the mutagenic ac-
tivity of various chemicals; and (3) strains and tests that can detect a broad  range of
mutational alterations.

Metabolic Activation—
  A  number of experiments were undertaken in the late  1960s and early  1970s to
make the microbial tests more "animal-like." Indicator cells (usually bacteria), the
chemical being tested, and an enzymatic activating system from an animal usually
are combined. One of the first approaches to testing the mammalian metabolites of
chemicals for genotoxic activity was an in vivo test, the host-mediated assay (Legator
and  Mailing, 1971). Indicator cells and the chemical are injected into the host, usually
mice; as a control, cells only are injected into other mice. If the chemical is a promut-
agen, it may be metabolized by the host's enzymes to an ultimate mutagen, which
may enter the indicator cells. After specified intervals, the animals are killed, and the
indicator cells are recovered, washed, and tested appropriately for percent survival
and  mutation  frequency.
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     5,000
                      1920
                                     1940
                                                    1960
                                                                  1980
 Figure  1.   The long  latent period of cancer illustrated by the  association between
            cigarette smoking and lung cancer (after Cairns, 1975).
   Metabolic activation of chemicals also can be tested for in vitro, in which case the
 indicator cells, the chemical, and an activating system are combined in a test tube or
 on a petri plate.  Appropriate controls are included, and mutation is scored in the in-
 dicator cells. Mailing (1966) showed that dimethylnitrosamine is activated chemi-
 cally  in vitro by a hydroxylating mixture to a mutagenic form. Mailing (1971) later
 showed that the same chemical is activated enzymatically in vitro by a microsomal
 fraction from an homogenate of mouse liver. The supernatant from a 9,000 x g
 centrifugation of the liver homogenate contains the microsomes, which are especially
 rich in certain of the activating enzymes. This supernatant is referred to as the  S-9
 fraction, and, in combination  with certain cofactors, as the S-9 mix; currently,  it is
 the most common method of  in vitro metabolic activation. This  procedure is rela-
 tively rapid and inexpensive, but has the limitation that the entire in vivo metabolism
 of an organ or organism is not being tested.
   Another important development has been the use of certain chemicals or mixtures
 of chemicals to induce (stimulate increased synthesis of) the activating enzymes. The
 most commonly used inducer is Aroclor-1254, a mixture of polychlorinated bi-
 phenyls. It is injected into rodents, and a few days later the S-9 fraction is prepared
 from  the livers or other organs of these animals. The induced S-9 fractions have a
 higher concentration of  certain activating enzymes, especially cytochrome P-450,
 than uninduced S-9 fractions. This procedure has greatly increased the utility  of the
 microbial (especially the bacterial) short-term tests for genetic toxicity.
   Aflatoxins are examples of the many chemicals that require metabolic activation
 for mutagenicity. Aflatoxins are synthesized by certain strains of the fungus Aspergillus
flavus as they grow on foodstuffs, such as peanuts. Aflatoxin Bi, which is a potent
 liver carcinogen in a number of animals, is not mutagenic in bacteria in the absence
 of an S-9 fraction but becomes  so in the presence of an S-9 fraction from rodent liver.
 Certain classes of chemicals,  such  as polycyclic aromatic hydrocarbons, require
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metabolic activation.  An example is benzo(a)pyrene, which is found in cigarette
smoke, automobile exhaust,  and many industrial byproducts, and is, therefore,
ubiquitous in air.
  As one progresses along the evolutionary scale from simple organisms to higher
plants and animals, there is an increase in the amount and diversity of the activating
enzymes, which appears to reach its zenith in the liver of mammals. The common
tester strains of bacteria can activate relatively few chemicals. Therefore, if bacteria
are used in a short-term test for genetic toxicity, some kind of metabolic activating
system, such as an S-9 fraction from rat liver, usually is included in the test. Fungi,
such as Neurospora and Saccharomyces, can activate certain chemicals, for ex-
ample, aflatoxin BI and dimethylnitrosamine; nevertheless, mammalian enzymes
are necessary for the activation of other chemicals.  Drosophila, on the other hand,
has a rather rich repertoire of activating enzymes. Higher plants, such as maize, have
the ability to activate certain chemicals; for example, some of the pesticides used on
cultivated plants appear to be activated by them to ultimate mutagens. If chemicals
are being tested in a mammalian mutation test, activating enzymes are, of course,
already present. An exception to this generalization is mammalian cell lines, which
have a very limited ability to activate promutagens; therefore, when such cells are
used as indicators, an S-9 fraction is added. The inclusion of methods for metabolic
activation into short-term tests  for genetic toxicity has been a very important ad-
vance in  their development and  use.
Sensitive Strains—
   The second problem in the development and use of short-term tests for genetic tox-
icity has  been the need to develop sensitive strains of the indicator organism. "Sensi-
tive" means that the strains are strongly mutagenized by low doses of a wide range of
classes of chemicals. The bacterial reverse-mutation  test using histidine-requiring
(his) mutants of Salmonella typhimurium is an example of the development of high-
ly sensitive strains. Hundreds of his mutants were tested for their ability to be revert-
ed by various chemical mutagens by Ames, Hartman, and coworkers (Ames, 1971;
Hartmanetal., 1971). Four mutants were selected as testers based on their sensitivity
to specific mutagens (Ames,  1972). All four mutants detect intragenic mutations:
three detect frameshift mutagens of different specificities, and one detects mutagens
that induce base-pair substitutions.  Walker  (1977) estimates that these mutants
would detect only about 20 percent of 175 known carcinogens.
   Ames and coworkers took additional steps to increase the sensitivity of these mu-
tants. Some kinds  of lesions induced by mutagens in DN A are repaired by a number
of different enzymatic repair pathways. Earlier work had indicated that microbial
mutants  deficient in one of the repair pathways, a nucleotide excision-repair path-
way, are more sensitive than repair-proficient strains to the killing and mutagenic ac-
tivities of a number of mutagens. This repair pathway is so named because the lesion
and some adjacent nucleotides in one of the polynucleotide chains of the DNA mole-
cule are recognized and excised. The gap created by excision is filled in (DNA repair
synthesis) with nucleotides complementary to those in the intact  polynucleotide
chain. This repair pathway is relatively error (mutation) free. Cells  defective in nu-
cleotide excision repair, such as  uvrB mutants of Salmonella, must use other path-
ways, at least one of which is error prone. Thus, uvrB strains are more sensitive than
uvrB* strains to the mutagenicity of chemicals that cause such lesions. Ames and co-
workers  incorporated the uvrB mutation into the four Salmonella his testers in order
to increase the sensitivity of the strains (Ames, 1972). The next step in sensitization
was to increase the ability of mutagens that are large molecules to penetrate through
the outer layer that surrounds the cell membrane. In normal Salmonella, the cell
membrane is surrounded  by a  lipopolysaccharide (LPS)-containing layer, which
acts as a  partial barrier to the penetration of certain molecules-generally, the larger
the molecule, the  greater the exclusion. Certain mutations cause a defective LPS
layer. One such mutation, rfa, was incorporated into the four his, uvrB testers, re-


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suiting in another increase in sensitivity (Ames et al.,  1973). The inclusion of these
two features (uvrB and rfa) is estimated to permit detection of about 65 percent of
175 known carcinogens (Walker,  1977).
  A final step in the sensitization of the Salmonella testers raised the level of detec-
tion of 175 known carcinogens to its current 90 percent (McCann and Ames, 1976).
This step was the incorporation of an R plasmid into the bacterial cells. Plasmids are
small circular molecules of DN A that replicate within bacterial cells, and the R plas-
mids are so named because they carry antibiotic resistance genes. Earlier research
had shown that a his strain containing the plasmid was more sensitive than a strain
not containing the plasmid to the mutagenicity of certain chemicals. McCann et al.,
(1975b) showed that two of their his, uvrB, rfa mutants that contained the R plasmid
pKMlOl were very sensitive to many carcinogens that the nonplasmid-bearing
strains did not detect or only detected weakly. One of these plasmid-bearing strains
is a base-pair substitution mutant (TA100) and one is a frameshift mutant (TA98).
Today, these two are the most widely used strains for screening potentially mutagen-
ic chemicals.
Detection of Many Mutational Alterations—
  The third problem has been the need to develop strains and tests that can detect a
broad  range  of mutational alterations, especially the four types discussed earlier:
changes in the number of chromosomes (aneuploidy); changes in the architecture of
chromosomes (chromosomal aberrations); changes within a gene (gene mutations);
and deletion of two or more contiguous loci (multilocus deletions). The development
of tests to detect mutations has been evolving since 1927, and, in fact, most of the
tests listed in Table 1 were developed prior to 1970 during studies on the complex
processes  of many types of mutations in a variety of organisms. As concern grew
about the possible hazard that  environmental chemicals might pose  to the genetic
material, it was realized that many short-term tests for genetic toxicity already were
available. Some could be  used as they were, but others  needed refinement and
development for the purpose of testing large numbers of chemicals or for detecting
certain types of mutational alterations. In addition, some new tests were developed.
The extensive use of short-term tests for genetic toxicity in the last ten years is a good
example of how basic research can be applied to a major problem.

Major Short-Term Tests for Genetic Toxicity
  The number and diversity of short-term tests for genetic toxicity are great. There is
no "best" test, because each has  its strengths and limitations. Our approach here will
be to discuss the tests that the Environmental Protection Agency selected for system-
atic review and evaluation during Phase I of the GENEtic TOXicology (Gene-Tox)
Program (Green and Auletta, 1980; United States Environmental Protection Agen-
cy, 1980; Waters and Auletta, 1981). Not included in this discussion is the cell trans-
formation test, because it is reviewed fully elsewhere in this Volume by Upton. In
Table 1, we have organized the tests generally in order of test organism, from least to
greatest complexity,  which reflects their degree of evolutionary  distance from
humans. There are some exceptions, because we have attempted simultaneously to
list them roughly in the order that they might be used to detect different types of mu-
tational alterations.
  Although the list in Table 1 is  extensive, it is deceptively simple,  because more
than one test is included in many of the single entries.  For example, in the bacterial
DNA repair tests (No. 3), 55 pairs of repair-proficient and repair-deficient strains
among three bacterial species have been used. In the case of the four fungal species
(test No. 7 to 9), about six different tests can be done with each, whereas among high-
er plants (test No.  10) numerous tests are performed using seven major species. Be-
cause the tests were developed in part to detect a broad range of mutational altera-
tions, we  have  shown in Table 1 the types of mutational alterations (genetic  end
points) detected by each. The genetic end points are grouped into five major types:


                                     19

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 Table  1.   An Outline of Twenty-Four Short-Term Tests for Genetic Toxicity Selected for Systematic Review and Evaluation
            Phase I of the EPA's GENE-TOX Program
                                                                                       m
   Outline and Brief Descriptive      Test
        Name of the Tests         ID No.
DNA
Dam.
                                                          Genetic End Points
Gene Mut.
                                                   Rev.
                                                            For.
                                                                     MLD
                                                                              CA
                                                                                       AP
                                          Test
                                          ID No.
Comments About the Tests or
     Genetic End Points
  . Prokaryote (bacterial) tests
   A. Reverse-mutation tests
     1. Escherichia coli VJPz and
       WP2 uvrA strains
    2. Salmonella typhimurium his
       strains

   B. DNA repair tests
II. Mammalian cells in culture;
  specific-locus tests
  A. Chinese hamster ovary
     (CHO) cells
  B. Chinese hamster lung
    (V-79) cells

  C. Mouse lymphoma (L5178Y)
    cells


. Lower eukaryote (fungal) tests
 A. Neurospora crassa tests
                                                                                                7.
                                                           One trp allele, usually with uvrA.

                                                           One or more his alleles; usually
                                                           TA98 and TA100, which are
                                                           uvrB,  rfa, pKM101.
                                                           Differential growth inhibition in
                                                           repair-deficient/repair-
                                                           proficient pairs of  strains.
                                                           6-Thioguanine resistance =
                                                           hypoxanthine-guanine phos-
                                                           phoribosyl transferase (HGPRT)
                                                           locus.
                                                           5-Br-deoxyuridine ortriFI-
                                                           thymidine resistance = thymi-
                                                           dine kinase (TK) locus.
                                                           6-Thioguanine or ouabain resis-
                                                           sistance = HGPRT locus or
                                                           NaVK+ ATPase locus.

                                                           "Mainly two ad-3 alleles; "mainly
                                                           at the ad-3 region; "ad-3 region.

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                                                       Table 1.  Continued
                                                          Genetic End Points
  Outline and Brief Descriptive
       Name of the Tests
 Test
ID No.
DNA
Dam.
                    Gene Mut.
                                                    Rev.
                                                             For.
                                                                     MLD
                                                                              CA
                                                                                       AP
        Test
       ID No.
Comments About the Tests or
     Genetic End Points
   B. Aspergillus nidulans tests
   C. Saccharomyces cerevisiae        9
     and Schizosaccharomyces
     pombe
IV. Higher plants: genetic and          10
   cytogenetic tests

 V. Insect (Drosophila melano-
   gaster) tests
   A. Chromosome abnormality       11
     tests
   B. Sex-linked recessive lethal       12
     test

VI. Mammalian tests
   A. Whole animal (in vivo) tests
     1. Mouse spot test              13
      2. Specific-locus test in mice     14
      3. Micronucleus test in bone     15
        marrow erythrocytes
      4. Dominant lethal test          16
      5. Heritable translocation        17
        test in mice.
      6. Sperm tests                 18
                    Sb       Sc


                   S,Gb     S,GC
                                     S,G




                                     Ga

                                     G




                                     S


                                     G
                                               G
                                               G
 S        8.   "Mitotic crossing over; bone arg
               allele; ctwo systems; dbalanced
               translocations.
S,G       9,   "Mitotic crossing over and mitotic
               gene conversion;b>one allele;
               °>one locus.
 S       10.   "Mitotic crossing over; b>one
               allele; °>one locus.
                                                                11.   "Translocations.

                                                                12.   The last two genetic end points
                                                                     are not distinguished routinely.
         13.   Coat color mutations in embryos;
               all genetic end points are not
               distinguished routinely.
         14.   Visible mutations at seven loci;
               all genetic end points are not
               distinguished routinely.
         15.   The genetic end points are not
               distinguished routinely.
         16.   Early (in utero) deaths.
         17.   Only translocations scored.

         18.   Sperm anomalies; genetic end
               points not well defined.

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                                                        Table  1.   Continued

                                                           Genetic End Points
Outline and Brief
Name of the
Descriptive Test
Tests ID No
DNA
Dam.
Gene Mut.
Rev.
For.
MLD
CA
AP
Test Comments About the I ests or
ID No. Genetic End Points
    B. In vitro and in vivo tests
      1. Sister chromatid exchange    1 9
        (SCE)test
      2. Cytogenetic tests             20
    C. Special tests
      1. Host-mediated assays         21
      2. Tests of body fluids
    B. Other DNA repair tests
22
 VII. Tests that detect repair of DNA
    A. Unscheduled DNA synthesis     23
      (UDS) tests
24
                                             S,G
19.   Sister chromatid exchanges.

20.   Chromosomal aberrations.

21.   Appropriate host, indicator
      organism, and test; genetic end
      points depend on test.
22.   Appropriate indicator organism
      and test; genetic end points
      depend on test.


23.   DNA repair replication in vitro,
      usually in primary rat hepato-
      cytes or human  fibroblasts.
24.   Various techniques; not
      presently useful as tests for
      genetic toxicity.
Abbreviations; Dam..damage; Mut., mutations; Rev. reverse; For., forward; MLD, multilocus deletions; CA, chromosomal aberrations; AP, aneuploidy,
              B, bacterial cells; S, somatic cells; G, germ cells; SCE, sister chromatid exchange; UDS, unscheduled DNA synthesis.

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DNA damage, gene mutations, multilocus deletions, chromosomal aberrations, and
aneuploidy. All but the first have been discussed earlier. DNA damage tests measure
different kinds of end points, none of which fits clearly into the other four types of
genetic end points. We have indicated whether the mutational alterations occur in
somatic or germ cells.
  In the limited space here, we can discuss only the main features of each test. Refer-
ences will not be given because many of the tests have been reviewed in Chemical
Mutagens: Principles and Methods for Their Detection (Hollaender,  1971; 1973;
1976; Hollaender and de Serres, 1978; 1982) and because a report on each test will be
published in Mutation Research: Reviews in Genetic Toxicology under the aegis
of the EPA Gene-Tox Program. In Part I of Table 1, we have entered the bacterial
tests: two detect reverse mutation, and one detects DNA damage. We discussed the
reverse-mutation test in Salmonella (No. 2) earlier, when we used it as an example of
how sensitive strains were developed. It is currently the most widely used test for the
detection of genotoxic chemicals. The bacterial tests are relatively simple, rapid,
inexpensive, and sensitive. Fig. 2 illustrates the  simplicity of the DNA damage test
using a DNA repair-deficient (pol A\) and a repair-proficient (pol A*)  strain of E.
co//.7V-Hydroxyurethane is scored as genotoxic because the chemical causes a larger
zone of growth inhibition in the pol A\ than in the pol A* strain. This differential in-
hibition of growth occurs because damage caused in DNA by this chemical  is
repaired less efficiently in the pol A i  than in the pol A* strain. When equal zones of
growth inhibition are observed in both strains, the chemical is scored as not geno-
toxic. Therefore, a plus or minus result for a single chemical can be obtained from
only two petri plates in a few days. The effect of an S-9 fraction can be assayed with
an additional two plates.
  The in vitro tests using mammalian cells in culture are entered next. The cells can
be handled with experimental procedures similar to those used with bacteria. All
three (No. 4 to 6) detect forward mutation at a specific locus  by using resistance to a
drug that is an analog of a normal metabolite. This selection system permits the tests
to be rather rapid, simple, and inexpensive, but not as much so as the bacterial tests.
Mammalian cell culture tests have the advantage that the genes being mutagenized
are in eukaryotic chromosomes. Furthermore, the use of forward mutation rather
than reverse mutation permits detection of a wide spectrum of gene mutations.
  The lower eukaryote (fungal) tests (No. 7 to 9) are entered next in Table 1. Because
fungi are microorganisms that are single cells during all or part of their life cycles,
some of the experimental procedures are similar to those used with bacteria. The
fungal tests are difficult to discuss, because for each species about six tests, which
detect different genetic end points, can be used. Like one of the bacterial tests, DNA
damage  can be detected in two fungi, but in  these cases, the damage is detected as
mitotic crossing over and gene conversion. In Saccharomyces cerevisiae, the test that
detects  gene conversion is rapid,  inexpensive, simple, and very sensitive. Reverse
mutation can be detected in fungal tests that are very similar to the bacterial reverse-
mutation tests. As in mammalian cell cultures, forward-mutation tests at specific
loci are used in fungi. In addition, the important genetic end points of multilocus de-
letions, chromosomal aberrations, and aneuploidy are  detected in appropriate
fungal tests; it is noteworthy that aneuploidy can be detected during meiosis in two
species.
  The higher  plant tests (No. 10) are especially complex to discuss, because nu-
merous genetic and cytogenetic tests with many genetic end points have been used in
seven major species. One  advantage of the higher plant tests is that often the genetic
end points are detected in germ cells.  Another advantage is that  the mutagenicity of
many pesticides often can be tested for in the plants on which they are used. Further-
more, higher plants appear to have some unique systems for metabolic activation of
certain chemicals.
                                     23

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Figure 2.   DNA damage test using the bacterium Escherichia coli. Effect of  N-
           hydroxyurethane on the growth of repair-proficientpoM* (left) and repair-
           deficient polAi (right) strains. This chemical is scored as genotoxic because
           there is a larger zone of growth inhibition in the pol At  strain (from
           Rosenkranz and Leifer, 1980).
   Tests using Drosophila (No. 11 and 12) are the first whole animal tests that we
 have listed in Table 1. They are very useful because there is an extensive literature on
 the mutagenicity of chemicals in Drosophila and because the metabolic activation
 systems of this organism appear to be similar to those of mammals. Also, a number
 of genetic effects, transmitted through the germ cells, can be assayed. The tests are
 somewhat more time consuming and expensive than many entered earlier. The sex-
 linked recessive lethal test is a forward-mutation test that detects recessive lethalsat
 ~600 loci on the X chromosome; it is the most sensitive and economical in vivo pro-
 cedure that measures mutation in all the germ cell stages of an eukaryote.
   Within the mammalian tests, we have entered seven whole mammal (in vivo) tests
 (No.  13 to 18), which are usually done in mice. Their general advantage is that the
 chemical  in question interacts with the mammal's activating and  inactivating en-
 zymes. Obviously, this situation is somewhat similar to that which humans face in
 their environment; therefore,  findings from these tests may be highly relevant to con-
 siderations of hazards and risks that chemicals pose to humans.
   In  the mouse spot test (No. 13), embryos heterozygous at several loci determining
 coat  color are exposed in ulero at nine to ten days of development by injecting the
 chemical  into  the  peritoneal cavitity of the  mother. Forward  mutation of the
 dominant allele at one of the heterozygous loci in an embryo produces a cell that is
 homozygous recessive for a mutant phenotype. This cell gives rise through division
 to a clone of cells, some of which may determine coat color. This mutation will be ex-
 pressed as a spot of mutant coat color, such as a tan spot, in an otherwise black coat;
 the mutations are scored about one week after birth, that is, about three weeks after
 exposure  in ulero (Fig. 3). This test detects forward mutation due to a number of
 mutational alterations, and is  the most rapid in vivo forward-mutation test in somat-
 ic cells of mammals.
   Although biochemical and  immunological markers can be used in the mouse spe-
 cific-locus test (No. 14), seven visible  markers, mainly affecting coat color are used
 most frequently. For example, a brown (bb) female is mated to a black (BB)  male
 that has been exposed to a test chemical. If a forward mutation from Blob occurs in
 a sperm that fertilizes an egg, a zygote that is bb will be formed, which will develop
                                     24

-------
into a brown mouse. Black progeny indicate no mutation. Thus, the incidence of for-
ward mutation at seven loci is scored. As in the sex-linked recessive lethal test in Dro-
sophila, the mutagenicity of a chemical at various stages of spermatogenesis can be
tested by varying the time between exposure of the male to the chemical and mating.
This test is relatively simple and easily scored, but it is debatable whether this test can
be called a short-term test. Nevertheless, the mouse specific-locus test is currently the
only practical and reliable method for determining whether a chemical induces heri-
table forward mutations in mammals. Therefore, it is an important test for estimat-
ing germ cell risk in humans.
 Figure 3.  The mouse spot test for detection of a forward mutation at one of the
           heterozygous loci determining coat color. The pelt shows a typical type of
           recessive tan spot over the right hip region (arrow). Photograph courtesy of L.
           B Russell.
   The micronucleus test (No. 15) has been used in several cell types from animals
 and plants, but recently formed  red  blood cells (polychromatic erythrocytes) of
 mammals (usually mice) have been used most often. Daughter chromosomes nor-
 mally migrate to opposite poles during cell division and become incorporated into
 two daughter nuclei. On the other hand, a chromosome fragment, which arises due
 to chromosome breakage, or a whole chromosome that fails to migrate becomes in-
 corporated into a structure that stains and looks like a small nucleus—a micronucle-
 us (Fig. 4). This test is the least expensive and fastest means available to detect these
 kinds of genetic end points  in mammalian somatic cells.
   In the dominant lethal test (No.  16) in mammals (usually mice or rats), males are
 treated with a chemical and then mated to females according to a protocol that per-
 mits all stages  of spermatogenesis to be tested. The females are killed at about 14
 days of pregnancy, and the  number of early deaths out of the total  number of im-
 plants is scored. Early deaths are scored as dominant lethals, which are thought to be
 due mainly to  multiple  chromosome breaks in the germ cells of the treated male.
 Although a positive result in this test indicates that a chemical causes chromosome
 breakage in germ cells, use of this test is limited by its relative insensitivity and by the
 fact that events other than chromosome breakage may contribute to what are scored
 as dominant lethals.

-------
  The heritable translocation test (No. 17) in mice is similar to the dominant lethal
test in some respects, but in this case the genetic end point, translocations that are in-
herited, is precise. Again, males are treated with the test chemical and then mated tc
females. A stage of spermatogenesis in the male progeny is analyzed microscopically
for translocations, which were induced in germ cells of the male parent and transmit-
ted to a son. Although relatively expensive, difficult, and lengthy, the test clearly
identifies the ability of chemicals to cause one class of heritable chromosomal aber-
rations in mammals.
Figure 4.  The detection of chromosomal aberrations by the formation of micronuclei.
           A single chromosome is represented; more would be present. A break occurs
           in one chromatid of the chromosome in the cell  at the left, producing a
           fragment. The two daughter chromosomes migrate to opposite ends of the
           cell by action of spindle fibers attached to the centromeres, but the fragment
           does not move. The daughter chromosomes become surrounded by a
           nuclear membrane to become daughter nuclei, and the fragment becomes a
           micronucleus. Illustration courtesy of A. S. Raj, A. B. Krepinsky and J. A.
           Meddle.

   Sperm tests  (No. 18) are  attractive because they detect  spermatoxic  agents in
mammals, including humans. The end points are sperm count, sperm  motility, and
sperm head morphology.  Unfortunately, when sperm count or motility  are de-
creased, or when sperm head morphology is altered, we do not know what fraction,
if any, of the effect is due to genotoxicity of the chemical. Therefore, the tests detect
spermatoxic, but not necessarily genotoxic, agents. One test, the fluorescent Y-body
(YFF) assay, has been used  occasionally in humans to detect aneuploidy of the Y
chromosome.
   Two tests are listed under mammalian tests as "in vitro and in vivo tests," because
they are used on cultured mammalian cells that have been treated with  a chemical or
on cells from exposed mammals, including man. This dual approach is  very useful in
certain situations. Test No. 19 is the sister chromatid exchange (SCE) test. At met-
aphase, each chromosome is  composed of two sister chromatids held together at the
centromere. If the sister chromatids are labelled differentially by appropriate pre-
vious  DNA replications  in  the presence of a nucleotide analog, 5-bromodeoxy-
uridine, the sister chromatids stain differentially with certain dyes. In such cells there
is a spontaneous frequency of SCEs, that is, places at which there has been a clear,
reciprocal interchange between sister chromatids (Fig. 5 A). Certain chemicals, such
as the drug mitomycin-C, increase their frequency (Fig. 5B). The molecular mech-
anism of SCEs is not known, although they must arise from breakage and reunion of
DNA molecules, which presumably occurs, probably indirectly, as a consequence of
DNA damage. Therefore, we classify the SCE test as a DNA damage test.  The assay
is relatively simple,  rapid, inexpensive, and sensitive.
   The cytogenetic tests (No. 20) in mammals also are done in vitro or in vivo. Six
major tests detect chromosomal aberrations due to chromosome breakage  as their
genetic end points. Four of these tests are done in vivo and use bone marrow cells,
                                     26

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spermatogonia, spermatocytes, or oocytes or early embryos. The fifth test is an in
vitro test in which cultured mammalian cells (No. 4 to 6), mainly Chinese hamster
ovary (CHO) cells, are used. Leukocytes, including those from  humans, are used
both in vitro and in vivo.
                      "•*•.  *
              Wv»  \^     X        •***•»
     .x^"      .,'
        "W

  **-l'
                    **S
         %-~ .   
-------
major steps: hazard identification, hazard evaluation, and risk estimation. As we
proceed through these three steps, we inevitably will focus on the risk that mutagenic
chemicals pose to humans, but each test also should be thought of as a means of
assessing effects  on other organisms as well.
  Starting with data on a chemical from one or more short-term tests, we can divide
our analysis into the three steps mentioned above (Table 2). In order to analyze the
results from the different tests under one or more of these three headings, we must
consider the genetic end points that each detects, and whether they occur in somatic
or germ cells. Although we have entered most of the tests from Table 1 in Table 2 to
demonstrate how each of them could be used at one or more steps of the analysis,
we do not imply that all of the tests entered under each step would be used for a given
chemical. The choice of which tests to use at each step is influenced by factors such as
the chemical properties of the compound being tested, its distribution in the environ-
ment, and the possible route(s) of human exposure.
  In the first step, hazard identification, a chemical is identified as being a genotoxic
hazard if it is positive in one or more tests. Tests used at this step should be relatively
rapid, inexpensive, simple, and sensitive; usually identification comes from the re-
sults in bacterial tests,  which efficiently  screen large numbers of  chemicals by
detecting  DNA damage or reverse mutation. The test for gene  conversion in S.
cerevisiae also could be used here. The more time-consuming and expensive tests
that  detect forward mutation,  multilocus deletions, chromosomal aberrations, or
aneuploidy usually are delayed until the next step of the analysis. However, if suit-
able  tests that detect multilocus deletions, chromosomal aberrations, or aneuploidy
are developed, they also might be included at this step. Tests for aneuploidy should
be included at the first step when they are thoroughly validated, because these tests
detect those chemicals that interfere with the disjunction of chromosomes through
interactions with proteins. To summarize the step of hazard identification, one
screens  many chemicals in simple  short-term tests in order to  identify rapidly those
rtiat  are genotoxic.
  In the second step, hazard evaluation, the main goal is to determine whether chem-
icals that are genotoxic in p'rokaryotic tests also are genotoxic in relatively simple
eukaryotic tests. A positive result  for DNA damage detected earlier in bacteria can
be tested for in fungal, higher plant, and mammalian DNA damage tests (No. 8 to 10,
19,23).  A positive result in reverse-mutation tests in bacteria can be tested for in fun-
gal and  higher plant reverse-mutation tests (No. 7 to 10). The test chemical can be
assayed in tests that detect forward mutation in eukaryotes (No. 4 to  10, 12). Other
eukaryotic tests can be used to determine if the chemical has clastogenic activity by
detecting chromosomal aberrations (No. 8, 10, 11). Aneuploidy can be detected in
certain tests in fungi,  higher plants, and Drosophila (No. 7 to II). Because multi-
locus deletions often act as lethals, they are not detected in bacterial and many other
tests. Yet, the detection of this type of mutation is very important, because  there is
evidence from the analysis of specific-locus mutations in mice that multilocus dele-
tions can have deleterious  or lethal effects in heterozygotes. Most of the mutations
detected in reverse-mutation tests (and some forward-mutation tests) are recessive
and will not be expressed in the first generation from a treated diploid parent. On the
other hand, because multilocus deletions often act as dominant or semidominant
mutations, they are expressed in the first generation from a treated diploid  parent.
The fraction of multilocus deletions can be determined in the ad-3 forward-mutation
test in Neurospora  (No. 7).
  From the results of the tests shown under hazard evaluation (Table 2), one verifies
or fails to verify  the positive results for genetic toxicity obtained with a chemical in
the first step of hazard identification. Simultaneously, one extends the data on a
given chemical to more kinds of organisms and genetic end  points. The results from
tests done on somatic cells of fungi, higher plants, and animals are relevant to evalu-
ating genotoxic hazards to somatic cells. If, however, certain of the tests shown un-
der hazard evaluation are done, information also is obtained on genotoxicity to


                                     28

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       Table  2.   Possible Use of Tests from Table 1 for Hazard Identification, Hazard Evaluation, and Risk Estimation

                                                                             Genetic End Points
Test DNA Gene Mut.
Test Name
Hazard Identification
Bacterial DNA repair tests
Bacterial reverse-mutation tests
Hazard Evaluation
SCE tests in mammals
UDS tests in mammals
Mammalian cells in culture tests
Fungal tests
Higher plant tests
Sex-linked recessive lethal test in
Drosophila
Chromosome abnormality tests in
Drosophila
Risk Estimation
Mouse spot test
Micronucleus test in mammals
Cytogenetic tests in mammals
Specific-locus test in mice
Heritable translocation test in mice
Dominant lethal test in mammals
ID No. Dam. Rev.

3 B
1,2 B

19 S
23 S
4-6
7-9 S S
10 S S,G

12

11

13 S
15
20
14
17
16
For. MLD CA






S
S S S
S,G S,G

G G

G

S S S
S
S,G
G G G
G
G
AP







S,G
S



G

S
S




Abbreviations:  Dam., damage; Mut., mutations; Rev., reverse; For., forward; MLD, multilocus deletions; CA, chromosomal aber-
               rations; AP aneuploidy; B, bacterial cells; S, somatic cells; G, germ cells; SCE, sister chromatid exchange; UDS,
               unscheduled DNA synthesis.

-------
germ cells and/or about transmissibility (heritability) of mutational alterations in
submammalian species. Under forward-mutation tests, certain of the higher plants
detect mutations in germ cells. The sex-linked recessive lethal test in Drosophila de-
tects forward mutations that are induced in one or more stages of spermatogenesis
and transmitted to the next generation. Similarly for chromosomal aberrations, cer-
tain of the tests detect chromosomal aberrations in germ cells and/ or measure trans-
mission of the aberration through germ cells from one generation to the next. Be-
cause some of these tests are whole plant or animal tests, the chemical is subjected to
the organism's metabolism. If a chemical is positive in some of the tests shown under
hazard evaluation, it should be tested further using the tests shown under risk esti-
mation.
   In the third step, risk estimation, tests are used that will provide data most relevant
to estimating the risk a chemical poses to human somatic or germ cells. Therefore,
the tests entered at this step in Table 2 are mammalian tests. The genotoxic potency
of a  chemical for the different types of genetic end points is important. The mouse
spot test, the micronucleus test, and certain cytogenetic tests (No. 13, 15, 20) can be
used for estimating somatic cell risk. The specific locus test and the heritable trans-
location test in mice (No. 14, 17) and the dominant lethal test and certain cytogenet-
ic tests (No. 16, 20) can be used for estimating the risk of a given chemical for the in-
dicated genetic end points in germ cells.
   The three-step approach shown in Table 2 is an example of what is sometimes re-
ferred to as a tier approach for assaying the genotoxicity of chemicals. As we indi-
cated earlier, all of the tests normally would not be used for a given chemical. If, how-
ever, a genotoxic chemical is known to be distributed widely in the environment or if
a large human population is known to be exposed, many tests, including those that
detect all of the genetic end points, should be included. By considering the potency of
a chemical for these end points as well as information such as the routes of human
exposure, the risk that the chemical poses for certain somatic cell and genetic
diseases may be estimated. For example, if a chemical is a potent mutagen in somatic
cell tests such as the mammalian spot test, but a very weak mutagen in germ cell tests
such as the mouse specific-locus test, one would estimate that the chemical poses a
greater risk for cancer than for genetic diseases. In another case, a chemical may
cause aneuploidy in all of the appropriate tests, but be negative in other tests; one
would estimate, therefore, that the chemical poses a risk for spontaneous abortion
and  aneuploidy diseases such as Down's syndrome.
Large-Scale Programs to Evaluate the Efficacy of Selected Short-Term
Tests for Genetic Toxicity
   Studies to evaluate the efficacy of certain short-term tests for genetic toxicity were
initiated independently in the United  States and Japan in 1972. In both studies, the
Salmonella/ microsome test was used, and many of the test chemicals were known to
be prevalent in the environment and/ or were known to be carcinogens or noncarcin-
ogens from human epidemiologic studies or rodent cancer tests. In the United States
study, sponsored by the National Cancer Institute, 102 chemicals were tested (Poirier
and de Serres, 1979), and, in the Japanese survey, sponsored by the National  Insti-
tute of Hygienic Sciences, 60 chemicals were assayed (Sugimura et al., 1976).  After
. 1972, these studies were merged by mutual agreement under the U.S.-Jap^n Cooper-
ative Medical Sciences Program. In 1975 and 1976, McCann, Ames, and coworkers
published a tabulation of the results on almost 300 chemicals that had been assayed
in the Salmonella/microsome test (McCann  et al., 1975a;  McCann  and Ames,
 1976). The chemicals were selected because they could be classified as carcinogens or
noncarcinogens based on previous data from human epidemiologic studies or rodent
cancer tests.
   In  1977, Imperial Chemical Industries, Limited, in Great Britain sponsored the
study of 120 coded chemicals in six short-term tests (Purchase et al., 1976; 1978),
and, in 1979, a large international program evaluated 42 chemicals in 30 short-term
tests (de Serres and Ashby, 1981). Two important features of the latter study are that

                                     30

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14 pairs of structurally related carcinogens and noncarcinogens were tested, and that
the bacterial tests were done in 15 laboratories, which permitted an interlaboratory
comparison of the results.
  Two major conclusions emerged from these studies. The first is that there is a
strong positive association between the carcinogenicity and the1 mutagenicity of
chemicals. We mean by this that a large majority of the known carcinogens are muta-
genic and that a large majority of the known noncarcinogens are not mutagenic. The
largest number of chemicals where this association can be noted is the almost 300
chemicals tabulated by McCann and Ames (1976): 90 percent (157/175) of the car-
cinogens are mutagenic in the Salmonella/ microspme test, and 87 percent (94/108)
of the noncarcinogens are not mutagenic in the same bacterial test. This strong pos-
itive association and other evidences provide the scientific basis for the concept that
short-term tests for genetic toxicity can be used to screen large numbers of chemicals
not only for genetic toxicity, but also with a high probability for potential carcino-
genicity. McCann and Ames (1976) also found that none of 46 common biochemi-
cals  is mutagenic. The second major conclusion, which became evident from the
studies where more than one test was included, is that no single test detects all car-
cinogens as mutagens. Therefore, a battery of short-term tests is required for a com-
prehensive screening of chemicals for genetic toxicity. The battery should include
tests that detect all classes of mutagenic chemicals and the major kinds of genetic end
points.
Complex Mixtures  of Chemicals  and  Environmental Monitoring
  We have focused  mainly on the usefulness  of short-term tests to detect the geno-
toxicity  of single chemicals, often  those that are  known to  be present in the
environment and/or that are known to  be  carcinogens. It is rare, however, that an
organism in its environment is exposed to a single chemical; rather, they are exposed
to complex mixtures of chemicals. Complex mixtures can be obtained from a point
source or from the environment. Obviously, these two kinds of souces overlap. Ex-
amples  of point-source complex mixtures are cigarette smoke condensates, hair
dyes, cosmetic formulations, charred meat,  soot,  and automobile emissions. Ex-
amples from our environment are particulates isolated from air samples and mix-
tures of  organic chemicals isolated from drinking water, rivers, and lakes. These
chemical  mixtures  can  be collected  and  then  tested  in  the  laboratory, or,
alternatively, test organisms can be placed in the environment. The mutagenicity
testing of complex mixtures and environmental  monitoring are relatively new,
rapidly growing, difficult, and important areas of research.
  A complex mixture of chemicals can  be subjected to short-term tests for genetic
toxicity using the same tier approach as described above and outlined in Table 2. An
example is cigarette smoke condensate, which is the particulate fraction of cigarette
smoke containing over 2,000  chemicals and commonly referred to as tar. It  is
mutagenic in a number of short-term tests (DeMarini, 1981). Cigarette smoke con-
densate has been fractionated in order to identify the mutagenic chemicals and to
remove competing toxic, but nonmutagenic,  chemicals. When testing for air pollu-
tion, samples of air are drawn through a filter,  on which the particulates are collected
and  concentrated. The particulates are dissolved  in a suitable chemical, such as
dimethyl sulfoxide, and tested for genotoxicity. The complex mixtures of chemicals
in air particulates from  certain heavily polluted urban  air samples are clearly
mutagenic (Hughes  et al.,  1980). The organic chemicals in samples of drinking wa-
ter can be concentrated by passing the water  through a resin in a glass column; the
organic chemicals then are eluted with a small amount of organic solvent. Certain of
these also have been shown to be mutagenic (Loper, 1980).
  The higher plant tests listed in Tables 1 and 2 are promising tools for in siiu envi-
ronmental monitoring, in which the test organism is placed in the environment or an
organism is used that is already present there. For example, maize seeds can be
planted in plots where various pesticides are applied to the soil (Plewa and Wagner,


                                     31

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1981). The maize seeds are of special varieties in which reverse or forward mutation
can be scored in the pollen. Certain pesticides are mutagenic in these tests. Sparrow
pioneered the use of the higher plant Tradescantia as an in situ environmental mon-
itor (Sparrow and Schairer, 1971). Somatic mutations are scored in cells of the
stamen hairs in flowers of plants heterozygous for a pair of alleles determining flow-
er color. The heterozygote (Bb) has blue stamen hairs; mutation of the B allele to the
b allele produces a pink cell in the chain of 25-30 cells of a stamen hair  (Fig. 6). This
short-term  test is being used in a mobile laboratory to monitor air samples from
different locales (Schairer et al., 1978). The additional genetic end points of micro-
nucleic and chromosomal aberrations can be scored in  appropriate tests in this
versatile plant. Another kind of in situ environmental test utilizes an indigenous
organism; Klekowski (1978) has used the royal fern Osmunda regalis as such a mon-
itor. This is a promising area of research, but much work still needs to be done.

Short-Term  Tests in Human Population Monitoring
   Human population monitoring is a natural extension from environmental moni-
toring. We noted that in indigenous in situ environmental monitoring an organism
that is a normal resident of a particular environment is used to monitor  the mutagen-
icity of that environment. Humans can be used as indigenous in situ environmental
monitors. We have developed the concept  throughout  this chapter  that the data
from short-term tests for genetic toxicity can be used to estimate genotoxic hazards
and risks to various organisms, including humans. Eventually, however, if we desire
to estimate risk to humans, we would like to use the human species as  the test orga-
nism, either  indirectly or  directly, in  human population monitoring. How do we
monitor human populations?  We will mention two main approaches that utilize
short-term tests: the use of human body fluids and human cells. In general, a specific
population known or suspected of being exposed to  genotoxic chemicals is com-
pared to a control (unexposed) population, selected to be as similar as possible to the
population under study. Often these populations are in a particular  occupation.
   A good example of the use of body fluids in human population monitoringcomes
from the work of Yamasaki and Ames (1977). They have shown that the urine of cig-
arette smokers is clearly mutagenic compared to the urine of nonsmokers, as assayed
in the Salmonella/microtome test. Epidemiologic studies have shown  that cigarette
smokers have a higher incidence of bladder cancer than nonsmokers. There are dis-
advantages of using urine  in human population monitoring, but many samples are
relatively easily obtained and tested. Another approach to human population moni-
toring is to use human cells. Blood is collected from individuals of the population un-
der study and from a control population, and lymphocytes are cultured in the labo-
ratory. Two important genetic end points that can be scored in these cells are SCEs
and chromosomal aberrations.
   One interesting aspect of human population monitoring is that there are individu-
als whose  genotypes  make them highly sensitive to mutagenic  agents in  their
environment. These individuals have a high risk of cancer. The best-understood ex-
ample of this is the disease xeroderma pigmentosum, which is due to an autosomal
recessive mutant allele. Affected individuals are highly sensitive to sunlight, develop
many skin cancers, and usually die of cancer before reproductive age.  Persons with
xeroderma pigmentosum  are  deficient in a nucleotide  excision-repair pathway,
which normally removes pyrimidine dimers  induced in DNA by the UV in sunlight
(McCormick and Maher,  1980). These persons are the human counterparts of the
uvrB mutants of Salmonella, which we discussed earlier. Other diseases are known
in which the risk of cancer is increased. Because the human population is genetically
highly heterogeneous, there  are probably other undiscovered cancer-prone geno-
types. One use of human  population  monitoring could  be to identify mutation-
prone individuals, who could be advised to adapt their lifestyles so as to minimize
their exposure to environmental mutagens.
                                    32

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      L-LU-1.1 I  I I  I 1
      0      5       10cm
(a)
(c)
Figure 6.   The use of the higher plant Tradescantia as an /ns/ru environmental monitor.
           (a) Normal stock plant of Tradescantia clone 4430 showing several mature
              inflorescences;
           (b) Enlarged view of a single inflorescence showing the range in bud size
              from the lowest buds containing meiotic stages to mature flower;
           (c) Enlargement of stamen hairs  with pink mutant events indicated by
              shading. Mutations in the flower color locus are not usually lethal. The
              chains of pink cells represent daughter cells of the initial mutated cell
              (from Schairer et a/., 1979).
                                     33

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Summary Comments on Short-Term Tests for  Genetic Toxicity
  One view of the use of short-term tests for genetic toxicity is that they are an im-
portant tool of preventive medicine. Through careful analysis of the data obtained,
one can identify genotoxic chemicals and chemical mixtures before they are put into
general use and released into the environment. For the chemicals already in our envi-
ronment, the tests can identify those that are genotoxic so that measures can be
taken to decrease their occurrence. Furthermore, for the chemicals that provide
great benefits to society, but which also are genotoxic, closely related structural
analogs can be synthesized that may retain the desired chemical property without be-
ing genotoxic. By striving to keep the number and concentration of genotoxic chem-
icals in our environment to the lowest levels possible, we hope to maintain the health-
iest environment possible.

FEDERAL LEGISLATION AND ACTIONS
   The Federal Government has recognized the threat that toxic substances pose to
the environment and to human health, and has responded by passing legislation that
governs the production, testing, formulation, use, and disposal of toxic substances.
The most  comprehensive act  concerning toxic  chemicals is the Toxic Substances
Control Act of 1976, which authorized the Administrator of the EPA to protect the
environment and the health of humans from harmful chemicals and chemical mix-
tures. The scope of this act was summarized by the EPA on October 7, 1976, as
follows:
     The Toxic Substances Control Act authorizes the EPA to obtain from in-
     dustry data on the production, use, health effects, and other matters con-
     cerning chemical substances and mixtures. If warranted, EPA may regulate
     the manufacture, processing, distribution in commerce, use, and disposal
     of a chemical substance or mixture. Pesticides, tobacco, nuclear material,
     firearms and ammunition, food, food additives, drugs, and cosmetics are
     exempted from the Act. These products are currently regulated under oth-
     er laws.
In this same document, EPA  comments about  the testing of chemicals:
     The Administrator of EPA may require manufacturers or processors of
     potentially harmful chemicals to conduct  tests  on the chemicals. Testing
     may be directed to evaluating the characteristics of a chemical such as per-
     sistence or acute toxicity, or to clarifying its health and environmental ef-
     fects,  including carcinogenic,  mutagenic,  behavioral, and synergistic ef-
     fects.  The manufacturers or processors  of a chemical must bear costs of
     testing that chemical.
   The Toxic Substances Control Act is very significant because it requires manufac-
turers and processors of chemicals and chemical mixtures to test them before they
are produced commercially. The emphasis of the Act is clearly on preventing toxic
substances from entering our environment.  The EPA recognized that an integral
part of implementation of the Toxic Substances Control Act would be short-term
tests for toxicity of the chemicals, and that short-term tests for genetic toxicity would
play an important role. Short-term tests for genetic toxicity have been used for over
50 years; therefore, a large and diverse scientific literature exists on the mutagenicity
of chemicals. There was a clear need for a mechanism to collect, organize, analyze,
and interpret all of these existing data, and to evaluate the relative merits of the many
different short-term tests. The Office of Toxic  Substances of the EPA established
Phase I of the "Gene-Tox Program" in 1979: an evaluation of the current status of
bioassays in genetic toxicology (Green and Auletta, 1980; United States Environ-
mental Protection Agency,  1980; Waters and Auletta, 1981). The evaluations were
presented at a meeting in Washington, D.C. in December 1980, and are being pub-
lished in the open literature (for example, Brusick et al., 1980; Generoso et al  1980)
                                     34

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  An Assessment Panel has appointed committees, which will evaluate the efficacy
of the various short-term tests to detect the major kinds of alterations in the genetic
material, mutagenic chemicals in various chemical classes, and somatic and germ
cell mutations. Based on their conclusions, certain short-term tests will be selected as
the  "best" combination to use in the future to detect mutagens and potential carcino-
gens.  The chemicals identified as genotoxic most likely will be subjected to short-
term tests for carcinogenicity, and the chemicals positive in short-term tests for car-
cinogenicity most likely will be validated in rodent cancer tests. From the informa-
tion obtained from these tests, along with other information, the EPA will evaluate
the  potential hazard and risk that a particular chemical poses to the environment
and the organisms in  it.

THE FUTURE OF GENETIC TOXICOLOGY
  We expect that the existing short-term tests for genetic toxicity will continue to be
improved and that new tests will be devised. These new and improved tests will be in-
creasingly validated for various classes of chemicals that are known carcinogens or
noncarcinogens, and that are  known mutagens or nonmutagens in germ cells of
mammals. In the last decade, the question of the mutagenicity of known carcinogens
and noncarcinogens has  been considered fairly extensively; we anticipate a major
emphasis on study of the reverse association. As chemicals not yet tested for carcino-
genicity are identified  as  mutagens or nonmutagens in short-term tests, they will be
studied for carcinogenicity.
  Most of our exposure to  mutagenic chemicals is not to pure compounds. Rather,
we are exposed to highly complex mixtures of chemicals, as for example, in air, wa-
ter, and food. Therefore,  we expect that there will be a greater emphasis on the study
of complex chemical mixtures, environmental  monitoring, and human population
monitoring. Because of the large number of samples, environments, and populations
that need to be surveyed, short-term tests for genetic toxicity are expected to have a
major role. Methods for environmental and human population monitoring are in an
early stage of development, and we expect an explosion of new techniques and find-
ings in these areas. Because the human population is highly heterogeneous geneti-
cally, we anticipate an emphasis on studies that seek to identify the genetic factors
that determine the relative sensitivity of individuals to environmental mutagens.
  Recent discoveries on the molecular biology of the genetic material—such as the
organization of nucleotide sequences in eukaryotic DNA, the organization of DNA
and proteins into nucleosome structures, the presence of intervening  sequences in
eukaryotic genes, and  the mechanism of peripatetic genes, which move from place to
place in the genome—must be considered in the design of experiments and in the in-
terpretation of the results from short-term tests for genetic toxicity. The knowledge
from  basic research in many fields of science will continue to have major influences
on the field of genetic toxicology as short-term  tests become increasingly important
in the evaluation of the risk that toxic substances pose to our environment and to our
health in this and future generations.
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McCormick, J.J., and J. M. Maher. DNA repair processes can alter the frequency of mutations
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Mendel, G. Versuch iiber Pflanzen-hybriden (Experiments on Plant Hybrids). Verh. Naturforsch.
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           SHORT-TERM  IN VITRO TESTS FOR

                IDENTIFYING CARCINOGENS:

          TRANSFORMATION OF  MAMMALIAN

                       CELLS IN CULTURE*

                           Arthur C. Upton


INTRODUCTION
   Since the pioneering efforts of Sachs and his coworkers, who showed that mam-
malian cells in culture can be transformed reproducibly by chemicals and X rays
(Berwald and Sachs,  1963, 1965; Borek and Sachs, 1966), the transformation of cells
in vitro has become one of the most productive approaches in research on carcino-
genesis. A number of systems for studying the transformation of cells in culture have
been developed (see reviews by DiPaolo and Casto, 1977; Borek, 1979; Casto, 1979;
DiPaolo,  1979; Heidelberger and  Mondal, 1979; Krahn, 1979; Pienta, 1979, 1980;
Sivak, 1979; Fisher and Weinstein, 1981).
   The development of methods for studying the in vitro transformation of cells rep-
resents a major advance. It enables cellular and subcellular aspects of carcinogenesis
to be investigated directly in ways that are not feasible in the whole animal.  It also
provides promising approaches for developing short-term tests for carcinogens, pro-
moting  agents, and cocarcinogens. It is thus of enormous potential importance to
those concerned with the detection and characterization of cancer-causing agents in
the environment.

HISTORICAL PERSPECTIVE
   The cultivation of mammalian cells in vitro goes back more than half a century,
but it has  not yet become possible to grow every type of cell in long-term culture.
Even cancer cells can seldom be propagated  for more than a short time after their
removal from the body.  Those cells which adapt most readily to culture, tending to
outgrow other elements, are mesenchymal derivatives which are presumably derived
from blood vessels. Until recently most of the studies of cellular transformation have
dealt  with such so-called fibroblasts (DiPaolo and Casto,  1977).
   One noteworthy characteristic of these cells is that they tend to grow in an orderly
pattern and to stop multiplying after they have formed a continuous sheet over the
floor of the culture vessel. When  detached, disaggregated and transferred into fresh
medium, they start multiplying again. The process by which cell division is normally
arrested after the cells  have  become confluent is known as density-dependent
inhibition. Although the  inhibition clearly denotes some form of cell-to-cell interac-
tion, its  precise  mechanism  remains to be determined.

*This manuscript was prepared under support by Grant No. ES00260 from the National Insti-
 tute of Environmental Health Sciences and Grant No. CA13343 from the National Cancer
 Institute.
The Author Arthur C. Upton is a faculty member of the Institute of Environmental Medicine,
New York University School of Medicine, New York,  N.Y.
                                    38

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  Another property of normal cells is their limited proliferative potential. Most ex-
plants of human fibroblasts die out after the cells have divided about 50 times, de-
pending on the age of the donor. It is only in rare instances that a normal cell sponta-
neously undergoes a change in culture that enables it to multiply indefinitely, giving
rise to a permanent cell line. This change is usually, if not always, accompanied by
chromosomal abnormality,  implying that it may signify a mutational alteration
(Hayflick, 1977).
  Along with the change in proliferative potential, transformed cells may show alter-
ations in size,  shape, staining properties, patterns of growth, and  other features,
including acquisition of the capacity to form tumors when inoculated into appropri-
ate test animals (Fig. 1, Table 1). On acquiring these properties, transformed cells ex-
hibit many of the characteristics of cancer cells. Hence, the process of cell transfor-
mation in vitro is analagous in certain respects to carcinogenesis in vivo.
  The first spontaneous neoplastic transformations  of cultured mammalian cells
were observed  in rat and mouse cells, the frequency of transformation being
appreciably higher in these species than in the guinea pig, hamster, or human. Since
the conditions under which cells of a given species are cultured may also influence
their proliferative behavior, including their rate  of  spontaneous transformation
(Sanford, 1967;  DiPaolo and Casto, 1977), the methods and criteria for assaying
transformation depend on the cells in question and the conditions under which they
are grown.

MAJOR ADVANCES IN THE PAST DECADE

Development of Cell  Transformation Assays
  Of the various properties of transformed cells (Table 1), the ability to produce tu-
mors in vivo is the ultimate test of neoplasia. Since this property is  not necessarily
associated with each of the other changes tabulated, efforts have been made to evalu-
ate different characteristics of transformed cells for their reliability as indicators of
tumorigenicity. In addition to being reliably indicative of neoplasia, the "ideal" assay
system should, of course, be reproducible, sensitive, predictive for all types of cells
(such as epithelial cells, fibroblasts), rapid, practical,  and quantitative (Steuer and
Ting, 1977).

Colony Assay—
  This approach, utilized in the initial studies made by Sachs and coworkers, con-
sists of seeding cells at low concentrations onto a "feeder" layer of cells previously
rendered incapable of dividing by exposure to X rays or the mitotic inhibitor, mito-
mycin-C. The cultures, usually of embryo fibroblasts, are then treated with graded
doses of the agent to be tested, incubated for one to two weeks, and scored (Fig.  2).
Colonies formed by  transformed cells are distinguished from colonies of normal
cells by the way in which the transformed cells pile up  into "hay stack,''or randomly
oriented, criss-cross patterns, and by changes in their size, shape, and staining prop-
erties (Fig. 3).
  One of the chief problems with the colony assay has been its variability. Sources of
variability include differences in the responsiveness of cells from different embryos
of the same species; the subjectiveness of the scoring system, which requires evalua-
tion of the growth patterns of the different kinds of cells in a culture of the whole em-
bryo;  variations among  batches  of calf serum (an  essential component of the
medium) in its ability to support the growth of newly transformed cells; and changes
in transformation frequency associated with storage of media (DiPaolo and Casto,
1977; Pienta, 1979;Sivak, 1979;  Casto and Carver, 1980). Although these difficulties
have yet to be eliminated, noteworthy progress has been made in efforts to standard-
ize the procedure through the use of pooled cryopreserved cells, meticulous attention
to technical details, and various types of control measures (Pienta, 1979).
                                     39

-------
                Initial cultivation
               of mouse fibroblasts
 Nonmultiplying
 nontumorigenic
   (anchorage-
 dependent) cells
                                                    The appearance of colonies
                                                        of transformed cells
                                           Multiplying, tumorigenic
                                           (anchorage-independent)
                                                    cells
                                                         Tumorgenicity
Figure 1.   Neoplastic transformation of mouse cells in culture. The transformed cells
          form piled-up colonies, are capable of multiplying in semisolid suspension,
          and form progressively growing tumors when inoculated into mice.
          From Cancer: Science and Society by John Cairns. W.H. Freeman and
          Company. Copyright 1978.
  A wide variety of agents have been tested with this method, largely in newly ex-
planted or early passage Golden Syrian hamster embryo cells (DiPaolo and Casto,
1977; Pienta,  1979; Casto and Carver, 1980). In the largest series of assays sum-
marized to date, comprising I 10 chemicals of various classes, a 98 percent corre-
lation was observed between transformation in vitro and carcinogenicity in vivo (Ta-
ble  2).  No false positives were obtained with the transformation test in contrast to
the  Salmonella  mutagenicity test; however, a small percentage of false neeatives
were obtained with both tests. The individual chemicals tested and the comparisons
are  given in Appendix 1                                           Comparisons
                                     40

-------
Table 1. Some Properties of Transformed Cells.
Ability to form tumors in test animals
Ability to proliferate indefinitely ("immortalization")
Ability to form multilayered clones
Ability to grow in semisolid suspension (loss of anchorage dependence)
Reduced serum  requirement
Increased saturation density
Increased rate of proliferation
Increased cloning efficiency
Morphologic changes (size, shape, nucleo-cytoplasmic ratio, staining, cell junctions,
 loss of cell-to-cell  orientation)
Altered membrane properties (lectin build-up, agglutination)
Altered antigenic specificity
Increased fibrinolytic activity
Increased uptake of nutrients
Increased glycolysis
Release of tumor angiogenesis factor
Destruction by leukocytes
Destruction by normal cell lysates


(From Martin and Anderson,  1976; DiPalo and Casto, 1977; Steuer and Ting, 1977;
Casto and Carver, 1980).


   Certain chemicals fail to transform embryo fibroblasts unless hamster liver micro-
somal homogenates (so-called S-9 homogenates), or hepatocyte feeder layers, are
added to the cultures.  This is because of inadequate  metabolic activation  of the
chemicals by hamster embryo fibroblasts alone, which lack the activating capability
in their microsomes. An alternative ("host-mediated") method for enhancing the ac-
tivation of such precarcinogens is to expose the embryo cells in utero, by inoculating
the chemicals into mid-term pregnant hamsters two to three days before the embryo
cells are explanted (DiPaolo et al.,  1973).
   Advantages of this assay system, as compared with others, include the facts that
the frequency of spontaneous transformation in hamster embryo cells is virtually nil,
the transformation of such cells has been observed to follow one-hit kinetics, and the
test results can  be scored within seven to ten days. Moreover, the transformed cells
have been found consistently to be tumorigenic on inoculation into young hamsters
(DiPaolo and Casto, 1977; Casto and Carver, 1980).

Focus Assay—
   Cells, seeded at a relatively high density into culture medium without any feeder
layer, are allowed to grow to confluence and scored for transformation by the pres-
ence of multilayered, randomly oriented foci of dense, piled-up growth. The assay
ordinarily uses  cells of permanent lines,  which  are aneuploid and  require several
weeks to score, in contrast  to the colony assay which typically uses diploid cells
which require only one to two weeks to score. Cells transformed in this system can
show the full spectrum of changes associated with transformation (Reznikoff et al.,
1973).
   Various focus assay systems have been developed thus far, including those using
C3H  mouse fibroblasts,  BALB/3T3 mouse embryo fibroblasts, C3H/10T 1/2
mouse embryo fibroblasts, rat embryo cells, rat embryo cells expressing an endoge-
nous oncorna virus, hamster BHK-21 cells, guinea pig embryo cells, and human skin
fibroblast KD  cells (Kakunaga,  1977;  Heidelberger  and Mondal, 1979; Borek,
1980a). A number of focus assay systems exploit the ability of carcinogenic agents to
                                     41

-------
Day 0

Reconstitute frozen standard
   feeder cells from liquid
nitrogen. Refeed after 8 hours.

Day 2

Subculture the feeder cells.


Day 3

Refeed the feeder cells.


Day 4

 X-lrradiate feeder cells with
5000R, then seed only 50 mm
dishes at 8 x 104 cells/dish.
Reconstitute target cells and
    refeed 6 hours later.


Day 5

Seed target  cells onto feeder
 layer cells at 500 cells/dish.


Day  6

 Add chemicals and incubate
 dishes without disturbing
 or refeeding.


Day  14

Fix with methanol, stain with
Giemsa, and monitor dishes
for transformation.
 Figure 2.   Outline of procedure of standard colony assay for in vitro transformation of
           Golden Syrian hamster embryo cells (adapted from Sivak, 1979).


 cause a dose-dependent enhancement of virus-induced transformation in cultured
 cells (DiPaolo and Casto, 1977; Sivak, 1979).
   The focus assay method has been applied to a wide variety of chemicals, with re-
 sults (Appendix 2) that are comparable, in general, with those obtained with the col-
 ony assay method. However, the aneuploid cells usually used in the focus assay have
 a higher rate of spontaneous transformation than the diploid cells used in the colony
 assay, the number of additional  transformed  foci  produced by a given dose of
 carcinogen is  inversely related to  the number of cells plated, and it is not certain
 whether each focus arises from a single transformed cell. Hence the rate of transfor-
 mation induced in the focus method cannot be calculated precisely for comparison
 with that in the colony method. Nevertheless, the focus method has certain advan-
 tages,  including higher cloning efficiency and  greater availability  of cloned non-
 transformed cells for comparative studies.
                                      42

-------
               Normal
                                                    Transformed
Figure 3.  Schematic illustration of the microscopic appearance of transformed
          fibroblasts as compared with normal fibroblasts. The normal fibroblasts grow
          in an orderly orientation, forming a single layer of relatively uniform,
          confluent eel Is, whereas the transformed fibroblasts vary in size, shape, and
          staining intensity, piling up on one another in a randomly oriented, criss-
          cross pattern. Reprinted from Pilot (1978) by courtesy of Marcel Dekker.


Colony Formation in  Soft Agar—
  Following the observations by MacPherson and  Montagnier (1964) that baby
hamster kidney (BHK) cells acquire the ability to form colonies in  soft agar after
transformation by the polyoma virus, a number of investigators have utilized growth
in semisolid suspension as a measure of cellular transformation (DiPaolo and Casto,
1977; Purchase et al., 1978; Bouck and Di Mayorca, 1980). This property, which de-
notes loss of anchorage dependence, is closely correlated with tumorigenicity in ani-
mals (Steuer and  Ting, 1977).
  In this assay method, cells are incubated in the presence of the agent to be tested,
subsequently resuspended in culture medium containing 0.3-0.4 percent agar, seeded
onto an agar base layer containing nutrients, incubated for one to three weeks, and
then  scored for  colony formation. Because  normal  cells cannot grow unless
anchored to a substratum, the culture conditions select for transformed cells. The in-
clusion of a rat liver microsomal preparation in the culture medium enhances meta-
bolic activation of the chemical to be tested, generally increasing colony formation
(Styles, 1978).
  In a comparative study of 120 chemicals of different classes, the method accurate-
ly identified 53 out of 58 carcinogens and 60 out of 62 noncarcinogens (Appendix 3).
Results obtained with human cells of the WI-38 and Chang lines were almost as good
as those obtained with hamster (BHK-21) cells, in spite of the fact that the frequency
of transformation was approximately 10 times lower in the human cells (Purchase et
al.,  1978). The method compared favorably with any of five short-term tests utilizing
end points other than in vitro transformation (Table 3).
                                     43

-------
 Table 2.   Comparative Effects of Different Classes of Chemicals with Respect to Transformation of Hamster Embryo Cells In
            Vitro. Mutagenicity in Salmonella, and Carcinogenicity In Vivo.*
Clinical Class
Direct alkylating agents
Polycyclic hydrocarbons
and derivatives
Nitrosamines and amides
Aromatic amines and
aminoazo dyes
Metal salts
Solvents
Miscellaneous compounds
Totals
Carcino-
genicity Number
in wVof Tested
+ 10
1
+ 7
5
+ 6
1
+ 27
8
+ 4
6
+ 19
14
+ 73
35
Cell Transformation In Vitro
Concurrence^
10/10
1/1
7/7
5/5
5/6
1/1
26/27
8/8
4/4
6/6
18/19
14/14
70/73
35/35
False
Negatives
0
0
1
1
0
-
1
(96%) 3
(100%) 0
False
Positives
0
0
0
0
-
0
0
0
Mutagenicity in Salmonella
Concurrence§
10/10
1/1
7/7
4/5?
6/6
1/1?
25/27
6/8
4/4?
6/6?
6/19
13/14?
58/73?
30/35?
False
Negatives!
0
0
0
2
4?
-
13
19?
False
Positives!
0
1
1?
2
-
1?
3?
7?
*From Pienta (1979) (Values tabulated do not include results on two nitrosamines of undetermined carcinogenicity).
f+ = positive result, - = negative result.
^Values include results obtained when hamster liver S-9 homogenate was added to the culture system.
§Question marks denote the occurrence of one or more equivocal results among the values tabulated.

-------
Table 3.   Results with the In Vitro Transformation Assay (Colony Formation in Soft Agar), as Compared with Five Other Short-
          Term Tests for Carcinogenicity.*
Compounds Identified
Chemical Class

Polycyclics
Carcinogenic
Noncarcinogenic
Arylamines
Carcinogenic
Noncarcinogenic
Alkylating Agents
Carcinogenic
Noncarcinogenic
Miscellaneous
Carinogenic
Noncarcinogenic
Total All Classes
Carcinogenic
Noncarcinogenic
Number
Tested


11
9

20
13

18
0

9
40

58
62
Cell
Transformation
(Nos.)

10/11
9/9

19/20
13/13

17/18
-

7/9
38/40

53/58
60/62
<%>

91
100

95
100

94
-

78
95

99
97
Salmonella
Mutation
(%)

100
89

95
92

83
-

89
95

91
94
Correctly
Degranu-
lation
(%)

73
55

95
46

61
-

33
82

71
71

Sebaceous
Gland
Suppression
(%)

100
78

55
75

67
-

55
57

67
64

Tetrazolium
Reduction
!%>

45
55

40
83

39
-

33
74

40
73

Implant
(%)

75
100

39
92

25
-

22
95

37
95
*Based on data in Appendix 3 (Purchase et al., 1978).

-------
Mass Culture—
  Confluent or subconfluent cells are incubated in the presence of the agent to be
tested, subsequently  transferred for several passages at high density (~50,000
cells/cm2), and then  observed  for changes  indicative of transformation  such as
random orientation, criss-cross  growth, ability to replicate at high density, ability to
grow at reduced serum concentrations and increased glycolysis (Table 1).
  The method shown in Fig. 4 entails repeated subculturing of cells over compara-
tively long periods of time (weeks or months) and cannot be used to determine the
precise frequency of transformation, but its use of increased numbers of cells at risk
enchances  its sensitivity to detect weak  carcinogens (Casto and Carver, 1980).

Epithelial Cell Culture  Systems
  Although most cell cultures have thus  far dealt with fibroblasts (Table 4), the ma-
jority of human cancers are epithelial in origin. Hence, culture systems for studying
epithelial cell transformation have been sought for years. The systems now under
most active study include cultures of liver cells (Borek,  19806; Montesano et al.,
1975; Williams, 1976; Weinstein et al., 1975), epidermal cells (Fusenig et al., 1973;
Colburn et al., 1978; Lillehaug et al., 1979), salivary gland cells (Brown,  1973), uri-
nary bladder epithelium (Hashimoto, 1978) and respiratory tract epithelium (Marchok
and Nettesheim, 1977; Steele et al., 1978).
  To date, experience with these systems remains largely exploratory.  None  have
developed far enough as yet to  be evaluated as a potential assay method (DiPaolo
and Casto, 1977; Sivak, 1979; Casto and Carver, 1980). The phenotypic markers of
the transformed state which are  characteristic of fibroblasts in vitro (Table 1) are not
as readily distinguishable in cultured epithelial cells. Ability to form colonies in semi-
solid suspension is currently the most reliable and consistent criterion of the epitheli-
al cell transformation, although capacity to produce progressively growing tumors
in animals constitutes the final proof of malignancy (Borek, 19806).

Organ Culture Systems
  The conditions under which cells are ordinarily cultivated in vitro—where cells of
a single type are propagated—differ profoundly from those in vivo, where growth
and differentiation involve complex interactions between mutually interdependent
cells of different types in organs and tissues. To investigate the transformation of
cells under conditions more closely resembling those in vivo, efforts have been made
to develop culture systems for various organs. Those currently under investigation
include organ culture systems for tissues of the respiratory tract, colon, and pancre-
atic duct (Saffiotti and Harris,  1979).
  Among the potential advantages of the organ culture approach are (1) the ability
to preserve the morphological and functional integrity of tissues for long periods, in-
cluding intercellular and epithelial-stromal  relationships; (2)  the ability to  study
effects of  carcinogens on  the tissue as a whole, and to compare the reactions of
component cell types concurrently; (3) the ability to use a tissue as an experimental
system in which to study the influence of microenvironmental changes; (4) the ability
to explore the process by which carcinogens are activated and detoxified in tissues,
during which their interactions  with tissue elements can be localized by autoradio-
graphic, biochemical, and biophysical techniques; (5) the ability to study the effects
of carcinogens at different levels of biological organization, for example at the level
of the whole tissue, the epithelium, and stroma, individual cell types, cellular organ-
elles, and molecular components; (6) the ability to observe histological tissue reac-
tions  preceding neoplasia, such as hyperplasia, metaplasia, and dysplasia; (7) the
ability to transplant the tissue, after its in vitro manipulation, into suitable recipient
animals for further observation  in vivo; (8) the ability to use the tissue as a source of
cell monolayer cultures; and (9) the ability to use human tissue in organ culture for
evaluating species  differences  in  responses  to  carcinogens, and  to compare


                                     46

-------
Day 0

Seed target cells inyo 25 cm2
flasks at 7 x 10s cell/flask.

Day 2

Refeed cells and add chemical

Day 9

Refeed cells and add chemical.
Day 16
Wash cells with BSS; split
into 3 new flasks.
Day 21

Refeed flasks.


Day 26

Split one culture into 3 new
flasks. Fix one flask with
methanol and stain with
Giemsa. Refeed one culture
and hold as backup.
Hold      Stain        Subculture
                           »
                 Continue passaging until
                 (1) focus formation is
                     observed in treated
                     cultures, or
                 (2) Cultures  have been
                     passaged 14 times
                     after treatment.
  Figure 4.  Outline of procedure of mass culture assay for in vitro transformation
           of Rauscher leukemia virus-infected Fischer rat embryo cells, (adapted
           from Sivak, 1979).
experimentally induced responses in human tissue with those that occur naturally
(Saffiotti and Harris, 1979).
  While the potential long-range promise of the organ culture approach as a research
tool should not be underestimated, it has not yet developed sufficiently to provide a
practical assay method at this time.

Assays for Cocarcinogens, Promoting Agents, and Other Modifying Factors
  Because of the multicausal, multistage nature of the cancer process, short-term as-
says for cocarcinogens and promoting factors are needed, as well as assays for initi-
ating agents and complete carcinogens. It is noteworthy, therefore, that in vitro cell
transformation systems have recently been shown to be applicable to the study of a
growing number and diversity of incomplete carcinogens and factors that modify the
                                     47

-------
Table 4.  Cell Culture Systems Investigated for Assaying the Carcinogenic
          Potential of Chemicals.*
       Type of System
  Assay Method
Scoririg Time
A.  Primary or Secondary
    Embryo Cultures
    1.  Syrian Hamster
    2.  Guinea Pig
    3.  Rat
    4.  Rat or Mouse
       infected with murine
       leukemia virus
    5.  Rat infected
       with adenovirus
    6.  Syrian Hamster
       infected with adenovirus

B.  Fibroblast Cultures
    1.  Human Skin Fibroblasts

C.  Fibroblast Cell Lines
       Mouse Prostate (Adult)
       Mouse 3T3t
       Mouse 10T1/2 t
       Syrian Hamster BHK21 §
       Rat Embryo
       Cell Line
       (Fischer rat embryo  )
Colony
Growth in Agar
Mass culture—Focus
Mass culture—Focus
Mass culture—Focus

Focus



Mass culture—Focus
Focus
Focus
Focus
Growth in Agar
Mass culture—Focus
8-10 days
4 months
130 weeks
3-8 weeks
25-42 days

25-30 days



7-14 weeks
4-6 weeks
10-28 weeks
4-6 weeks
2-4 weeks
40-43 weeks
). Epithelial Cell Cultures
1 . Rat Liver
2. Mouse Epidermis
Growth in Agar
Growth in Agar
8-36 weeks
12-16 weeks
 "From'Casto (1979), Fisher and Weinstein (1981).
 fCell line established from BALB mouse embryos.
 JCell line established from C3H  mouse embryos.
 §Established from newborn Syrian hamster kidneys.
 || Transformation occurred in later passage cultures treated with carcinogens.
 process of carcinogenesis. Furthermore, since promoting agents have thus far been
 distinguished from initiating agents, cocarcinogens, and complete carcinogens large-
 ly on the basis of operational definitions (Table 5), the availability of culture meth-
 ods for identifying such agents and studying their mechanisms of action is of scientif-
 ic, as well  as practical, importance.
   Based on the available evidence, the transformation of cells in vitro, like carcino-
 genesis in vivo, has been inferred to be  a multistage  process (Heidelberger and
 Mondal, 1979; Weinstein et al., 1979; Poiley et al.,  1979). The tumor-promoting
 agent, 12-0-tetradecanoyl-phorbol-13 acetate (TPA), among other agents, has been
 observed to enhance the transformation of certain cells in vitro by various chemicals
 (Heidelberger  et al., 1978; Colburn et al., 1978; Weinstein et al.,  1978), ultraviolet
 light (Mondal and Heidelberger, 1976), X rays (Kennedy et al., 1978), and viruses
 (Weinstein et al., 1978). Its transforming effects in vitro are associated with many of
 the biochemical alterations that TPA produces in vivo (Weinstein et al., 1978), and
                                      48

-------
Table 5.   Biological Properties of Promoting Agents, as Compared with
           Initiating Agents.
 	Promoting Agents	Initiating Agents	

1.  Not carcinogenic alone-must be pre-     1.  Carcinogenic by themselves--"solitary
    ceded by an initiating agent               carcinogens"
2.  Require  prolonged exposure(s)          2.  Single exposure is sufficient
3.  Action is reversible (at early stage)      3.  Action is relatively irreversible and
    and not  additive                          additive
4.  Probable threshold                     4.  No apparent threshold
5.  No evidence of covalent binding         5.  Yield electrophiles that bind cova-
                                            lently to cell macromolecules
6.  Not mutagenic                        6.  Mutagenic
(From Weinstein et al., 1978).

the effects are elicited at low concentration (in the range of 10 9M) in cells of diverse
species and types. Not all types of cells respond similarly, however, nor is the re-
sponse and its modification by cofactors the same in vitro as in vivo (Fusenig and
Samsel,  1978; Weinstein et al., 1978).
   In view of the diversity of factors influencing the growth and transformation of
cells in vitro, and the fragmentary state of present knowledge about the dynamics of
cell regulation, much further research will be necessary before practical in vitro assay
systems for cocarcinogens and promoting agents will become available.

Comparative Results with  Different Assay Systems
   Most types of chemical carcinogens have been found to transform mammalian
cells in vitro under suitable test conditions, although some  fail to do so in certain as-
say regimens because of inadequate metabolic activation  in vitro. The addition of
liver microsomal extracts or an hepatocyte feeder layer has corrected this deficiency
in many such instances. The absence of transformation in vitro with other agents (for
instance, croton oil and asbestos) may signify that their carcinogenic activity in vivo
is mediated through promoting effects or effects on posttransformational stages in
the cancer process.
   In general, in vitro transformation  assays have not thus far materially outper-
formed the Ames test or certain hepatocyte DNA repair tests (Martin et  al., 1978;
Williams, 1979) in the percentage of carcinogens identified, but they have tended to
yield fewer false positives (Tables  2  and 3). Furthermore, as noted above, in vitro
transformation  systems  are   potentially  able to  identify  cocarcinogens and
promoting agents of  types that are not detectable by mutagenicity or DNA repair
tests. In vitro transformation tests have also identified correctly an appreciably high-
er percentage of carcinogens than  has been identified by degranulation of rat liver
endoplasmic reticulum, reduction  of terazolium red in mouse skin,  suppression of
sebaceous glands in mouse skin, histologic reaction to chemically impregnated filter
discs  implanted subcutaneously in  mice  (Table 3),  or other short-term tests in-
vestigated  to  date, including transplacental  blastomagenesis, piperidine  alkyla-
tion, and the iodine and acridine tests (Purchase et al., 1978).
   A combination of complementary tests can, obviously,  be expected to identify a
higher percentage of carcinogens than any one test alone; however, it may also yield
a higher percentage of false positives (Table 6). An optimal balance between false
negatives and false positives among the test results tabulated (Table 3) is achieved by
combining the results of only two of the six tests: the cell transformation test and the
                                      49

-------
Table 6.   Comparative Results of Short-Term Tests When Used Alone or in
           Combinations.*




Testf
CT
BM
Deg
SI
SG
TR
CT+BM
CT+BM+Deg
CT+BM+SI
CT+BM+Deg+SI
CT+BM+Deg+SG
CT+BM+SI+SG
CT+BM+Deg+SI+SG
CT+BM+Deg+SG+TR
CT+BM+SI+TR
All 6 Tests
% of
Carcinogens
Which Would be
Positive in at
Least One TestJ
91
91
71
37
67
40
99.19
99.77
99.49
99.85
99.92
99.83
99.95
99.95
99.90
99.97
%of
Non-carcinogens
Which Would be
Positive in at
Least One Test:):
3
6
29
5
36
27
8.8
35.3
13.4
38.5
58.6
44.6
60.6
69.8
59.5
71.33
% of
Errors
(False Negatives
plus
False Positives)
12
15
58
68
69
87
9.61
35.53
13.91
38.65
58.68
44.77
60.65
69.85
59.60
71.36
 *From Purchase et al. (1978), based on the results in Appendix 3.
 tCT = cell transformation; BM = bacterial mutation; Deg = degranulation; SI = subcutane-
 ous  implants; SG = sebaceous gland; TR = 1  tetrazolium reduction.
 |The  number of decimal places does not represent the likely accuracy of the figures but
 demonstrates the likely size of the differences between them.
Salmonella mutation test, which together yield a total error rate (false negatives plus
false positives) of only 9.61 percent (Table 6).
  The relative importance of false negatives, as compared with false positives, can be
expected to vary, depending on the ratio of carcinogens to noncarcinogens among
the compounds to  be tested. The effects  of  variations in this ratio, analyzed by
Purchase et al. (1978) on the basis of the data summarized in Table 3, are shown in
Figs. 5 and 6. It is evident from this analysis that the transformation test and the bac-
terial mutation test  can complement and reinforce each other (Fig. 5) but that one
test adds little to the reliability of the other when the results of the two are contradic-
tory (Fig. 6). The reliability of the two tests has been analyzed further on the basis of
three performance criteria suggested by Cooper et al. (1979) as (I) "sensitivity,"or
the proportion of known carcinogens that can be  identified correctly; (2) "specific-
ity," or the proportion of known  noncarcinogens that can be identfied correctly;
and (3) "predictive value," or the proportion of chemicals giving positive results that
are actually carcinogens (that is, the proportion of positives that are true positives).
When judged on the basis of these performance criteria, appropriate use of the two
tests in combination has been calculated to yield results of relatively high reliability
(Table 7). Since, however, the ability of the Salmonella mutagenicity test to identify
carcinogens varies appreciably  among different classes of chemicals (Rinkus and
Legator, 1979), the  figures shown  in Table 7  must be interpreted accordingly
                                     50

-------
     1.0-t
                                                               I
                       20           40           60           80
                    Percentage of carcinogens in the sample tested (p)
100
Figure 5.   Variation in the probability that a compound will be correctly identified as a
           carcinogen (true positive result) by the combined use of the in vitro
           transformation test and the Salmonella mutagenicity test, in relation to the
           percentage of carcinogens among the samples being tested (from Purchase
           eta/.. 1978).

          Each curve represents a particular test result: (a) bacterial mutation and cell
          transformation positive; (b) cell transformation positive; (c) bacterial
          mutation positive; (d) bacterial mutation negative and cell transformation
          positive; (e) bacterial mutation positive and cell transformation negative;
          (f) bacterial mutation negative; (g) cell transformation  negative; (h) bacterial
          mutation and cell transformation negative. The curves are calculated from
          the formula:
                                             PA
                             P = •
                                         pA
          where P is the probability that a compound producing a particular test result
          is a carcinogen, p is the percentage of carcinogens in the samples being
          tested, A is the probability of obtaining the test result with carcinogens, and
          B is the probability of obtaining the test result with non-carcinogens.
          The values for A and B were obtained from Appendix 3.
                                       51

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     1.0 —k
     0.8 —
     0.6 —
     0.4 —
     0.2 —
                  Percentage of carcinogens in the sample tested (p)
Figure 6.  Variation in the probability of true positive and false positive results provided
          by the combined use of discordant results, from the in vitro transformation
          test and the Salmonella mutagenicity test,  in relation to the percentage of
          carcinogens among the samples being tested (from Purchase ef a/., 1978).

          The curves d and e (true positives) are the same as in Figure 5. The false
          positives, shown in curve dd (Salmonella mutation test negative, in vitro
          transformation test positive) and ee (Salmonella mutation test positive,
          in vitro transformation test negative) were calculated from the formula:
                       P =
                                   pA + (1-p)B

          where P is the probability that a compound producing a particular test result
          is a carcinogen, p is the percentage  of carcinogens in the samples being
          tested, A is the probability of obtaining the test result with carcinogens  and
          B is the probability of obtaining the test result with non-carcinogens '
          The values for A and B were obtained from Appendix  3.
                                     52

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Table 7.  Comparative Reliability of Results from In Vitro Transformation
          Test and Salmonella Mutation Test, used Alone or in Combina-
          tion, on Various Classes of Chemicals.*
                                         Performance Criterionf
 	Test	Sensitivity	Specificity	Predictive Valuej

                                       (accuracy in percent)
In Vitro Transformation           91              97                 77
Salmonella Mutation (Ames)      91              94                 63
Both Tests Combined
  1. When both agree            96             100               100
  2. When a positive result
     in either test is
     considered positive          99              91                 55
*From Purchase (1980), based on results in Table 3.
fSee text for definition of terms.
^Values denote predictive value for a positive test when carcinogens comprise ten per-
 cent of compounds being tested.
IMPLICATIONS   OF  ENVIRONMENTAL  LEGISLATION  AND
REGULATORY ACTIONS
  During the past 25 years cancer has come to be viewed increasingly as a multicaus-
al disease in which environmental carcinogens play a significant etiologic role (Doll,
1978; Higginson, 1979). This concept, along with growing evidence that tumor-
forming  activity in laboratory animals can be  predictive of carcinogenicity for
humans has prompted regulatory agencies to make increasing use of experimental
evidence as a  basis for determining whether a substance may pose a risk of cancer to
man. Thus, in the Food Additives Amendment of 1958, the Delaney Clause prohib-
ited  approval of any food additive that causes cancer in test animals on ingestion.
This clause has been construed by the Food and Drug Administration to require dis-
approval of the addition of a substance to food in any amount if it has been identified
as a carcinogen through appropriate animal test (Hutt, 1978).
  In the years since 1958, legislation conferring regulatory authority for carcinogens
has increased greatly in scope and complexity. Partly in an effort to avoid inconsist-
ency in regulatory policies on carcinogens, several agencies together formed the In-
teragency Regulatory Liaison Group (IRLG) in 1977  and two years later issued a
common policy statement setting forth the types of evidence on which carcinogens
are to be identified, the scientific bases for evaluating the evidence, and methods for
assessing risks to human populations (IRLG, 1979). With respect to short-term tests
for carcinogenicity, the IRLG Report (1979) concluded that in  vitro mammalian
transformation systems are useful in exploring the mechanisms of carcinogenesis, in
screening suspect agents for further study, and in supporting evidence derived from
animal or epidemiological investigations, but that they are insufficient by themselves
to establish the carcinogenicity of an agent  for humans or experimental animals.
These conclusions are in accord with those of the National Cancer Advisory Board's
Subcommittee on Environmental Carcinogenesis (1977), and with preliminary re-
sults of a three-year international collaborative study to evaluate in vitro cell trans-
formation among 30 short-term assay systems for their ability to identify 42 coded
                                     53

-------
carcinogens and noncarcinogens (National Toxicology Program, Bulletin, 1980). In
view of the importance of these reports for short-term tests, their conclusions are
given in full in Appendix 4.
  Although insufficient by themselves to establish the carcinogenicity of a substance,
short-term tests assumed increased importance as indicators of presumptive risk to
humans with the passage of Public Law 95-622, in 1978. This law requires the Secre-
tary of the Department of Health and Human Services to publish annually a report
containing "..  .a list of all substances (i) which either are known to be carcinogens or
which may reasonably be anticipated to be carcinogens and (ii) to which a significant
number of persons residing in the United States are exposed...." Compliance with
this mandate will inevitably focus increasing attention on the significance of data
from short-term tests in the assessment of cancer risks and in public policy directed
toward the control of environmental carcinogens.

OUTLOOK  FOR THE FUTURE
  In vitro cell  transformation systems have only recently come under serious study
as potential short-term assay methods for detecting carcinogens, but it is clear that
they have much to offer.  Although their methodology  is  still in its infancy, the
systems already appear capable of identifying carcinogens as reliably as any other
short-term tests now in use. In addition, they offer potential advantages over other
systems in (1) scoring oncogenic transformation in vitro, an end point that is more
closely analogous to carcinogenesis than the end points scored in other short-term
tests such as mutagenicity assays or suppression of sebaceous glands; (2) possessing
the  capability  to utilize cells of diverse sources and types,  including human cells,
thus enabling direct  analysis of species and organ differences in  response to
potentially carcinogenic stimuli; and  (3) adaptability to the  detection and char-
acterization of incomplete carcinogens, cocarcinogens, promoting  agents, and
other factors modifying the process of carcinogenesis.
  While the prospects for in vitro cell transformation assays are highly encouraging,
their ultimate practical usefulness will depend on the extent to which various techni-
cal problems can be resolved. One problem  encountered  with all the assay systems
investigated thus far is variability among target cell populations. Cells of different
clones, as well as those of different embryos, vary in their growth characteristics and
transformation response in vitro. Because of this variability, cell populations used
for assays must be selected with care, monitored closely, and standardized, insofar as
possible, by the use of cryopreserved pools of cells which are carefully defined and
derived by expansion from rigorously maintained and uncontaminated foundation
stocks.
  Additional factors affecting the reproducibility of assays  include variations in
successive batches of culture medium, serum, trypsin, and other animal-derived re-
agents, glassware, duration of storage of cells and culture materials, quality of air
and water, number of times the cells are subcultured before use, and cell population
density at seeding(Pienta, 1979). Attention to these matters is essential for reproduc-
ibility of results, but despite all such efforts to date,  inexplicably large variations
continue to be observed from time to time in positive and negative controls, empha-
sizing the complexity of culture systems and the need for further refinements in their
methodology (Sivak et al., 1980).
  Another important variable is the ability to convert precarcinogens into active me-
tabolites. It is  evident  that a supplementary system for metabolic activation, in the
form  of microsomal homogenates or hepatocyte feeder layers, is necessary for the
transformation of certain types of cultured cells (especially embryo fibroblasts) by
some classes  of chemicals  such as  aromatic amines and nitrosamines.  It would
appear, moreover, that the effectiveness of such exogenous metabolic activation
systems may vary, depending on their species  and organ of origin, the enzyme in-
ducer (if any)  used for priming the donor animal from which they are derived, the
                                     54

-------
type of chemical to be activated in the assay, and the target cells to be transformed
(Sivak and Tu, 1980). Further complicating the problem are  variations in the
toxicity of microsomal preparations for different cells in vitro, the dependence of cell
transformation on the diffusion  of active metabolites to critical sites within the
target cells, and the likelihood that both deactivation and activation are occurring
simultaneously in  the system. In view of these complexities, it would appear that
different classes of chemicals call for different assay regimes, the details of which
cannot yet be specified. Hence, the design of an optimal strategy for each class  of
compounds will require further research. Moreover, because S-9 homogenates differ
in their activation patterns from the intact cells whence they are derived, feeder layers
of intact cells would seem preferable to homogenates as exogenous metabolic activa-
tion systems (Bigger et al., 1980).
   Cytotoxicity also constitutes a source of variation, since the frequency of transfor-
mation is a function of the number of cells surviving exposure to the carcinogen and,
in turn,  cell density. No simple relation  between cytotoxicity and transforming
activity is evident from studies to date. With many chemicals, transformation is elic-
ited only over a narrow range of doses, high concentrations being too toxic although
not necessarily lethal, and low concentrations requiring treatment of many replicate
cultures over extended periods of time (Casto and Carver, 1980). With such chemi-
cals, negative responses may merely reflect failure to test an appropriate range  of
doses. With carcinogens that transform only at high levels of toxicity or at low fre-
quency, the use of mass cultures can be advantageous by increasing the number  of
cells at risk and amplifying transformational changes through successive passages  of
the affected cells. This technique has the disadvantage, however, of being unable  to
indicate  the precise frequency of transformation, since  each of the  foci that are
scored cannot be assumed to have originated from a single transformed cell. A simi-
lar limitation restricts the use of focus assays, in general, as quantitative tools. These
problems warrant continuing study.
   Although it is apparent from existing data that in vitro transformation assays are
capable of identifying carcinogens of a wide variety of chemical classes, further vali-
dation is clearly called for. Efforts aimed at such validation should include attempts
to determine the most appropriate assay regimen for every class of chemicals, com-
paring responses to noncarcinogenic as well as carcinogenic  analogues in each class
(Purchase et al., 1978). Only in this way will it eventually become possible to select
the most reliable test, or battery of tests, for evaluating unknown substances.
   Further refinements in methodology should reduce the frequency of false negative
results in the future. Such results may never be eliminated entirely, since some
substances undoubtedly contribute to carcinogenesis in vivo by acting on posttrans-
formational stages in the cancer process, through immunosuppression or other ef-
fects on homeostasis at the tissue or organ level. It is noteworthy that some in vitro
transformation systems have been found capable of detecting the effects of various
promoting agents,  cocarcinogens, and other modifiers of carcinogenesis. Since these
factors may conceivably be as important in the pathogenesis of most human cancers
as complete carcinogens or initiating agents, and since there is little prospect  of
assaying for them on a large scale in whole animals with the limited resources that
are available, the possible application of in vitro cell transformation systems is one
of the most important questions to be explored.
   It  is noteworthy  that false positives have been observed in  less than one percent  of
assays. Their occurrence in some instances may actually signify that the chemicals  in
question have received insufficient study in vivo and would, in fact, turn out to be
carcinogens if investigated more adequately. Alternatively, it is entirely conceivable
that transforming activity in vitro will  not always be correlated with carcinogenicity
in vivo, because of the different mechanisms that operate in vivo (for example, de-
activation, DNA repair, or immunological surveillance) which may alter the final
outcome  (Purchase et al., 1978). To minimize the frequency of false positives, it  is
                                      55

-------
important that the criteria used for scoring transformation include the ability of the
transformed cells to form tumors in test animals.
  Based on the evidence available thus far, the in  vitro cell transformation assay
appears to be the most reliable and versatile short-term test for carcinogenicity. The
assay can be performed in a matter of days or weeks, at a cost ranging from a few
hundred to a few thousand dollars. It is, therefore, far less costly and time-consum-
ing than  bioassays  of whole animals. Pending further validation  of the assay for
chemicals of diverse classes, and in view of continuing improvements in its sensitivity
and reliability, it should be considered for inclusion in any battery of short-term tests
employed for detecting and screening  environmental carcinogens. Furthermore,
because of its long-range potential  as a test  that may be applicable to promoting
agents, cocarcinogens, and other factors modifying the carcinogenic process, further
research  to explore and refine such applications should receive high  priority.

ACKNOWLEDGMENTS
   The  author thanks Ms. Lynda Witte and Ms. Jean Smith for  assistance in the
preparation of this manuscript.


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 APPENDIX 1

 Transformation of Golden Syrian  Hamster Embryo Cells By Chemicals of
 Different Classes.*
                 Chemicals
                   Tested
Reported
Carcino-   Transfor-
genicity    mation
in wVof    in vitro^
 Reported
 Mutagen-
   icity in
Salmonella^
 Direct Alkylating Agents

 -Caprolactone
 Glycidol
 1,4-Butane sultone
 1,3-Propane sultone
 Methyl iodine
 Dimethylcarbamyl  chloride
 Benzyl chloride
 Glycidaldehyde
 Ethyl-p-toluenesulfonate
 1,2,3,4-Diepoxybutane
 Propyleneimine

 Polycyclic Aromatic Hydrocarbons and
  Derivatives

 Pyrene
 Benzo(a)pyrene
 Benzo(e)pyrene
 Phenanthrene
 3-Methylcholanthrene
 Anthracene
 Benz(a)anthracene
 7,12-Dimethylbenzanthracene
 1,2,3,4-Dibenzanthracene
 1,2,5,6-Dibenzanthracene
 Chrysene
 1,8,9-Trihydroxyanthracene

 Nitrosamines and Nitrosamides

 /V-Nitrosodiethylamine
 /V-Nitrosodimethylamine
 /V-Nitrosoethylurea
 /V-Nitrosopiperidine
 /V-Nitrosodiphenylamine
 /V-Methyl-/V'-nitro-/V-nitrosoguanidine
 Methylazoxymethanol acetate
               +§
               +
               +
               +

               +
               +
                                       59

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Aromatic Amines, Aminoazo Dyes,
 and Nitro Compounds

1-Naphthylamine                               -                       +
2-Naphthylamine                               +          +            +
1-Anthramine                                  -                       +
2-Anthramine                                  +          +            +
Benzidine                                      +          +            +
2,4-Toluenediamine                             +          +            +
2-Nitro-p-phenylenediamine                     +          +            +
4-Nitro-o-phenylenediamine                     U          +            f
m-Phenylenediamine                            U          +            +•
4-Methoxy-m-phenylenediame                   +          +            +•
4-Aminoazobenzene                            +                       +
4-Dimethylaminoazobenzene                     +           <-            +
3-Methoxy-4-aminoazobenzene                  +          +§           +
2-Methyl-4-dimethylaminoazobenzene
3-Methyl-4-dimethylaminoazobenzene            +          +            +
7,9-Dimethylbenz(c)acridine                     +          +            +
Acridine orange                                +1-            +
Aniline
o-Chloroaniline                                 -          -
p-Chloroaniline
p-Rosaniline                                    +          +§
4-(o-Tolylazo)-o-toluidine                        +          +            t-
Azaserine                                       +•          +            +
2-Aminobiphenyl
4-Aminobiphenyl                                +•          +            +
2,3'-Dimethyl-4-aminobiphenyl . HCI             +          +            +
4-Nitrobiphenyl                                 +          +            +
2-Nitronaphthalene                              t          +            +
2-Nitrofluorene                                 +          +§           +
4-Nitroquinoline-1-oxide                         <•          +            +
4-Hydroxylaminoquinoline-1-oxide               +          +            +
2-Fluorenamine                                 *•          +            +
/V-4-Acetylaminofluorene
/V-2-Acetylaminofluorene                         <•          +            +
A/-Hydroxy-2-acetylaminofluorene                 +          +            +
/V-Acetoxy-2-acetylaminofluorene                 <-          +            +
Bis(p-dimethylamino)-diphenylmethane           +          +            -

Heavy Metal Salts

Lead acetate                                   +           f            ?
Beryllium sulfate . 4H20                        +           +            ?
Titanocene dichloride                             +•           +            ?
Nickel  sulfate hexahydrate                       +           +            ?

Solvents

Acetone
Ethyl alcohol
Methyl alcohol
Dimethyl sulfoxide
Dimethyl formamide                             -
1,2-Propanediol                                 -                       ;>

Miscellaneous Classes

Acetamide                                      +          +
Thioacetamide                                  +          +
Thiourea                                       +•          +
                                     60

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5-lododeoxyuridine
5-Bromodeoxyuridine
5-Fluorodeoxyuridine
Bromobenzene
Hydrazine sulfate                               +           +
1,2-Dimethylhydrazine                           +           +
Safrole                                         +           +
Aflatoxin 61                                    +•           +
Aflatoxin B2                                    t           +
/V-[4-(5-Nitro-2-furyl)-thiazolyl]-formamide         +           +
2-(2-Furyl)-3-(5-nitro-2-furyl)-acrylamide          +           +
a-Napththylisothiocyanate
Saccharin                                      +
Methyl carbamate
Ethyl carbamate (Urethan)                       +          +§
Succinic anhydride                              +           +
Auramine                                      +          +§
Ethionine                                      +           +
3-Amino-1,2,4-triazole                           +           +
1-Phenyl-3,3-dimethyltriazene                   +           +
Natulan . HCI (Procarbazine)                     +          +§
12-O-Tetradecanoylphorbol-13-acetate            P
Aroclor  1254 r                                  -
Limonene                                      P
Caffeine
Methotrexate
Hycanthone methanesulfonate                   +           +
Sodium nitrite                                  -
Hydroxylamine   HCI
Methoxychlor                                            .
'From Pienta (1979).
t+ = positive result; - = negative result; P = promoter; U = undertermined.
JData summarized by Pienta (1979) from McCann et al. (1975).
§Chemical transformed cells only when hamster liver S-9 homogenate was added to the
 system.
                                     61

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APPENDIX 2

Transforming Effects of Various Chemicals on  Murine Leukemia Virus-
infected Fischer Rat Embryo Cells.*
                                             Carcinogenic       Transforming
                                               Activity            Activity
 	Chemical Class	in wVof	in

A zo Dyes

Aminoazobenzene
4-Dimethylaminoazobenzene                        +
4-Dimethylazobenzene                             +
2-Methyl-4-dimethylaminoazobenzene
3'-Methyl-4-dimethylaminoazobenzene              +
6-(4-Dimethylaminophenylazo)quinoline              +

Polycyclic Compounds

Anthracene
 Naphthalene
Phenanthrene
 Fluoranthrene
3-Methylcholanthrene                              t-
7,9-Dimethylbenz(c)acridine                         +•
 Dibenz(a,/))acridine                                 +
 Dibenz(a,/')acridine                                  +
 Benzanthracene                                    +
 Dibenz(a,/?)anthracene                              +
 7,12-Dimethylbenz(a)anthracene                     +
 Benzo(a)pyrene                                     +
 Benzo(e)pyrene                                     +
 1,2,5,6-Dibenzanthracene                           +

Acromatic Amines

Aniline
/V-Acetoxy-/V-2-fluorenylacetamide                   +•
/V-Hydroxy-/V-2-fluorenylacetamide                   +
/V-2-Fluorenylacetamide                             +
 2-Aminofluorene                                   +
3-Aminofluorene                                   ±
4-Aminofluorene
 1-Naphthylamine
 2'-Naphthylamine                                  +
 2-Aminobiphenyl
4-Aminobiphenyl                                   +
0-Toluidine                                        +
 3,3'-Dichlorobenzidine                             ±
 4-Nitroquinoline oxide                             +

Nitrosamines

 Diethylnitrosamine                                 +•
 Dimethylnitrosamine                               +
 Diphenylnitrosamine
 A/-Methyl nitrosoguanidine                          +
 Nitrosoethylurea                                   +
 Nitrosodiphenylamine                              +
                                       62

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Miscellaneous Compounds

Glyceraldehyde                                    4-                 +
Dimethylcarbamylchloride                           +                 +
Methyl carbamate                                 4-                 4-
Ethyl carbamate (urethane)                          4-                 +
Methylazoxymethanol acetate                       f                 +
Propane sultone                                   +                 +
Uracil mustard                                    +                 4-
Cyclophosphamide                                 +•                 +
Thioacetamide                                     +                 +•
Acetamide                                        ±                 +
Formamide
Acetone
Propyleneamine                                   +                 f
1,2-Epoxybutane                                  +                 ±
Beryllium sulfate                                  +                 +•
Lead acetate                                      +                 4-
Nickel sulfate                                     +                 +
Natulan                                          +                 4-
Thiourea                                         +                 4-
 *From Sivak(1979); Price and Mishra (1980).
 t+ = positive result; - = negative result;
 ±= variable or borderline results.
APPENDIX 3

Comparative Activities of Various Chemicals as Evaluated by In Vitro Cell
Transformation and Five Other Short-Term Tests for Carcinogenicity.*
Compoundf
Acridine
2-Acetylaminofluorene
4-Acetylaminofluorene
Aflatoxin B
4-Aminoazobenzene
2-Aminobiphenyl
4-Aminobiphenyl
2-Aminochrysene
6-Aminochrysene
3-Aminopyrene
2-Aminonaphthalene-
1-sulfonic acid
Aniline
p-Anisidine
Anthracene
2-Aminoanthracene
Anthranilic acid
Anthraquinone
Chemicc
P
AA
AA
M
AA
AA
AA
AA
AA
AA"

AA
AA
AA
P
AA
AA
M
1.1 1 11 1
i- ^ — £ 0 " U>
CO -. CD CO CO 5 *
O • £ O t: OQ E Q
_ _
4-4-4-4-
- 4-
4- 4- + +
4-4-4-4-
4-4-4-
4-4-4-4-
4-4-4-4-
4-4-4-4-
4-4-4-4-

_ 4-
-
- 4-
- - 4-
4- 4- + 4-
-
-
?! a °'£. c
£ k S « ™
J2 D. t -i5 °-
w io K £ —
_
-
Nt Nt Nt
4-
4- 4-
+
+
+ 4-4-
4-4-4-
4- 4-

-
-
+
-
4-
-
4-
                                    63

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Anthrone                 M      -                                   +
1,2-Benzanthracene         P      4-      4-      +       4-       +•      f       ~
Benzanthrone             M                            +
Benzidine                AA      +      *•      +       +       +
Benzimidazole              P      -      -                            +
Benzole acid              M
3,4-Benzpyrene            p4-4-      +       4-<--++
6-Benzoyl-2-naphthol      M      -      +              +      +
Biphenyl                  M             -                     *
Bis azo compound7        M       -                            +
Bis(Chloromethyl)ether    Alk       t-       +•       +             +       +      +
/V,A/'-Bis(2-naphthyl)-p
  -phenylenediamine       AA              -             *•              -      +
Butanesultone            Alk       <-      +      +      +       +       +     Nt
Caffeine                   M                                    +
Calmagite1                 M              -             +       f
Camphor                  M                     -              +       +      +
Carbazole                  P                             +              -
 Chlorambucil             Alk       +      +      +       -       +       -     Nt
Chloramine-T              M
 Cholesterol                M
 Colchicine                 M       -             -              +       +     Nt
 Croton oil                 M       +             +       +       +       +      +
 Cyanocobalamin (B1Z)      M                     -                     *
 Cycasin acetate2          Alk      +      +      +       *       +•
 Cyclohexylamine           M              -              t       +       -
 Cyclophosphamide        Alk      +      +      +       +                     +
 3,3'-Diaminobenzidine    AA                     4-       +       +
 2,7-Diaminofluorene      AA      +      +              +              +      +
 3,4,5,6-Dibenzacridine      P      +             +       +       4-
 1,2,3,4-
  Dibenzanthracene          p      4-      +       <•       -       +            ++
 3,4,9,10-Dibenzpyrene      P      +      +       t       +       +      +     4-+
 3,3'-Dichlorobenzidine    AA      +      +       +       +
 2,4-Dichloro-
  phenoxyacetate            M      -              -      +
 Dicyclohexylamine          M                                          +
 DDT3                      M
 Dieldrin4                   M                                         Nt
 Diethylnitrosamine        Alk      +•      +       +       +             f
 Diethlstilbestrol            M      +•                                   +
 3,3'-Dimethoxybenzidine    AA      1-      +       +       *•      +      -
 4-Dimethlaminoazo-
  benzene                AA      +       +       +      +•              +       +
 9,10-Dimethylanthra-
  cene                      p      4-       4-       +             4-       4-       +
 p- Dimethyl ami nobenz-
  aldehyde                AA
 7,9-Dimethylbenz-
  acridine                  P      4-       +       +      4-      +             Nt
 7,10-Dimethylbenz-
  acridine                  P      +       4-       +      4-      f             Nt
 9,10-Dimethyl-1,2-
  benzanthracene           P      +      4-       +      4-4-4-+4-
 1,1 '-Dimethyl-4,4'-
  bipyridinium
   dichloride                M                                   +
 3,3'-Dimethylbenzidine    AA      +      +       4-       4-      +
 Dimethylcarbamoyl
  chloride                Alk      4-4-4-              4-       _      ++
 Dimethylformamide         M
                                       64

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Dimethylnitrosamine      Alk      +       +       _      +      _      +      _
2,3-Dimethylquin-
 oxaline                   P              -              +      +
Dinitrobenzene            M              -       -             +
2,4-Dinitrofluoro-
 benzene                 M      +       +       <--      +
2,4-Dinitrophenol          M              -              +
Dinitrosopentamethly-
 ene tetramine            M              -              -      -      -
DL-Ethionine             Alk      +       +              +             +
1,1'-Ethylene-2,2'-
 bipyridinium dibro-
  mide                    M                                   +      -
Ethylenethiourea          M      +       f       +
Ethyl methane-
 sulphonate              Alk      +       +       +             +
Hexachlorocyclohexane      M      -                     +      -      +
Hexamethylphosphor-
 amide                    M      +       +       +
Hydrazine                 M      +       +       +             +
Hydrocortisone            M      -       +       -             +
Indole                      P              -       -             +      +
Merchlorethamine5       Alk      +       +       +             +
20-Methylcholanthrene       P      +       +       +      +      +      +++
Methylene bis
 (2-chloroaniline)         AA      +       +       +      +      +      -
2-Methylindole             P              -              +             +
MNNG6                  Alk      +       +       +      +      +      +
3-Methyl-4-nitroquin-
 oline-/V-oxide            AA              -
Mitomycin C             Alk      +       +       +      +      +      _
Morgan's base             P      +       <-       +      -      +      -     Nt
Naphthalene               P                                   -
1-Naphthol                M      -              +
2-NaphthoI                M                     -                    +
1-Naphthylamine         AA      -
2-Naphthylamine         AA      +              +      +      +      +
2-Naphthylamine-1,5-
 disulfonic acid di-
  sodium salt            AA              -                            +
Nitrobenzene              M                     -      -             +
2-Nitrobiphenyl           AA      +       +       <-      +      _            Nt
4-Nitrobiphenyl           AA      f       +       +      +      +
2-Nitrofluorene           AA      +       +       +      +                   Nt
4-Nitroquinoline-
 /V-oxide                 AA      +       +       +      +      +      +      +
/V-Nitrosodiphenyl-
 amine                    M      ____      +      +
/V-Nitrosoephedrine       Alk      +       +       +      <-      +      -
/V-Nitrosofolic acid        Alk      +       +       +                    _
4-Nonylphenol/ethylene
 oxide condensate         M                     -             -
Orotic acid                M              -       -             +
Perylene                   P                     +                    +
Phenobarbital             M              -                     +      +
/V-Phenyl-2-
 naphtnylamine           AA              -              +      +      +
Propanesultone           Alk      +       +       +      +      -      +      +
/3-Propiolactone           Alk      +       +       +      +      f
Resorcinol                M      -       -              -             -      -
Riboflavin                 M      -
                                      65

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Safrole                   M      +      +      +      +      +
3,3',5,5'-Tetramethyl-
 benzidine                AA      -                    +
Toluene                  M
Toluene-2,4-diisocya-
 nete                    M                                                 +
2,4,5-Trichlorophen-
 oxyacetate               M              -                    +      -      -
Trimethylphosphate        M       -      _      +      -       -      -Nt
Urethane                 M       +      +      +•                    +      +
Vinyl chloride            Alk       +                           +
 From Purchase et al. (1978).
 Compound:
  (1) Calmagite:  2-hydroxy-1-(2-hydroxy-5-methylphenylazo) naphthalene-4-sul-
     fonic acid.
  (2) Cycasin (acetate): methyloxymethanol acetate.
  (3) DDT: 1,1,1-trichloro-2,2-bis(4-chlorophenyl)ethane.
  (4) Dieldrin: 1,2,3,4,10,10-hexachloro-6,7-epoxy-1,4,4a,5,6,7,8,8a-octahydro-
     endo-1,4-exo-5,8-dimethanonaphthalene.
  (5) Merchlorethamine: bis(2-chloroethyl)methylamine hydrochloride.
  (6) MNNG: /V-methyl-/V'-nitro-/V-nitrosoguanidine.
  (7) Bis azo compound: 2,2'-bis[1-(3-octadeclyaminopropylimino)ethyl]-2,2'-[3,3'-
     dichloro-4,4'-biphenylylene)bis(azo)]bis(acetanilide).

 fChemical class: AA = arylamines and related compounds; Alk = alkylating agents; P =
  polycyclic aromatic hydrocarbons; M = miscellaneous compounds.
 §Carcinogenicity in vivo: + = carcinogen;  - = noncarcinogen.
 ||Cell transformation: growth of BHK-21 cells in soft agar.
 #lmplant test: ++ = tumor at site of implant; Nt = not tested.
 APPENDIX 4
   In addition to the Federal Food, Drug, and Cosmetic Act (1938), a variety of other
 laws has since come into  being, conferring regulatory responsibilities on several
 agencies (Appendix 4, Table  1).
   To facilitate coordinations of regulatory policies, the CPSC, EPA, FDA and
 OSHA in 1977 formed the Interagency Regulatory Liaison Group. In their 1979 Re-
 port on short-term tests for carcinogenicity, they concluded:
       The study of carcinogenesis at the cell level presently offers an effective
     means to identify carcinogenic effects and mechanisms. In vitro mammali-
     an cell transformation systems are  simple  models for the study of the
     mechanisms of chemical  and physical  carcinogenesis.
       As these systems become more widely used as test methods, they will
     lead not only to better development and definition of screening techniques
     but also  to  better  understanding  of the  underlying  mechanisms of
     carcinogenesis.
       Short-term tests for chemical carcinogens presently do not, in the ab-
     sence of animal bioassays and epidemiology data, constitute definitive evi-
     dence that a substance does (or does not) pose a carcinogenic hazard to hu-
     mans. However, positive responses in these tests are considered suggestive
     evidence of a carcinogenic  hazard.
                                      66

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Table A of Appendix 4.  Major Legislation Providing Regulatory Authority
                         for the Control of Environmental Carcinogens.*
 	Statute	Regulatory Agencyf

Federal Food, Drug, and Cosmetic Act June 25, 1938              FDA, EPA
Public Health Service Act P.L. 91 -696 (1912)                        FDA
Federal Insecticide, Fungicide, and Rodenticide Act P.L.
 92-516(1947)                                                 EPA
Federal Hazardous Substances Act P.L. 92-516 (1972)               CPSC
Occupational Safety and Health Act P.L 91-596 (1970)              OSHA
Clean Air Act P.L 88-206 (1963)                                  EPA
Poison Prevention Packaging Act P.L. 92-516 (1972)                 CPSC
Clean Water Act  P.L. 95-217(1977)                                EPA
Consumer Product Safety Act P.L. 92-573 (1972)                    CPSC
Safe Drinking Water Act P.L. 93-523 (1974)                        EPA
Fair Packaging and Labelling Act P.L. 93-608  (1975)                 FDA
Resource Conservation and Recovery Act P.L. 94-580(1976)           EPA
*From Dept. of Health and Human Services. First Annual Report on Carcinogens (1980).
fEPA = Environmental Protection Agency; FDA = Food and Drug Administration; CPSC =
 Consumer Products Safety Commission; OSHA = Occupational Safety and Health Ad-
 ministration, Department of Labor.
      Such positive results also supply supporting evidence to positive animal
    bioassays or epidemiology results. In some instances results from short-
    term tests may conflict with animal bioassay data. If an animal bioassay
    shows a positive response, it cannot be dismissed because a negative re-
    sponse was observed in these tests. However, positive responses in such
    short-term tests are ordinarily sufficient to provide suggestive evidence of
    carcinogenicity, even if the substance tested has shown only negative re-
    sponses in some animal bioassays. As the degree of certainty attached to
    the negative responses in animal bioassays increases because the observa-
    tion is reproduced in other animal species and strains or under more rigor-
    ous test conditions, the suspicion about the chemical as a result of short-
    term tests may be reduced and  eventually eliminated. These conclusions
    are in accord with those of the National Cancer Advisory Board's Subcom-
    mittee on Environmental Carcinogenesis  (1977);
      At  the present, none  of the short-term tests can be used to establish
    whether a compound will or will not be carcinogenic in humans or experi-
    mental animals.  Positive results obtained in these systems suggest exten-
    sive testing of the agent in long-term animal bioassays, particularly if there
    are other reasons for  testing. Negative results  in a  short-term  test,
    however, do not establish  the safety of the agent.
  These conclusions are in accord with the preliminary results of an international
collaborative study, which have been interpreted as follows by the National Toxicol-
ogy Program (1980):
      Short-term tests can be used to predict carcinogenic activity, but no sin-
    gle assay or battery of assays is readily apparent as best suited for this pur-
    pose.
      Reliability of data from any assay system depends on thorough under-
    standing of the system, awareness of pitfalls, and careful conduct of experi-
    ments.
                                     67

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  Most assays have produced false negatives and false positives; thus, to in-
clude any assay in a battery of tests requires a tradeoff between these two
classes of errors.
  Specific conclusions about test systems performance and relative utility
must await more detailed analysis of the data base.
  Strong evidence for the use of a test battery has been obtained, yet recom-
mendations on test batteries must await a clear definition of their applica-
tion and the relative importance of false positive and false negative results.
                                  68

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         SHORT-TERM TESTS FOR IDENTIFYING
                           TERATOGENS*

                         Thomas H.  Shepard


INTRODUCTION
  The biological survival of the human race is dependent upon the success of three
major processes:  (1) maturation of the female and male germ cells and  their
favorable union (gametogenesis and fertilization), (2) intrauterine development of
the conceptus, and (3) postnatal growth and development. Teratology is the science
dealing with the causes, mechanisms, manifestations and prevention of intrauterine
developmental defects of either structural or functional nature. A teratogenic agent
(teratogen) may be a chemical, a drug, a virus, a physical condition or a deficiency
state.

Scope of Societal Problem

Incidence of Reproductive Loss—
  Although it is recognized clinically that from 15 to 25 percent of human pregnan-
cies end in spontaneous abortion, the majority during the first  trimester, there are
lines of evidence that over 50  percent of all conceptuses are lost. In what is now a
classic series of studies published in the 1940s and 1950s by Hertig, Rock and others
(see Hertig, 1967),  a series  of gravid uteri of less than 4 weeks' gestation were exam-
ined following hysterectomy. Because coital and menstrual dates were known, it was
possible to study the loss of the conceptus as related to actual gestational age. Histo-
ries could be compared with endometrial histology, acting as a check on menstrual
dating. The criteria in these studies for immediate or impending loss consisted of
severe pathologic features such as absence of inner cell mass or trophoblastic insuffi-
ciency for the stage of development.
  The total percentage of conceptuses in  the process of loss in these studies ranged
from 34 to 43 percent. However, when Hertig (1967) reconsidered the findings, a
more impressive rate of loss emerged. It was found that in any single menstrual cycle
15 percent of oocytes were unfertilized despite optimal conditions for fertilization to
take place. A further 10 to 15 percent were fertilized but failed to implant. Another
30 to 35 percent  loss occurred  during the first  week following  implantation.
Therefore, between 55 to 65 percent of oocytes were lost and only a total of about 40
percent of the starting group of women missed a menstrual period.
  Recent studies utilizing highly sensitive chemical tests of pregnancy have been
able to  detect a postimplantation conceptual loss  of 41 percent among  healthy
•Supported in part by the National Institute of Child Health and Human Development (HD00836).

The A uthor: Thomas H. Shepard is on the staff of the Central Laboratory for Human Embryology and the
Departments of Pediatrics and Obstetrics and Gynecology, University of Washington, School of Medicine,
Seattle. He is also affiliated with the University's Child Development and Mental Retardation Center and
Regional Primate Center.
                                     69

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women (Williamson and Miller, 1980). Based upon the use of theoretic calculations
from the rates of chromosomal defects in spontaneous abortions, Boue'et al.(1975)
have estimated that 50 percent of all conceptuses may be lost from chromosomal
errors.
  In this important general area of human biology, there is an immense need for
further  information.  The surveillance  of spontaneous abortions  is made very
difficult by the absence (in most cases) of good control or baseline data. Unfortu-
nately, with existing surveillance methods, the loss rate that is found is proportional
to the intensity of the inquiry. A more detailed review of this subject has been pub-
lished (Shepard and Fantel, 1979).
Incidence of Congenital Defects—
   Approximately three percent of all human newborns have a congenital anomaly
requiring medical attention, and one-third of these conditions can be regarded as life
threatening. With increasing age, over twice as many congenital defects are detected.
Close to one-half the number of children in hospital wards are there because of pre-
natally acquired malformations of one kind or another. Another three percent of our
population is mentally retarded, although a certain part of this problem is caused by
postnatal factors. A congenital defect, whether structural or functional, exists for an
individual's entire life span. It is not a year or two, or ten years. In terms of human
suffering and the financial and  social dislocation of  the  family, this  toll  is
tremendous. Historically, after identifying the causative mechanism, the opportunity
for prevention in this field has proven to be outstanding. Two examples of this are
the near prevention of kernicterus by preventing maternal immunization to Rh fac-
tor and the congenital rubella syndrome by mass immunization.
   Our knowledge of the cause and prevention of human malformations is extremely
limited  in that approximately 70 percent are of unknown causes. About 10 percent
are associated with gene mutations, five percent with chromosomal aberrations and
less than 10 percent are known to be due to the specific teratogenic agent. Although
there are more than 600 agents known to produce congenital anomalies  in experi-
mental  animals, less than 25  of these are known to cause defects in the human
(Schardein, 1976;  Shepard,  1980). The reasons for this apparent wide discrepancy
between the numbers of animal and human teratogens are discussed more fully later
in the chapter, but are particularly related to the immense doses which investigators
are able to give experimental animals.

HISTORICAL PERSPECTIVE
   There are many archaeologic artifacts which show that men and women of many
countries and continents have been fascinated by monstrosities. Babylonian tablets
give various congenital defects which were thought to be prognostic of the political
and  economic future of nations. A long history of how maternal impressions  may
cause congenital anomalies has been recorded; this idea,  in part, is at the root of
many of the  guilt feelings still found in  parents producing defective offspring.
   A more descriptive aspect of teratology, along with early biologic inquiry, began
in the 19th century and continued through the early 20th century. Some of the scien-
tific descriptions by  Meckel, the Saint-Hilaires, Taruffi, Schwalbe and Ballantyne
rival or better those of present day. Further details of this fascinating history can be
obtained from Warkany (1971).
   Bridging between the ancient historical concept of teratology and the present-day
experimental approach, there was a period of time when most malformations were
considered as being the result of gene mutations. With the discovery by Hale (1937)
and by Warkany and Nelson (1940) that vitamin deficiencies could cause defects in
experimental animals, a new impetus to the use of experimental methods arose  In
1960 the Teratology Society was founded, and some years  later teratology societies
were established in Europe and Japan.
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  The thalidomide tragedy of the early 1960s gave further impetus to experimental
teratology, and, in particular, pointed out the unique teratogenic dangers of our en-
vironment. Government as well as the pharmaceutical industry in particular
responded by beginning to test agents in small animals for their teratogenic poten-
tial. In the past few years with the appearance of the Ames test for mutagenicity,
much pressure has been applied to produce a similar short-term tool for teratololgy.
PRESENT STATE OF TERATOLOGY

Principles

Relationship between Teratogenic Susceptibility and Stage of Development—
  To a large extent, teratogenic susceptibility depends upon the period of develop-
ment when the conceptus is exposed to an agent (Fig. 1). Damage during the implan-
tation and presomite periods (0-15 days) generally produces little altered morpho-
genesis because the ovum either dies or regenerates completely, whereas, during
major organ formation (18-60 days), the embryo is highly sensitive and exposure
may produce significant morphologic changes. During the subsequent period, the
fetus is less sensitive to morphologic alterations, but changes in functional capacity,
such as intellect, reproduction or the rate and process of aging, may develop in later
postnatal life. This time  specificity has been found in nearly all cases where terato-
genesis in the human has been proved and studied in detail.

Species Variation in Response to Teratogenic Agents—
  Some species of animals are much more susceptible to specific teratogenic agents
than others. Aspirin, cortisone and several vitamin deficiencies are highly terato-
genic in the rodent, but there is no solid evidence of their teratogenicity in humans.
The thalidomide epidemic would not have been prevented by testing prenatal mice
and rats, but the drug is teratogenic in rabbits, monkeys and humans. It seems likely
that there are certain common embryologic processes between experimental animals
and man. Various pharmacologic and physiologic mechanisms that operate to con-
trol the maternal blood concentration of a given teratogen are shown in Fig. 2. In ad-
dition, interspecific differences exist in placental transport and embryonic metabo-
lism. One developing hypothesis is that the species variability is due in large part to
variations in biotransformation of the administered drug. The hepatic microsomal
monooxygenase (mixed-function oxidase) system plays a role  in the metabolism of
more than 70 percent of drugs and chemicals. The rate of breakdown or the type of
byproduct produced by  this system in different animals might help to explain the
species specificity.

Drug Quantity and Teratogenicity—
  Any drug given in large enough amounts will adversely affect fetal development.
This action usually occurs through deleterious effects on maternal health and is ex-
pressed as either embryo-fetal death, fetal growth retardation or osseous retarda-
tion. Both sodium chloride and sucrose given in sufficient amounts to experimental
animals will produce these embryo-fetotoxic effects. Many of the warnings in drug
inserts about  potential teratogenicity  are  related to this  phenomenon.  When
extrapolating the dose-response curve from animal experiments to humans, it is im-
portant to take into consideration the ratio (on a per kilogram basis) between the ter-
atogenic dose in the animal and the therapeutic or exposure dose in the human (see
Setlow, this volume). Figure 3 illustrates the usual relationship between dose and the
zones for teratogenicity, embryo-fetal lethality and maternal  lethality.
                                     71

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                                                Functional maturation  —-3
          |	 Organogenesis  	
          Embryonic period                       Fetal period
                        Entire developmental span
Figure 1.  Curve illustrating the susceptibility to teratogenesis from fertilization
          throughout intrauterine development (from Wilson, 1973, used by permission).
                                      \  Large or
                                      )  highly charged
                                      \  molecules cross
                                                                     Kidney
                                                                           Bile
                          Placental
                           transfer
Hemeostatic
  dispersal
Figure 2.   Diagram of the factors that influence embryonic dose of a foreign chemical
          present in the maternal blood stream. Major individual and species differences
          in absorption and homeostatic dispersal control the blood concentration to
          which the placenta is exposed. Variations in placental transfer exist between
          species but very little work has been done on interspecific differences in
          drug metabolism by the embryo (from Wilson,  1973, used by permission).
                                       72

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     f                  Embryotoxic range                  ^
                                                                       100%
                                                                        50%
   Increasing dosage


CD No effect zone  II Teratogenic zone  [13 Embryolethal zone  HJMaternalethal zone
 Figure 3.  Diagram showing that each drug tested has a dose spectrum ranging
           from no effect to one of maternal lethality. The teratogenic zone may be
           broad, very small or, in some cases, nonexistent (adapted from Wilson,
           1973). Naturally occurring rates in untreated animals have been corrected
           for by subtraction from all three zones.
 Multifactorial Causes of Congenital Defects—
   According to their etiology, congenital defects can be divided into three general
 types: (1) those due to a single action of a mutated gene (for example, achondroplasia
 or phenylketonuria); (2) those due to the single action of an environmental agent (for
 example, congenital rubella); or (3) those produced by a combination of several or
 more gene defects and/or environmental agents. These multifactorial causes proba-
 bly account for the major proportion of congenital defects. Congenital dislocation
 of the hip and most forms of cleft lip and palate are commonly mentioned as exam-
 ples of multifactorially  caused congenital malformations.  Fraser and his  students
 have illustrated this mechanism in certain inbred strains of mice and have begun to
 make practical human applications (Fraser, 1969). The A/J mouse exhibits a higher
 natural incidence of cleft lip and palate than do certain other strains; this is because
 of differences in the topographic relationship of the embryonic facial processes. Oth-
 er genes controlling the development of the palate are known to predispose to clefts.
 Examples such as shortening of the head, changes in mandibular length or mecha-
 nisms by which tongue obstruction might prevent normal palatal shelf closure have
 been studied using mutant genes in inbred animal models. Most of the environmen-
 tal agents (for example, aspirin or cortisone) known to produce clefts are more effec-
 tive in these inbred strains. It seems imperative to accelerate the application of these
 principles to man. This requires quantitative methods for identifying the susceptible
 human genotypes and the environmental agents that might contribute to the multi-
 factorially caused defects.

 Experimental Animal Tests
   Common practice is to select for testing two or  three small animal species, usually
 rats, mice and rabbits. The dosage regime usually employed is as follows: the maxi-
 mum dose used is usually in considerable excess, such as 100 times human therapeu-
 tic dose; a fractional dose of the maternal LD-50 (the dose required to kill 50 percent
 of maternal population); and a minimal dose, preferably one that causes  minimal
 maternal toxicity. Since nearly all agents can produce some embryo-fetotoxicity ex-
                                      73

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pressed as weight loss or minor defects at some level, it has been recommended that
this level be determined and taken into consideration in establishing the minimum
dosage.
  Three general time  periods for administering the test agent have been recom-
mended (Kelsey, 1974). The first is a general test for reproductive effects where both
the male and female are started on the drug before pregnancy. Midway during preg-
nancy, one-half of the females are sacrificed and the number of corpora lutea of
pregnancy (ovulated eggs) and the  number and state of implantation sites  are
examined. A decrease  in ovulation,  fertilization or implantation as well as an in-
crease in early embryonic death can  be determined. The remainder of the pregnant
females is allowed to go to term and to litter. The offspring are examined and reared,
and subsequently rebred  to measure any intergenerational change in reproduction.
   A second type of experiment calls for administration of the compound during
varying periods of major embryonic organ  development and then sacrifice of the
mother just before parturition to measure and examine the fetus for visceral  and
bone defects. A third approach is to administer the drug during the latter part of
pregnancy and to observe the offspring in the perinatal and postnatal period. A more
complete discussion of these  tests is available (Wilson, 1975; Palmer, 1978).
   Embryonic growth and placental function in the human are more like those in
subhuman primates than in the rodent and other small animals. Although it would
seem that these primate species would best approximate the human, their cost, the
long duration of pregnancy and the availability of sufficient numbers of pregnant
animals mitigate against their use as  a standard animal. Testing in these species may
be indicated for agents which  must be used during human pregnancy (antihyperten-
sives, hypoglycemics, anticonvulsants) or for agents which are likely to be inadvert-
ently taken during pregnancy. They may be used for testing agents about which a
question of safety arises  after regulatory approval for widespread exposure.
 In Vitro Tests

 Study of Ovigenesis and Spermatogenesis—
   With the identification of the very exacting nutritional requirements for mamma-
 lian ova by Brinster (1975), it became possible to perform in vitro studies of the ef-
 fects of specific emvironmental contaminants.  For instance, Brinster and Cross
 (1972) using mouse ova exposed to copper were able to establish a dose response and
 a lethal concentration. This type of analysis, applied to other chemicals and coupled
 with the knowledge of intrauterine concentrations found in exposed workers, could
 be a great help in establishing safe exposure  levels.  An approximation of uterine
 levels in exposed  women might be obtained by analysis of curettage tissue removed
 for unrelated health reasons.
   The in vitro tests of mammalian eggs have been applied to the study of abnormal
 chromosome  division (Donahue,  1975).  There is  evidence  that  accumulated
 radiation exposure or delayed ovulation may result in increased chromosome imbal-
 ances in the human ova (for discussion and references see Shepard, 1980).
   Sperm counts,  along with the study of their morphology and motility, have been
 used in monitoring men at risk. Unfortunately, these parameters are not well corre-
 lated with fertility. A new test which appears to be better associated with the state of
 fertility utilizes the frequency with which human sperm penetrate hamster eggs in
 vitro (Rogers et al., 1979). Infertility associated with hypospermia has been linked to
 workplace  exposure   to  the  pesticide,  l,2-dibromo-3-chloroPropane  (DBCP)
 (Whorton et al.. 1977). At airborne concentrations between 0.4 to 1.0 ppm of DBCP,
 chronically exposed workers had reduced sperm concentrations and were infertile!
                                     74

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Cell Culture—
  Individual cells are grown in much the same way as bacteria. All the organization
of the original tissue in such cultures is lost, and cell multiplication and growth in
uniform populations are the dominant interests. The technique is very useful in pre-
dicting alteration in cell replication and has been used by some laboratories to screen
new chemicals and drugs. Such a routine test led to the discovery of the important
biologic action of cytochalasins by S.B. Carter (1967) of Imperial Chemical Indus-
tries, Ltd. namely, that these chemicals prevented cellular, but not nuclear, division.
More specific questions about the growth of cells could be answered by using special
cell lines (teratocarcinoma, neuronal, and endocrine) or cultures of different tissues
such as fetal lung or myocardium.

Organ  Culture—
  Small pieces of differentiating organs or limb buds removed from experimental
animals or from human abortion material can be maintained for a number of days in
culture. During this time growth and differentiation can be assessed during exposure
to environmental toxicants. Mainly cartilaginous or endocrine tissues and, to a lesser
extent, renal and cardiac rudiments have been used for this technique. Explanted
thyroid tissue from human abortuses has been used to determine the period of devel-
opment when iodide can be concentrated. This is an important question since thera-
peutic amounts of I31I, if administered to a thyrotoxic woman, can be concentrated
by the fetal thyroid and cause its destruction. This test was done by adding radioac-
tive iodide to the growth medium and measuring its incorporation into the  explant
(Shepardand Pious, 1978). Concentration commenced in thyroid tissue from fetuses
of 70 to  75 days of age.

Embryo Culture—
   Mammalian embryo culture has been feasible since 1967 when D.A.T. New dem-
onstrated normal growth of presomite- and somite-stage embryos maintained in a
serum  medium (New, 1978). A major attraction of this method is that a dose-re-
sponse curve can be established and the no-effect level may prove valuable to regula-
tory agencies faced with the problem of safety standards. A number of agents have
been studied including hyperthermia, ethanol, trypan blue, B vitamins, anesthetics,
alkylating agents and autonomic stimulating drugs. A suggestion has been made
that the serum of at-risk workers could be tested with this system (Chatot et al., 1980).
   Many drugs and chemicals require bioactivation by liver enzymes (the P450 mon-
ooxygenase system). Fantel et al. (1979) have shown that this fraction of liver can be
added directly to the embryo-culture system and will activate teratogenic activity. In
Fig. 4, the use of this bioactivation system with cyclophosphamide, an antitumor
agent, is illustrated. In addition to expanding the number of chemicals which show
in vitro activity against embryos, possibly species variation (see second principle)
may be better understood by using maternal P450 systems from teratogenically sen-
sitive and insensitive species.

Other Short- Term Tests—
  Many new test schemes are being described and evaluated. Since most of these are
in preliminary stages, the reader is referred to the abstracts of the Teratology Society
(1980, 1981, 1982). Several reviews on the use of various in vitro tests in teratology
have appeared (Ebert and Marois,  1976; Wilson, 1978, Shepard and Pious, 1980).
Although the main use of in vitro techniques has been to learn about mechanisms of
action,  there  has  been the expectation that a simple, inexpensive screen for
predicting teratogenic activity in humans could be developed. Unfortunately, this is
unlikely since the basic initiating causes of teratogenic activity involve many biologic
processes which include cell replication and cleavage, cell differentiation, muscle
                                     75

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innervation, as well as cell adhesion, motility and interaction (Moscona, 1975). The
complexity of these causes of altered development is given in Fig. 5.
  Even though these short-term tests may eventually be helpful in predicting terato-
genicity in humans, most teratologists believe that testing of pregnant animals will
continue to be necessary; otherwise there is little doubt, as has been sadly learned,
that the ultimate test  subject for a new agent may continue to be the human.
                          In vitro test for teratogenicity

                         Bioactivation of teratogenic drugs
                     Remove sites on day 10 (early somite stage)
             Drug
    Drug + S-9
or Drug + cofactors
Drug + S-9
+ cofactors
                     Grow for 24 hours - Rotator - 5% 02-37°C
                Normal
                                      Normal
                               Defects,
                              Decrease in
                          growth and protein
Figure 4.  Diagram giving an example of an in vitro test of a teratogen, using a growing
           rat embryo. The drug used was activated by addition of a P450 monoxygenase
           liver fraction (S-9) to the medium. In the bottle containing drug alone and in the
           bottles containing S-9 alone or cofactors alone, the embryos grew in a normal
           fashion over a 24-hour period. In the bottle containing the complete system (drug,
           S-9 and  cofactors), there was  a  dose-response relation  with decreased growth,
           protein content, and an increase in malformations. This example was drawn from
           experiments using cyclophosphamide (Fantel  et al.,  1979).
                                        76

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                                CAUSES
                       Action of an environmental
                            agent on embryo
                               Radiation
                               Chemicals
                               Infections
                                Hypoxia
                            Physical trauma
                                 Drugs
                           Dietary imbalance
                             MECHANISMS
                        Reaction(s) within embryo
                       Chromosomal nondisjunction
                                Mutation
                       Lack of precursors, substrates
                         Altered energy sources
                            Enzyme inhibition
                          Changed membrane
                             characteristics
                            MANIFESTATIONS
                       Pathogenesis, initiated as one
                         or  more of the following:
                                Cell death
                          Failed cell interactions
                          Reduced biosynthesis
                         Impeded morphogenetic
                                movement
                          Altered differentiation
                                schedules
                            Intrauterine death
                              Malformation
                           Growth retardation
                            Functional deficit
Figure 5.  Diagram showing the relationship between cause, mechanism and
          manifestation of a teratogenic agent (adapted from Wilson, 1973).
                                   77

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PRESENT DEFENSES AGAINST TERATOGENIC AGENTS
  It is possible to envision these existing defenses .against teratogenicity as walls or
hurdles (Fig. 6). The standard teratogenicity testing in pregnant laboratory animals
would represent part of the first barrier. Created only after the devastating effects of
thalidomide in the early 1960s this defense is held by a few to be effective, but the ma-
jority of scientists recognize its severe limitations. Animal tests of new drugs may
have prevented the introduction of new human teratogens. Various aspects of these
tests have been described above. The use of chemical structure and pharmacologic
activity (also a part of this first defense) as a predictor of teratogenicity is theoreti-
cally of great promise, but to date, has seen little practical application except, per-
haps, in  revealing the teratogenic effect of chemotherapeutic agents. Since extreme
variations in drug clearance exist between individuals but not between identical
twins, it is very probable that an individual's pharmacologic defenses are under
strong genetic control. This knowledge could lead to identification and protection of
women who might be more teratogenetically vulnerable than others to common en-
vironmental exposures. Pharmacologic variations in the metabolism of diphenylhy-
dantoin  might account for the small proportion of women in which the drug is tera-
togenic.
   The second defense, early monitoring of the fetus and newborn, is necessary be-
cause the first defenses are still inadequate. Although fetal monitoring and amni-
ocentesis are able to reduce the number of severely  crippling genetic and morpho-
genetic defects, the disadvantage of this form of monitoring is that it is after concep-
tion,  and many people believe that therapeutic abortion is not a morally acceptable
tool.  One would expect that prenatal loss might be a more dramatic and sensitive in-
dex of response to teratogenic agents. By study of the spontaneous losses during the
first trimester, an earlier warning system  might be established that would provide
epidemiologic information some six months  before the effects of a teratogenic agent
would be detected  in  newborns.  Another advantage to the study of spontaneous
 abortuses is that it shortens the period from the time of maternal teratogenic ex-
posure to the time of inquiry. This period may be less than a week as compared to the
seventh- to eighth-month time interval involved when a history is obtained  from the
mother of a newborn.
   Monitoring facilities for defects in neonates exist in many countries. Generally,
only easily recognized  physical defects are  recorded; the larger portion of congenital
diseases (60  percent)  is  identified only after  the newborn period and so  is not
included. Minor changes in  brain function  or long-term carcinogenesis would be
missed.  Although this monitoring system  produces variable results because of
artifacts associated  with data collection, a  continuous registration of time and place
of congenital defects should provide an important warning of teratogenic action by a
newly introduced agent, whether chemical, physical or infectious. In the United
States, a computerized system recording gross congenital defects from 1500  hospitals
(with an average of one million births per  year) is maintained by the Center for
Disease  Control and Commission of Professional Hospital Activities.
   Later  monitoring (defense 3) is illustrated best by the discovery of vaginal carcin-
omas in young women exposed in utero to  diethylstilbestrol. The carcinomas are
thought  to develop  because of misplacement of genital epithelium during  embryo-
genesis.  This defense,  later  monitoring, is manned  generally by the alert medical
practitioner. In Appendix 1  is listed sources  of information available on reproduc-
tive hazards.
   More  than 2,000 new chemicals are synthesized or otherwise produced each year,
and as many  as 200 of these may find their way into the human environment in
measurable  amounts.  These cannot all be  tested.  In Appendix 2,  I outline the
selection of agents to be tested by government agencies and industry, and the way in
which the dangers posed by  teratogens are categorized.
                                     78

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                                                                  Defense III
                                                                late monitoring
                                                 Congenital defects
                                            ,     S&UM^  ~
                                            [Newborni

                                                     9
                                                           Defense II
                                                        early monitoring
                                             Defense I
                                              testing
Figure 6.  Perspective of our major defenses against teratogenic agents. Although the
          in vitro test wall is currently used after animal tests, it should precede animal
          testing in the future (from  Shepard, 1974, used by permission).

 Interpretation of Epidemiologic Findings
   There is evidence that about one-half of all human conceptions terminate in spon-
 taneous abortion, and the reported  incidence of reproductive waste increases in pro-
 portion to the sophistication and care of the interviewers or methods used in  the
 study. This means that any form of publicity will increase the recorded losses and
 give the false impression of an epidemic.

 Factors That Increase False Positive Findings—
   There are many preliminary published reports that associate drugs or other agents
 with congenital malformations. The conclusions drawn from these reports are either
 of  borderline  significance  or  are  produced  by artifacts  of  data  collection,
 questionable  statistical analysis  or both. This incomplete  imformation, released
 through news reports or drug package inserts, may lead to panic in exposed pregnant
 women and their doctors. Subsequent, detailed studies which often do not support
 the original report are either omitted or given only brief mention. For example, sev-
 eral lay publications have created concern and even panic in the minds of women
 using a common antinauseant during pregnancy, through biased and inaccurate re-
 porting, while considerable experience with this drug has supported the opinion by
 most experts that the drug is not teratogenic. Positive rather than negative associa-
 tions are  naturally more likely to be written up by the investigator, and also more
 likely to be accepted by scientific journal editors.
   Regardless of how hard one tries  to be impartial, there is a distinct tendency to be
 overenthusiastic about positive associations and at the same time to be less rigorous
 and critical when searching for malformations  in the control groups. Collecting
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positive and interesting associations can easily create a snowball effect in that the ini-
tial observation, after being broadcast, tends  to attract other case associations; in
this  manner,  the size  of  the  exposed  population  remains  untabulated  and
consequently the true risk is not assessable. Double-blind studies are nearly impossi-
ble to carry out since the person who is being interviewed will generally mention the
presence of his health problems.
  Other false positive biases that occur in the collection of data include improved re-
call of events by parents  of malformed children; increased defect rates found by
multiple observers (for example, the hierarchy of examiners in the university hospi-
tal where at-risk pregnancies are likely to be referred); and an increased rate found
when a full autopsy is performed. These last two factors are especially important
 when considering rate conditions such as diabetes which tend to be seen more often
 in large medical centers. As an example, the newborn of the diabetic mother is more
 likely to die and complete autopsy examinations can inflate the number of malfor-
 mations in this group.
   By use of computers, statistical analyses of many hundreds of associations between
agents and particular malformations can be performed easily. The great majority of
these associations are made without a prior hypothesis. By the laws of chance alone a
small proportion, perhaps five out of each 100, will fall into what is considered to be
statistically significant. These results are  reported frequently in the literature and
cause another increase in false positive associations.
   Another serious drawback in epidemiologic studies is the extremely large number
of cases needed to prove teratogenicity at a certain probability level. This is especially
true where only limited numbers of women are susceptible to the teratogenic action
of a drug. Given the extreme genetic heterogeneity of the human population, there is
always  the possibility that a single individual or a small number of individuals will
 have a heightened  susceptibility that could  lead to excessive accumulation of a
 relatively safe drug  or agent or of a toxic metabolite and consequently, to embryo-
 fetal toxicity.
   Several improvements in the collection and analysis of data have been made re-
cently.  In large studies it is  often possible to match the affected  mother to a cohort
with similar parity, age and social class. In the analysis of data from the Collabora-
tive Perinatal  Project*, a mathematical model was developed  to adjust for con-
founding variables that are related both to rates of drug usage and to malformations
(Heinonen et al., 1977). Another technique that controls memory bias is the use of a
control group of parents who have offspring with types of congenital defects that are
known  to be unrelated to the drug under study. For example, a study of an agent
may include parents of infants with Down's  syndrome as controls. Another more
expensive, but not foolproof way, is illustrated by the work of Milkovich and Van
den  Berg (1974) which linked computer-stored prescription data on mothers to the
findings in their newborns. Some interesting positive associations for meprobamate
and  chlordiazepoxide were found, but another large prospective study based upon
history  taking failed to confirm them (Hartz et al., 1975). It is of interest that in the
computerized study of Milkovich and Van den Berg (1974) all of the significant
prescriptions were written by physicians treating women for conditions other than
pregnancy, while none were given by the obstetrician. This highlights the fact that
mucl^of the teratogenic period of pregnancy may occur before definite diagnosis of
pregnancy is made.
 *A large study earned out by the National Institute of Neurological Diseases and Stroke.
                                     80

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 FUTURE NEEDS FOR DEVELOPMENT OR EXPANSION
   Nearly all the recommendations in this section are dependent upon our ability to
 validate and transmit information. Cooperation of the entire spectrum of our society
 from the school-age child through pregnant women, administrators and scientists is
 needed to compile and digest this information (Appendix 1).

 Short-Term Tests
   Small-animal testing  for  effects on  reproduction by new and other untested
 agents must be continued even though they are not completely predictive for humans.
 Although a number of biologic systems for short-term tests in vitro are mentioned
 above, none has yet met all the criteria needed fora prescreen. These criteria are: (1)
 simplicity, (2) lack of expense, (3) rapidity, (4) based upon multiple mechanisms and,
 most importantly, (5) accurate confirmation of known  and  future human ter-
 atogens. The culture of mammalian embryos in association with activation of com-
 pounds by maternal enzymes seems to come closest at present to filling these criteria.
 The growth period of these cultures needs to be extended beyond embryogenesis in
 order to study late developmental aspects (palate and skeletal formation) and the
 effect on the chorioallantoic placenta.
   The development of short-term tests  for teratogenicity is hampered by the exis-
 tence of multiple mechanisms which contribute to  the molecular and pathogenic
 expression of abnormal development (see Fig. 5). Unlike mutagenesis, and probably
 carcinogenesis, the cellular targets include other organelles as well as the nucleus.

 Linkage  or Association  between  Environmental  Agents  and  Human
 Disease or Disabilities
   In the past 50 years, disease states with short incubations have been fairly easily
 linked with causative factors. There is now a challenge to associate early exposures to
 long-term health events.  For instance, women who  smoke heavily appear to enter
 menopause at an earlier age than non-smokers. Is this also true for women who have
 their only exposure to smoking during prenatal life? Are there any prenatal determi-
 nants to the onset and course of atherosclerosis and  hypertension in old age? What
 percent of childhood malignancies are causally related to prenatal exposures? Beside
 diethylstilbestrol and possibly ionizing radiation, there is some evidence appearing
 which suggests that diphenylhydantoin  may be a transplacental carcinogen.

 Triangulation  from  Existing Data Bases
   Triangulation is a navigational technique which allows a traveler to plot his loca-
 tion and subsequent course by determination of his position in respect to certain
 known points such as stars or coastal markers. Similarly, scientists are finding that
 the cause and prevention of certain disease states can be determined by linkage of
 three fixed, but expanding data bases (Fig. 7). These data bases can be labeled as uni-
 versal identifiers since they are world-wide. They  consist of (1) individuals, (2)
 agents, and (3) disease syndromes. At birth,  an individual should receive a unique
 health identification number to be used throughout life for all health-related records.
 Such a system, must, of course, incorporate proper safeguards for protection of pri-
vacy. An example of how this system could be useful would be the identification and
recording of the health numbers of offspring of women exposed to workplace agents
 of unrealized long-term toxicity during pregnancy. There is a reasonable chance that
 some existing workplace  toxicants will be identified  later as agents which can cross
the placenta and initiate  changes  which, over a long term, will produce cancer or
other pathology in the offspring. Such  a multigenerational  identification system
could foster public health measures to protect the unborn young of exposed preg-
nant women.
                                    81

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                               Data bases for
                             triangular attack on
                             congenital defects
    Individual
  (parent-child)
      Agent
(Chemical, physical,
     genetic)
                                Mechanisms
                                     \
                                Prevention
  Clinical
Identification
  Figure 7.   Diagram of a scheme whereby three data bases (individual, agents and
            clinical syndromes) might be used to determine the mechanisms which
            cause congenital defects and their prevention.
  The second universal identifier system, causative agents, is partly in place since
unique numbers are being assigned to chemicals (Chemical Abstracts Service (CAS)
Registry Numbers). There is a delay in the assignment of CAS numbers to many
physical and infectious agents.
  The area of congenital syndrome identification (syndromology) is providing a
great deal of useful and important data. An example of the utility of specific syn-
drome identification is the  fetal alcohol syndrome which appears to account for a
major part of our population in which a specific cause of mental retardation can be
assigned. McKusick's Catalog of Mendelian Inheritance in Man (1978) represents a
good updated annotated system for labeling human mutations and their associated
syndromes.
  Some disease states have  been controlled without  knowledge  of their cause
because of the discovery of effective treatment. An example of this is the surgical cor-
rection of congenital pyloric stenosis. Congenital rubella is partly controlled by im-
munizing young women, but we do not know the exact mechanism by which the fetus
is in some cases protected by natural defenses. Since we lack the intimate knowledge
of mechanisms of pathogenesis, these two congenital syndromes are still not com-
pletely preventable.
  The concept of linkage of these three data bases was proposed and more fully
discussed in a public document produced for federal legislators, Human Health and
the Environment—Some Research Needs (Nelson and Whittenberger, 1977).

Identification and Investigation  of Teratogenic Outbreaks

The Life Span of an Epidemic—
  The time course of an epidemic can be plotted in sequence from the introduction of
the  causative agent through hypothesis testing to removal of the agent (Fig. 8). The
time course of the thalidomide epidemic in different countries varied from two years
to  over four years. The  process was lengthened by the then slow international
                                     82

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      Life span of an epidemic
              Recognition
                  of
               epidemic
Rorschach
  effect
 multiple
hypotheses
      Etiologic
       agent
     introduced
Hypothesis
  testing
         Acceptance by
           scientific,
          government
            and lay
  Cause  communities
associated
                                          Exposure
                                          stopped
                                          resolution
                                         of epidemic
                                   Time
 Figures.  Events between the introduction of a new agent causing widespread
          disease and the ending of the epidemic. The vertical plot indicates the
           number of cases. The Rorschach stage is named after the psychiatric test
          where seemingly unstructured ink blots take on meaningful shapes when
          studied. The puzzling circumstances associated with an  early outbreak
          are equally without meaning until hypotheses are generated and studied
          (modified from Oakley, 1976).
exchange and assimilation of scientific reports and by delay in mechanisms leading
to removal of the drug from the market. Much improvement has since occurred in
these two areas. An example of the slow association between a clinical syndrome and
an agent is that of the oral anticoagulants (Warkany, 1976). These dicoumarin anti-
coagulants were associated with two case reports of infants born with very small
noses in 1968. The rare linkage between the use of anticoagulants during pregnancy
and the unusual reduction in the size of the nose should have signaled an association
or at  least a concerted search for further supportive evidence. Instead, it was not
confirmed until 1973 when additional associations were finally brought to light at a
meeting dealing with malformation syndromes. Parenthetically, it is of interest that
an animal model for this human teratogen does not exist.  The association between
alcoholism and the fetal alcohol syndrome was delayed literally for centuries. These
examples emphasize the continued need for sound monitoring of our population in
order to make epidemics visible against the background of malformations and also
for  exchange  of  information  between  experimental   teratologists,  clinical
syndromologists and regulatory agencies.

A Proposal for Triage, Investigation, and Management of Toxicologic Outbreaks—
  Since such  a  system should combine all  toxicologic  dangers  (organ-specific
carcinogenesis, mutagenesis and teratogenesis), the phrase toxicologic is used  here.
An executive secretary and small permanent staff should be located geographically
and administratively near to the National Toxicology Program, Center for Disease
Control or other federal body. These resource people should be consulted by tele-
phone for purposes of triaging new reports of hazards and for appointment of ad hoc
committees to validate, investigate and take action on toxicants. Active members of
relevant research societies should  be called on to help in these  processes.
                                     83

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 ACKNOWLEDGMENTS
   Thanks are due to Ms. Barbara Brownfield for her help with editing and producing
the manuscript. The author thanks his coworkers and several teratologist friends for
their original ideas incorporated into this chapter. Drs. Alan Fantel, Philip Mirkes
and Trent Stephens offered valuable criticisms.
   The manuscript is respectfully dedicated to Dr. James G. Wilson in thanks from a
student and admirer.
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Brinster, R.L. Teratogen testing using preimplantation mammalian embryos. In Methods for
    Detection of Environmental Agents  That Produce Congenital Defects, edited by T.H.
    Shepard, J.R. Miller, and M.  Marois, North-Holland, Amsterdam; American Elsevier,
    New York, pp. 113-124, 1975.
Brinster, R.L., and P.C. Cross. Effect of copper on the preimplantation mouse embryo. Nature
    (London) 235:398-399, 1972.
Carter, S.B. Effects of cytochalasins on mammalian cells. Nature (London)213:2d\-2M, 1967.
Chatot, C.L., N.W. Klein, J. Piatek, and L.J. Pierro.  Successful culture of rat embryos on
    human serum: use in the detection of teratogens. Science 207:1471-1473,  1980.
Donahue, R.P. Chromosomal anomalies and the meiotic divisions of the oocyte. In Methods
   for Detection of Environmental Agents That Produce Congenital Defects, edited by T.H.
    Shepard, J.R. Miller, and M.  Marois, North-Holland, Amsterdam; American Elsevier,
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Ebert, J., and  M. Marois. (eds.) Tests of Teratogenicity in vitro. Proceedings of the Woods
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Fantel, A.G., J.C. Greenaway, M.R. Juchau, and T.H. Shepard. Teratogenic bioactivation of
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Federal Register 44 (124):37462-37466, June 26,  1979.
Fraser, F.C. Gene-environment interactions in the production of cleft palate. In Methodsfor
    Teratological Studies in Experimental Animals and Man, edited by H. Nishimura, J.R.
    Miller,  and M. Yasuda, Igaku  Shoin Ltd., Tokyo, pp. 34-49, 1969.
Gorlin, R.J., J.J. Pindborg, and M.M. Cohen, Jr. Syndromes of the Head and Neck, 2nd ed.,
    McGraw-Hill Book Co., New York, 1976.
Hale, F.  Relation of maternal vitamin A deficiency to  microphthalmia in pigs.  Tex. State J.
    Med. 33:228-232, 1937.
Hartz, S.C., O.P. Heinonen, S. Shapiro, V. Siskind, and D. Slone. Antenatal exposure to
    meprobamate and chlordiazepoxide in relation to malformations, mental development and
    childhood mortality. New Engl. J. Med.  292:726-728,  1975.
Heinonen, O.P., D. Slone, and S. Shapiro. Birth Defects and Drugs in Pregnancy, Publishing
    Sciences Group, Littleton, Mass., 1977.
Hertig, A.T. The overall problem in man. In Comparative Aspects of Reproductive Failure,
    edited by K. Benirschke, Springer-Verlag, New York,  1967.
Hunt, V.R. Occupational Health Problems of Pregnant Women. A report and recommenda-
    tions for the Office of the Secretary of the Department of Health,  Education and Welfare,
    1975. Copies available from C.G. Schiffer, Rm. 5139, H EW-North, 330 Independence Ave.
    S.W., Washington, D.C., 20201.
Kelsey, F.O. Present guidelines for teratogenic studies in experimental animals. InCongenital
    Defects: New Directions in Research,  edited by D.T.  Janerich,  R.G. Skalko, and I.H.
    Porter,  Academic Press, New York, pp. 195-204, 1974.
McKusick, V.A. Mendelian Inheritance in Man. 5th ed.. The Johns Hopkins University Press,
    Baltimore, 1978.
Milkovich, L., and B.J. Van den Berg. Effects of prenatal meprobamate and chlordiazepoxide
    hydrochlonde on human embryonic and fetal development. New Engl. J. Med. 297:1268-

Moscona, A. A. Invited discussion: Embryonic cell and tissue cultures as test systems for terato-
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   Defects, edited by T.H. Shepard, J.R. Miller, and M. Marois, North-Holland, Amsterdam;
   American Elsevier, New York, pp. 103-107, 1975.
Nelson, N., and J.L. Whittenberger. Human Health and the Environment-Some Research
   Needs. Report of the Second Task Force for Research Planning in Environmental Health
   Science, DHEW Publication No. NIH 77-1277, 1977.
New, D.A.T. Whole embryo explants and transplants. In Handbook of Teratology, Vol. 4, ed-
   ited by J.G. Wilson, and F.C. Fraser, Plenum Press, New York, pp. 95-133, 1978.
Oakley, G.P., Jr. Birth defect surveillance in the search for and evaluation of possible human
   teratogens. Birth Defects: Orig.  Artie. Ser.  12 (5): 1-6,  1976.
Palmer, A.K. The design of subprimate animal studies. In Handbook of 'Teratology, Vol. 4, ed-
   ited by J.G. Wilson and F.C. Fraser, Plenum Press, New York,  pp. 215-253, 1978.
Rogers, B.J., H. Van Campen, M. Ueno, H. Lambert, R. Bronson, and R. Hale. Analysis of
   human spermatozoal fertilizing ability using zona-free ova. Fertil  Sleril. 52:664-670, 1979.
Schardein, J.L. Drugs as Teratogens, CRC Press, Cleveland, 1976.
Shepard, T.H. Teratogenicity from drugs—an increasing problem. Disease-a-Month, June: 1-
   32, 1974.
	A Catalog of Teratogenic Agents, 3rd ed., Johns Hopkins University Press,  Bal-
   timore,  1980.
Shepard, T.H., and A.G. Fantel. Embryonic and early fetal loss. Clin. Perinatal. (5:219-243,
   1979.
Shepard, T.H., and D. Pious. Cell, tissue and organ culture as teratologic tools. In Handbook
   of Teratology, Vol.4, edited by J.G. Wilson  and F.C. Fraser, Plenum Press, New York, pp.
   71-93, 1978.
Smith, D.W. Recognizable Patterns of Human Malformation, 2nd ed., W.B. Saunders Co.,
   Philadelphia, 1976.
Staples,  R.L. Teratogens and the Delaney Clause. Teratology 10: 1, 1974.
Teratology Society. Teratology 21:  A1-A77, 1980.
Warkany, J. Congenital Malformations: Notes and Comments, Year Book Medical Publish-
   ers, Chicago,  1971.
	 Warfarin embryopathy. Teratology 14:205-209, 1976.
Warkany, J., and R.C. Nelson. Appearance of skeletal abnormalities in the offspring of rats
   reared on a deficient diet. Science 92:383-384, 1940.
Whorton, D., R.M.Krauss, S. Marshall, and T.H. Milby. Infertility in male pesticide workers.
   Lancet 2:1255-1261, 1977.
Williamson, E.M., and J.F. Miller. A prospective study into early conceptual loss. Clin. Genet.
   17: 93, 1980.
Wilson, J.G. Environment and Birth Defects,  Academic  Press, New York,  1973.
	 Critique of current methods for teratogenicity testing in animals and suggestions
   for their improvement. In Methods for Detection of Environmental Agents That Produce
   Congenital Defects, edited by T.H. Shepard, J.R. Miller, and M. Marois, North-Holland,
   Amsterdam;  American Elsevier, New York, pp. 29-48, 1975.
             Feasibility and design of subhuman primate studies. In Handbook of Teratology,
   Vol. 4, edited by J.G. Wilson and F.C. Fraser, Plenum Press, New York, pp. 255-273, 1978.
Wilson, J.G., and F.C. Fraser (eds.) Handbook of Teratology, Vol. 1-4, Plenum Press, New
   York, 1978.
APPENDIX 1:

SOURCES OF INFORMATION ON  REPRODUCTIVE HAZARDS
Publications
  An excellent general introduction to and discussion of teratology is available in
Wilson's Environment and Birth Defects (1973). The annotated A Catalog ofTera-
togenic Agents (Shepard, 1980) includes about 1100 agents that have been studied
for teratogenesis in animals and man. Schardein's 1976 book on teratogenic drugs is
another useful source. Handbook of Teratology edited by  J.G. Wilson and F.C.
Fraser (1978),  a comprehensive  set of 4 volumes,  summarizes  many general and
specific topics  as well as techniques important in teratology.
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  Heinonen et al. (1977) reported on the outcome of more than 50,000 pregnancies in
which drug exposures were known and listed their risk rates. Hunt (1975) has ad-
dressed problems of pregnant women in the work place. Congenital Malformations:
Notes and Comments (Warkany, 1971), Recognizable Patterns of Human Malfor-
mations (Smith, 1976)  and Syndromes of the Head and Neck (Gorlin et al., 1976)
help to define and extend the description of human defect syndromes. McKusick's
Mendelian Inheritance in Man (1978) includes more than 3000 congenital syndromes
produced by gene mutations.

Computerized Information Retrieval
  The National Library of Medicine (NLM) maintains a number of data bases in-
cluding the computer  service, Toxline,  which lists  references  dealing with toxic
properties. These  listings  are  on line to  most regional medical libraries. The
chemicals are stored by CAS numbers (Chemical Abstracts Service Registry Num-
bers), and in one or two days it is possible to have a listing of articles dealing with the
reproductive  toxicologic aspects  of certain chemicals. The Environmental Tera-
togen Information Center (ETIC), maintained by the National Institute of Environ-
mental Health  Services, contributes to this service (Toxline) and also maintains its
own answering service at  Research Triangle Park, N.C.

Answering Services  for Public and Professionals
   Individuals and groups dealing with exposed persons often find computerized sys-
tems described  above too slow or insufficiently detailed to provide data on reproduc-
tive dangers. These questions often deal with the advisability of pregnancy termina-
tion or transfer of pregnant women in the work place. Pertinent texts may not be
available to those  asking questions. As a result, a number of telephone answering
services are maintained by teratologists who can either direct the caller to recent liter-
ature or  to other professionals.  Names and addresses of members of the Teratology
Society,  along  with their geographic location, are given in each December's issue of
the journal, Teratology.

Exchange of  Scientific Information
   A large and inaccessible area of scientific data is that of negative findings. Pharma-
ceutical companies often fail to release results of safety tests of their new products. In
addition, scientific journals do  not publish many manuscripts on agents which are
not  toxic in  some  way. In Japan, industries  and others are encouraged by the
government to publish their safety tests and at least one journal,  Oyo  Yakuri,
publishes these results.
   Scientific exchanges with other countries are recognized as important. As a result
of this, the computerized retrieval of literature references on toxicologic substances
(Toxline) of the National Library of Medicine is available through a number of out-
lets in Western  Europe, Central and South America and Africa. The library tapes are
also accessible  in Japan and Australia. These countries  contribute in a significant
way to collection and validation of these data bases, particularly with scientific arti-
cles  originating in their own countries.  Printing of texts  by computer  and other
means of reducing the publication time and cost of scientific information should be
fostered.

Training
   Most medical students have only modest exposure to toxicology and over one-half
of the medical schools in the United States have no specific courses or trained faculty
lor teaching teratology. It is interesting that those schools with very strong programs
in developmental biology seem  to be the weakest in teratology,  which in itself is a
form of applied developmental biology. Human embryology should be reinstated as
a medical school requisite.
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APPENDIX 2:
SELECTION OF AGENTS TO BE TESTED

Government Selection
  A number of existing federal regulatory agencies have their own investigative ca-
pabilities. The larger of these agencies include the Center for Disease Control (CDC),
Environmental Protection Agency (EPA), Food and Drug Administration (FDA),
National Cancer Institute (NCI), the National Institute of Child Health and Human
Development (NICHD),  National  Institute of Environmental Health  Sciences
(NIEHS) and the National Institute of Occupational Safety and Health (NIOSH).
Other departments of government, such as Transportation, Labor, Energy, Defense
and Agriculture, also foster some teratologic work.  The National Center for Toxi-
cologic Research (NCTR) in Arkansas, funded  through the  FDA and EPA, has a
mandate to develop new tests for toxic substances and, in particular, to do long-term
low-dose experiments in large numbers of experimental animals (referred to by some
as "mega mouse" studies). All of these agencies choose agents which they believe to
be important to their regulatory or mandated programs. A recent development in
toxicologic testing has been the formation of a National Toxicology Program (NTP)
for selecting and testing chemicals and developing new test methods. A chemical
selection unit located at NCTR prepares summaries  of data on agents which may be
nominated by government, industry or private individuals. The assigning of priori-
ties and testing of these chemicals is performed under  the NTP.
  The Toxic Substances Control Act (TSCA) charges the EPA to identify and test a
specific  number of chemicals and some of these tests will be of teratogenic nature.
Training money for toxicologists was included in this legislation.

Industry  and Academia
  Many pharmaceutical and  larger chemical producers have elaborate facilities for
teratologic testing of their products. Although many of these results are published,
some  degree of secrecy enters due to the competitive nature of the enterprise. The
faculty of the universities and other research units contribute many of the new obser-
vations and techniques to teratology as well as acting as consultants to both indus-
try and  government.

Categorization of the Danger of Teratogenic Agents
  Some methods of regulating exposure to  known or suspected teratogens have
been developed. The FDA has published five categories of danger for drugs that may
be taken by pregnant women (Federal Register, 1979). The classificatioin is based
upon animal and human exposure and takes into account the absence of toxicologic
information (Table 1). The FDA recommends that where data are available they be
summarized and referenced in the accompanying package insert for  the drug.
  The International Agency  for Research in Cancer (IARC) of the World Health
Organization has started to review reproductive  and teratologic data and assess the
hazards of selected chemicals. This organization produces many useful publications
based upon ad hoc committees drawn from experts in the field of carcinogenesis,
mutagenesis and teratogenesis.
  Since the Delaney Clause was passed by Congress in 1958 forbidding the  use of
known animal carcinogens in food substances, a similar exclusion of teratogens was
considered. Because  nearly every agent  in  sufficiently large doses  can produce
embryo-fetal toxicity, it seemed unwise to have a similar regulation for teratogens.
In one of the very few political activities taken by the Teratology Society, the mem-
bership  was  surveyed  on  this question  and the  overwhelming opposition was
recorded in several scientific  journals (Staples, 1974).
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Table 1.  Categories of Danger of Teratogenic Agents (Federal Register,
         1979).
Category
a
b

c


d


X



Results of
Animal Study

Negative
Positive
Positive


Negative or
unknown

Positive



Results of Human
Pregnancy Exposure
Negative
Unknown
Negative
Unknown


Positive


Positive



Use During
Pregnancy
Use if necessary
Use if necessary
Use if necessary
Use if the benefit
outweighs the
risk
Use for life-
threatening
situations only
Contraindicated
for use: risk
clearly outweighs
benefit.

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     SHORT-TERM  TESTS FOR RENAL  TOXICITY

                            David S. Miller

                                    and

                        Anthony M. Guarino


INTRODUCTION
  In "Seven Gothic Tales," Dinesen (1972) defined man as a ingenious machine de-
signed to turn, with "infinite artfulness," red wine into urine. Human kidneys pro-
cess about 1600 liters (400 gallons) of blood per day and produce about one liter of a
highly specialized concentrated fluid known as urine. The kidney accomplishes some
of the most important functions necessary for survival: it excretes waste products of
metabolism and xenobiotics (chemical substances foreign to  the body); it regulates
levels of salts, water and, to some extent, nutrients in body fluids; it maintains the
appropriate acid-base balance of blood; and it serves as an endocrine organ (secre-
ting  hormones such as erythropoietin, renin,  and prostaglandins). The  elaborate
physiologic functions that the kidney performs require a high degree of structural
and functional complexity. Certain widespread and chronic environmental pollu-
tants—for  example,  heavy  metals—clearly  affect renal function, and  simple,
short-term  tests of renal toxicity are needed to protect human health. In order to
design appropriate tests, we must possess detailed knowledge  of normal renal physi-
ology and biochemistry so that appropriate indices of renal function can be estab-
lished. Also, we must recognize the sites and mechanisms of  action of nephrotoxic
chemicals so that sensitive targets can be identified. Ideally, in both cases, our knowl-
edge would extend from the whole organ down to molecular levels of organization.
Such is not the case at present. Indeed, little attention has been focused on the devel-
opment of simple, widely applicable tests for renal toxicity, although some fairly so-
phisticated  new procedures, such as those using isolated cells and  subcellular frac-
tions, show great potential as short-term tests.

KIDNEY  FUNCTION
  In order to appreciate fully the difficulties encountered in designing test systems
for renal toxicity studies, one must consider the complex anatomy, biochemistry and
phsiology of the normal kidney. The vertebrate kidney contains renal tubules, blood
vessels, lymphatics and interstitial tissue, and each plays a role in  the various pro-
cesses that  ultimately result in the production of urine. The composition  of final
urine is a result of the (1) functional interactions between anatomical elements; (2)
water and solute  transport characteristics of the cellular membranes of glomerular,
The Authors: David S. Miller and Anthony M. Guarino both worked at the Mount Desert Island Biological
Laboratory, Salsbury Cove, Maine. Dr. Guarino is also affiliated with the National Center for Drugs and
Biologies, U.S. Food and Drug Administration, Rockville, Maryland.
                                     89

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vascular and tubular tissue; and (3) control mechanisms both from within the kidney
and from without (see reviews by Orloff and Berliner, 1973; Brenner and Rector,
1976).
  The human kidneys are paired, bean-shaped organs that are located in the rear of
the peritoneal cavity, alongside the vertebral column. Each kidney is supplied by a
single renal artery and drained by a single renal vein and  a ureter. Two distinct
regions can be identified: a darker outer region, the cortex, and a pale inner region,
the medulla. The structural and functional unit of the kidney is called the nephron,
and consists of the glomerulus-Bowman's capsule, proximal tubule, loop of Henle,
distal tubule and collecting duct and their respective blood supplies (Fig. 1). There
are about  one  million nephrons  in each kidney. The glomerular apparatus and
proximal and distal segments lie within the renal cortex; the loop of Henle and col-
lecting ducts lie within the medulla.
   Urine  formation by the kidney involves the movements of water and  solutes
through selectively permeable cellular membranes. The permeability characteristics
vary with the region of the nephron and are a  direct result of the arrangement of
lipids and  specialized proteins within cellular membranes. These proteins perform
structural, enzymatic (metabolism or transport) or receptor functions. Since the lipid
core of the membranes has a hydrophobic character, small water soluble molecules,
such as ions, sugars and amino acids, can only pass slowly through the lipid  regions
of the membrane, but move more readily through protein-lined aqueous pores or
with the aid of specific carrier proteins. Certain substances may be"pumped"across
the  membrane against  their electrochemical  potential ("uphill"  transport) by
transport proteins  that utilize metabolic energy stored in adenosine triphosphate
(ATP) or in transmembrane ion gradients. Thus, an exothermic process (splitting of
ATP by transport ATPase or "downhill" transport event) is  used to drive  a coupled
endothermic process (uphill cellular accumulation of K+, glucose or amino acids).
At the molecular level, evidence  indicates  that certain widespread environmental
contaminants, such as heavy metals, can selectively modify passive and metabolically
driven permeability characteristics of renal cell membranes,  thus  impairing the
membranes' ability to conserve proteins and nutrients, excrete metabolites and
maintain  salt and water balance.

Blood Supply
   Total blood  supply to the kidneys is extremely high; in a  70 kg man the 300 g of
renal tissue receives 20-25 percent of the cardiac output. Under normal conditions,
the intrarenal distribution of blood flow is not  uniform; it decreases progressively
from the outer cortical to the inner medullary regions. Thus, the outer regions of the
kidney arc better oxygenated than the inner regions and the proportion of oxidative
to glycolytic metabolism of the cells within these regions reflects the differences in
blood supply.  In each kidney, the renal artery divides several times to form  smaller
arteries, arterioles and eventually one or more capillary beds at the glomerulus and
around the renal tubules. This double capillary bed arrangement is unusual, but not
unique to the  kidney. From the tubular capillary beds,  blood flows into the renal
venous system  and  returns to the systemic circulation at the inferior vena cava.

Glomerular Filtration
   One anastamosing capillary bed is contained  within the Bowman's capsule. The
outer walls ol'the porous capillary endothelium are lined with a basement membrane
which, in  turn,  is covered by the epithelium that lines the urinary space. The entire
apparatus functions as a submicroscopic sieve, producing an  ultrafiltrate of blood in
the urinary space (Fig. 2). Ultrafiltration  is powered  by hydrostatic pressure in the
glomerular capillaries, a direct result of work done by the pumping heart.  The ultra-
filtraie in Bowman's space is nearly identical in composition to plasma (cell-free


                                     90

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blood), except for the nearly complete absence of proteins. The glomerular sieve per-
mits some low molecular weight proteins to pass into the urinary space, but these
proteins are generally reabsorbed in the proximal tubule, so that, normally, the final
urine is essentially protein-free. Pathological conditions which might affect blood
pressure (hypertension) or decrease plasma oncotic pressure (caused, for example,
by liver disease) clearly can alter glomerular filtration rates; increased filtration pres-
sures may alter the  ultrastructure  and filtration characteristics of the glomerular
sieve.
  Arcuate
   artery
                                      Glomerulus
                                               Bowman's
                                                capsule
                                                          Proximal tubule
                                                                  Distal
                                                                  tubule
                                                                  Collecting
                                                                    tubule
                                                                   Loop of
                                                                    Henle
                                                                      Thin
                                                                     segment
                  Vasa
                  recta
                                                                     Pelvis
 Figure 1.   Diagrammatic representation of the relationships between the component
            parts of the nephron and associated blood vessels. Actual spatial
            relationships  have been greatly simplified. Adapted from Guyton (1976).
                                       91

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                   Capillary endothelial cell
                     I
                             Basement membrane
                              1
    Fenestra
                                                        Slit-pore
   Figure 2.   Simplified diagram of the glomerular filtration apparatus. Adapted
             from Guyton (1976).
Tubular Reabsorption and Secretion
  The proximal tubule modifies tubular fluid primarily by (1) reabsorbing essential
substances and either utilizing them intracellularly or passing them on to the peritu-
bular capillary  nets; and (2) clearing the peritubular circulation of excretory pro-
ducts and secreting them into the tubular fluid (Fig. 3). In the proximal tubule,
nearly all the filtered organic nutrients are reabsorbed, most by specific, metabol-
ically powered transport proteins that  are  embedded in the luminal  plasma
membrane. Two-thirds of the filtered NaCl  and water are reabsorbed in this segment,
                                     92

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   Glomerulus
   Proximal tubule
                                                         Ultrafiltration
                                              NO+,C|-,H2O,HCO-3, K+,PO4-3
                                              sugars, amino acids,
                                                proteins, uric acid
                                              H + , organic acids,
                                                  and bases
                               \
    Loop of Henle
                                           NaCI
    Distal tubule
                                              Na+,CI-
                                              H2O
                                              H+.K+
                                              NH4+
    Collecting duct
Na+.Ch
H20
                                                                      NH4+
Figure 3.  Simplified summary of the movement of water and solutes in the different
          regions of a model nephron. Arrows show direction of the transfer from or to
          luminal fluid. Modification  of the composition of luminal fluid as it passes
          through each segment of the nephron is indicated.
                                     93

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as are substantial amounts of K+, Ca2+, and phosphate ions, whilst H  ions are secret-
ed into the urine to maintain the body's acid-base and nitrogen (NHs) balance. NaCl
transport across the tubular epithelium is driven by an ATPase (Na, K-activated
ATPase) and the accumulation of NaCl near the basal and lateral surfaces of the
cells draws water from the tubular lumen towards the interstitial spaces (osmotically
coupled salt and water transport). Proximal tubular secretion of organic acids and
bases removes normal metabolites, drugs and other xenobiotic compounds and their
metabolites  from the blood.
  The loop  of Henle is a consistent anatomical feature of the kidney  of all animals
that produce a urine more concentrated than their  body fluids.  Because of the
hairpin bend at the end of the loop and the arrangement of passive and metabolically
driven salt transport processes within the loop, this portion of the nephron functions
as a countercurrent multiplier, creating an osmotic gradient in the tubules, interstitial
fluid and blood  vessels  (Fig. 4). The  primary interstitial  solute is  urea and the
osmotic gradient increases deeper into the medulla. This gradient is the driving force
for the removal of water from the fluid in the collecting ducts thus producing a con-
centrated final  urine. The osmotic gradient, hence, the final urine concentration, can
be modified at the "loop" level by diuretic drugs and certain heavy-metal compounds
which act on specific transport proteins in the cellular membranes of the thick as-
cending  limb.
   Tubular fluid that passes from the ascending limb of Henle to the distal tubule is of
low osmolality (Fig, 4). In the distal segment, NaCl and water are reabsorbed and K+,
H+ and N H/ are secreted. Dilute urine entering the collecting ducts then passes once
more through the mudulla where the reabsorption of  water occurs. This process is
promoted by an antidiuretic hormone (ADH) from the pituitary which increases the
permeabilities  of the collecting duct epithelium to water and urea. Water is drawn
osmotically from the collecting-tubule fluid by the steep medullary osmotic gradient
created by the countercurrent multiplier system in the loop of Henle. Urea, the major
solute of the final  urine, equilibrates with the  medullary interstitium. In well-hy-
drated individuals,  circulating ADH levels are low and a dilute urine is produced. In
dehydrated  individuals, circulating ADH levels are high, and a more concentrated
urine is produced (Fig. 4).

RENAL TOXICOLOGY

Target Organ and Critical Organ Concepts
   Over the past decade, increasing attention has been  focused on the detection and
evaluation of toxicity as manifested by alteration of individual organ function. One
must recognize the individual role each organ plays in maintaining homeostasis, that
is, constant extracellular  and intracellular levels of ions, nutrients, excretory pro-
ducts and gases, so that enzymes, cells, tissues, and whole organisms can function
optimally. At each  organizational level, the role of excretory organs,  especially the
kidney, is pivotal (see reviews byOppelt, 1970; Beliles, 1975; Foulkesand Hammond,
1975; Maher,  1976; Foulkes,  1977; Magos and Clarkson, 1977). Through homeo-
static mechanisms the organism usually is able  to adapt to environmental changes.
Indeed, it is remarkable that the  kidneys  have a reserve that allows  normal renal
function  even when compromised to greater than  50  percent of original capacity.
  One early clue to  the potential site of toxicity of a particular agent is its localization
in certain cells and organs; this is a signal to focus further on the association between
tissue accumulation and adverse effects. Particularly in studies  of heavy-metal con-
taminants, it has often been shown that undesirable functional changes appear to-
gether with renal accumulation of the toxicant. These effects are generally associated
with a critical concentration of toxicant within the cell; increases beyond this critical
level cause cell death, thus, defining the lethal concentration for that cell Analogous
terminology is  applied to organs, with  the critical organ concentration being the
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      1200 _
        900 _
        600-
        300
        200 _
        100 _
          0-1
                  Proximal
                  tubule
Collecting
 tubule
Figure 4.  Concentration of total solutes in tubular fluid as a function of segment
          of the nephron. Increases in osmolarity indicate net secretion of solutes into
          the tubular fluid; decreases indicate net reabsorption of solutes. Iso-osmotic
          movements of fluid are not shown. The shaded area indicates that portion of
          the final urine osmolarity that is under ADH control. Adapted from Guyton
          (1976).
mean concentration in the organ at the time that any of its cells reach their critical
concentration. The concept of a critical organ refers to that particular organ which
first attains  its critical  concentration of the  pollutant;  the organ  of greatest
accmulation is not necessarily the critical organ.
  It is rare to find a chemical which affects the function of a single organ. Overall
toxicity must be considered to be a result of the complex relationships between expo-
sure conditions (uptake,  distribution, and excretion of toxicant) and cellular and
nutritional factors which might affect toxicity in several  organs. Furthermore,
subcellular partitioning among various organelles and  intracellular fluids may also
be of considerable  importance. For example, it is known that specific proteins whi-'h
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have preferential affinity for some metals, such as cadmium, are present in cells.
These proteins bind the metal so that the cadmium is unable to interact with normal
cellular constituents; this may constitute a major, natural detoxification mechanism.
Because  of these  relationships, it is currently extremely difficult to formulate
practical standards for exposure or tissue levels with regard to renal toxicity alone.
Indeed,  interspecific  and  individual differences  in  normal biochemical  and
physiological processes, as well as in patterns of toxicant uptake and distribution, can
be so great as to make one organ critical for one individual and a different organ crit-
ical for another. In setting standards in certain areas, the Environmental Protection
Agency (EPA) has therefore placed in high-risk categories individuals who might be
extra sensitive to certain  pollutants. These considerations show that it is of impor-
tance to determine which organs and which groups of cells within them are most sus-
ceptible.
  The kidney is clearly one important target of chemical toxicity because (1) renal
blood-flow rates are especially high, thus exposing tissues rapidly to a large fraction
of circulating toxicant; (2) filtered toxicants may be concentrated in tubular fluid as
salts and water are reabsorbed, exposing luminal membranes to high concentrations
of toxicants; (3) secreted toxicants may be concentrated in the interstitium,  tubular
cells and tubular fluid; and (4) renal tissue contains many chemically reactive sites,
such as sulfhydryl  groups, that  are functionally important.
   Nephrotoxic agents can be divided into those that affect the four major morpho-
logic components of kidney: glomeruli, tubules, interstitial tissues, and blood vessels.
This approach can be useful in that the early manifestations of lesions affecting each
of these sites tend to be distinct.  Further, there  often is site specificity such that
glomerular lesions are most frequently mediated through immunologic mechanisms,
whereas tubular and interstitial effects are more commonly associated with toxic or
infectious  agents. On the  other hand, it must  be emphasized that because of the
anatomic interdependence of the components of  the kidney,  damage to one com-
ponent will invariably induce secondary changes  to the others.

Glomerular Toxicity
   Few environmental chemicals affect the glomerulus compared with the large num-
ber which  affect the other three sites. Nonetheless, there are some agents which can
cause  glomerulonephritis as a result of immunologic-type reactions. A few drugs
such as hydralazine, phenylbutazone, and sulfonamides cause glomerulonephritis,
and a number of biologic products can also institute acute renal failure by this mech-
anism. Vaccines, plant poisons, and antigens, elaborated as a result of drug-induced
hypersensitivity, induce anaphylactic allergic reactions and, thus, a proportion of
their nephrotoxicity is likely to  be caused through  glomerular lesions.


Tubular and Interstitial Toxicity
  Toxicities to  the tubules tend to involve local cytotoxic action, enzyme inhibition
and  osmotic disruption.  The structure of renal tubular cells varies throughout the
nephron, and Correlates somewhat w.ith the functional role of the tubular segment in
question. Thus, the proximal tubular cells have a highly developed structure, with
long microvilli, many mitochondria and extensive intercellular interdigitations,
which  are correlated with the major transport functions of this segment, namely re-
absorption of about  70  percent of filtered sodium and water as well as a large
proportion of filtered glucose, potassium, proteins, phosphate and amino acids.
There  is considerable evidence that a sodium pump is located in the basal lateral
membranes of the proximal tubules, whose operation is mediated through the mem-
brane-bound Na,K-ATPase and the  adjacent mitochondria (which supply the re-
quired  energy  through  oxidative  phosphorylation).  Against  this  specialized
morphologic and biochemical background, it is no surprise that the proximal tubule
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is highly vulnerable to a large number of xenobiotics. Furthermore, since a number
of toxicants are reabsorbed or secreted by the proximal tubule, cells may be exposed
to particularly high levels of toxicants.
  The interstitium is an additional component of the kidney which can be affected
by nephrotoxic agents. It can be considered as those tissue structures remaining after
discussion of the glomeruli, tubules, and blood vessels; the interstitial space contains
capillaries, lymphatics, and some fibroblast-like cells. Interstitial fluid contains the
solute gradients which are essential for the countercurrent production of urine. Thus,
the roles of the tubule and interstitium are highly cooperative and they both are the
common sites of the effects of toxic  xenobiotics.

Tubular and Interstitial Nephrotoxins
  We will briefly discuss current knowledge on the renal effects and mechanisms of
toxicity for a selected group of nephrotoxic substances. A more complete listing is
given in Tables 1 and 2.
Analgesics—
  While of no direct concern to EPA, this class of nephrotoxic drugs is mentioned
for two reasons. First, the lesions are quite typical of those seen with a number of
other agents. Second, since these drugs are used extensively, one must anticipate that
actual nephrotoxicity in a particular individual may be the result of exposure to not
only environmental contaminants but also to analgesics and other drugs. Thus, we
must consider substances in the working environment and in food and water, as well
as nephrotoxic solvent exposure from hobby activities. The actual lesions induced
by  analgesics  involve both the tubules and interstitium;  they are classified as
tubulointerstitial toxic agents. Morphologically, there are cortical changes, consist-
ing of loss or atrophy of tubules, interstitial fibrosis, and inflammation, which seem
to be secondary to  obstructive atrophy caused by tubular damage in the papillae.
There seems to be more extensive lesions in patients or animals where combinations
of analgesics are used. Some recent studies have shown that the phenacetin metabo-
lite, acetaminophen, is an oxidant, and since it is concentrated in the renal medulla
it is thought to cause oxidative damage to renal cells. Not  only are each of these
chemicals available as analgesics but they usually are taken in combination with as-
pirin. Aspirin is known to inhibit prostaglandin synthesis; it may potentiate neph-
rotoxic effects by blocking the usual vasodilatory effects of prostaglandin, and there-
by predisposing renal tissues to ischemia. The papillary damage is likely to be due to
a combination of direct cytotoxic effects of phenacetin and its metabolites, as well as
to the indirect effects of ischemic injury to both tubular cells and blood vessels.
  The list of over-the-counter analgesic ingredients includes the following subtances
which may be implicated in causing or being  associated with nephropathies:
phenacetin, salicylamide,  acetaminophen, caffeine and, in many cases,  metabolites
of these drugs. Obviously, to this list, one must also add aspirin although its toxicity
may not be as severe as some of the others. Interestingly, aspirin may potentiate
toxicity through yet another mechanism since it can uncouple oxidative phosphoryla-
tion, thus decreasing the energy  available for important transport functions of the
tubules, for example, to energize Na,K-ATPase.

Mercurials—
  A number of drugs and environmental contaminants, including diuretics, several
inorganic salts of mercury, and fungicides, fall under this category. Mercuric chlo-
ride (bichloride of mercury or corrosive sublimate) was used in mediaeval times to
poison one's enemies, death being induced by acute renal failure. The most impres-
sive microscopic changes occur rather  specifically in the proximal convoluted tu-
bules; they are dilated with degeneration, fragmentation, and necrosis of epithelial
cells. Less striking changes occur in the ascending limbs of Henle, distal convoluted
tubules and collecting tubules. Earlier signs of toxicity include cytoplasmic vacuola-

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Table 1.   Nephrotoxic Metallic Compounds
   Metal
                              Environmental
Significant Hazard
                                                               Occupational
Boron
Cadmium
Chromium
Lead
Mercury
Platinum
Silver
Thallium
Uranium
Vanadium
Table 2.  Nephrotoxic Organic Compounds.
Aniline
Camphor
Carbon tetrachloride
Chlordane
Chloroform
Creosote
Ethylene dichloride
Ethylene glycol
Formaldehyde
Guaiacol
         Naphthalene (mothballs)
         Oxalates
         Parathion
         Pentachlorophenol
         Phenol
         Phosphorus (yellow)
         Resorcinol
         Thiourea
         Thymol
tions and  loss of the brush border, while later signs include  rounding of the
mitochondria. At the cellular level, inorganic mercurials appear to disrupt both tu-
bular secretion (organic anions) and reabsorption (sugars and amino acids), and cell-
volume regulation; they are potent  inhibitors of cytoplasmic, mitochondrial, and
plasma membrane enzymes by binding of Hg to essential sulfhydryl groups. The or-
ganomercurial fungicides are more lipid soluble than are the inorganic Hg salts and
hence their distribution in the body is dissimilar; differences in renal toxicity are
more quantitative than qualitative. Exposure to inorganic salts of mercury may arise
from numerous diverse sources, including drinking water (particularly from wells),
food, and industrial activities such as during manufacture of scientific instruments,
electric meters, mercury arc or vapor lamps, alloys, soldering compounds, mercury-
based antifouling paints, disinfectants, and pesticides and during bronzing, photoen-
graving, and mirror silvering.

Uranium Salts—
  Uranyl acetate is used as a model tubular poisoning agent in animal studies. The
lesion is focused in the proximal cells, as  in the case of mercury salts, and the more
soluble salts, such as  chloride and nitrate, are more nephrotoxic than are the less
soluble ones. Early signs of toxicity  include impaired tubular function, such as de-
creased transport of p-aminohippuric acid (PAH), amino acids and glucose as well
as decreased Na transport in the ascending limb of Henle's loop, which results in im-
pairment of urinary concentrating ability. Experimental uranium poisoning has pro-
vided interesting information concerning the early hemodynamic changes which are
sometimes  associated  with nephrotoxicity and precede the appearance of tubular
lesions. Thus, following low doses of uranium salts there are decreases in renal corti-
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cal blood flow, total renal blood flow, and glomerular filtration rate, with coincident
increases in plasma renin, an enzyme which liberates angiotensin which, in turn, ex-
erts changes on blood pressure. Because of the toxicity of uranium salts to other tar-
get organs, it is difficult to attribute their actions to any specific enzymatic site. Fur-
thermore, heavy metals often exert  their effects via  general  cytotoxic actions.
Nonspecific enzymatic inhibition can be expected, since uranium can form covalent
metal-salt complexes with a number of enzymes having functional groups such as
-SH,-PO3H2, -COOH;  uranyl salts inhibit  renal ATPase activity.

Cadmium—
  As is the case with most toxic metal compounds, the effects of Cd differ in target or-
gans depending on how it entered the body and whether exposure is acute or chronic.
For example, when foods are stored or cooked in cadmium-galvanized containers,
violent gastroenteritis can  occur shortly after eating. Renal toxicity is found more
frequently after low-level, long-term Cd exposure (Friberg et al., 1971). Cd can enter
the atmosphere through mining and manufacturing activities. Exposures from inha-
lation involve the lungs ("fume fever") primarily, but the kidneys are involved in an
important way, since they are both the major excretory organ and a target organ for
this metal. In animals and  man, the morphological changes are mainly confined to
the proximal tubules, whereas the glomeruli are less affected.  There are unusual
mechanisms involved in the nephrotoxic action of Cd. During initial exposures only
small amounts of Cd are excreted in the urine while accumulation occurs in the kid-
neys, but after a  certain renal level of Cd is reached, urinary Cd excretion increases
and then the amounts in the kidneys will decrease or stay about the same. Researchers
have found that a low molecular weight (10,000 daltons) protein, metallothionein,
binds Cd; this Cd-metallothionein is filtered at the glomerulus and reabsorbed in the
proximal tubule. With increasing exposure, more Cd will accumulate in the kidneys
than can be bound by the available metallothionein and so will exert toxic actions on
the tubules. Thus, urinary  Cd levels may at certain times give an erroneous view of
what is  happening in the kidney.

Lead—
  While there is some occupational exposure to the compounds of lead, most of the
current cases of chronic lead poisoning come primarily from children who chew paint
from their furniture and home. The other major source of lead is the 200,000 tons per
year emitted into the air from vehicles.  The kidneys are one of the three target organ
systems affected by lead; the central nervous and blood-forming systems are the oth-
er two. Signs of the renal lesion include increased urinary excretion of amino acids,
glucose, and  phosphates, indicating impaired proximal tubular function. Studies
with animals and with cells grown in culture have shown that lead salts are accumu-
lated by mitochondria and inhibit their respiration. At the electron microscopic level,
one also sees inclusion bodies in the cell nuclei of the proximal tubules,  and these
have become the current diagnostic hallmark for lead poisoning. While the increased
lipid solubility of tetraethyl lead causes some differences in body distribution com-
pared with inorganic compounds of lead, the  major target organs  are the same.

Platinum—
  Compounds of Pt are used in the exhaust systems of automobiles as the catalyst
designed to decrease emission of certain air pollutants. When disposed of, these anti-
pollution devices may introduce quantities of Pt into the terrestrial and aquatic en-
vironments, which  begets a "second-generation'1 contamination problem. Platinum
also is used in several new drugs where the need to develop water-soluble forms of
this heavy metal  for therapeutic uses has led to important answers as to the mecha-
nisms of toxicity of this metal (Guarino et  al.,  1980). In animals and man, Pt com-
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pounds caused nephrotoxicity, which was localized in the proximal tubules, and
available evidence favors  inhibition of  Na,K-ATPase as  a  major mechanism of
toxicity.

Organic Compounds—
  Carbon tetrachloride (CCU) is extensively used as an industrial solvent and is
widely distributed throughout air and water. Although liver damage is dramatic af-
ter ingestion of this solvent, acute renal failure is usually the cause of death. Morpho-
logically, tubular necrosis in the loops of Henle is most obvious. The extensive stud-
ies of how CCU exerts its toxic actions have shown that it forms highly reactive, free
radicals, which, in turn, cause disruption of membranes. In the case of mitochondria,
such effects result in swelling, loss of function, and other effects similar to those
caused  by hypoxia, that is,  disruption of cellular energetics.
  Ethylene glycol and diethylene glycol (antifreeze) exert their nephrotoxic actions
by being metabolized to oxalate crystals which accumulate and clog  the proximal
and distal convoluted tubules. However, there may be other mechanisms occurring
to impair tubular function in addition to the obstruction caused by  the crystals.
  Two major classes of nephrotoxic insecticides, chlorinated hydrocarbons (such as
chlordane) and organophosphates (such as parathion), cause acute tubular necrosis
with subsequent renal failure. Some of the major nephrotoxic organic compounds
are listed in Table 2.

ASSESSING RENAL FUNCTION
  Ideally, predictive or diagnostic tests should be simple to perform, sensitive, spe-
cific, easy to interpret and applicable to a wide range of animals, including man. In
actual practice, no single procedure  can fully meet all criteria and substantial trade-
offs must be accepted, such as specificity for simplicity. Indeed, many techniques can
be used only in animal studies; and, for example, microperfusion is currently limited
to a few selected species. Comparative studies of renal structure and function indi-
cate that differences exist even between closely related mammals. One would also ex-
pect to find species  differences  in the  distribution of and cellular sensitivities to
nephrotoxic chemicals. Thus, when evaluating data from animal experiments, one
must consider how predictive the model chosen will be for human toxicity.
  The nature of the measurement that is being made in the tests must also be consid-
ered.  Many tests of renal function are designed to measure the maximal capacity of
the organ to perform a specific function. The kidney does not normally function at
this high level, and impairment  would indicate a reduction in the reserve capacity
which might not affect day to day function. Such tests tend to be conservative, that
is, they exaggerate toxicities, but their use is important because reductions in func-
tional capacity may become physiologically significant during times  of stress.
  In addition to choosing  a preparation for study, the processes used to evaluate re-
nal function must also be carefully  selected. To measure filtration rates,  inulin or
polyethylene  glycol  clearances  suffice.  Quantitative changes in the glomerular
filtration apparatus (altered equivalent pore size) can be evaluated by the use of, for
example, dextrans of various molecular weights.  Organic substrates,  such as glucose,
amino acids, and organic anions and cations, are transported in the proximal tubule.
Use of nutrients and model excretory products as substrates for transport allows the
evaluation  of  proximal tubule reabsorptive and secretory function, respectively.
Since  such transport  is  dependent on cellular oxidative  metabolism and  on
transmembrane ion gradients, malfunction in both reabsorptive and secretory trans-
port may indicate a generalized metabolic disruption. On the other hand, if only one
process is affected, the specific locations of defects in major transport functions (rec-
ognized by the accumulation of nutrients on luminal membranes or excretory pro-
ducts on basal and lateral membranes) would suggest toxicant action at one surface,
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but not the other. Conveniently measured transport processes that are specific to
other nephron segments are difficult to identify. For ascending thick limb cells, di-
uretic-sensitive coupled Na and Cl transport could be measured; for collecting ducts,
ADH- and cyclic AMP-sensitive fluid transport might do.
In Vivo Methods
  Since the kidneys bring their major product, urine, to the outside of the body, sam-
pling procedures are simple and tests are relatively easy to conduct in vivo. In gen-
eral, in vivo methods include analyses of blood and urine samples to detect altera-
tions in solute  or  enzyme levels resulting from renal  dysfunction. Traditionally,
blood urea nitrogen (BUN) and urinary enzymes, protein, glucose, ketones and
amino acids have been used. Urea, the end product of protein catabolism, is elimi-
nated from the body primarily through the kidneys; since urea is a major component
of the BUN value,  it is the most frequently employed renal function test. In certain
species, however, BUN does not become elevated until at least 75 percent of the re-
nal function has been  lost. Serum creatinine levels provide essentially the same in-
formation as does BUN, but the former may be a more sensitive indicator of nephro-
toxicity. In addition, urine sediment is examined microscopically for red blood cells,
white blood cells, epithelial cells and casts. Such procedures have proved to be of
more value to physicians than to experimental investigators.
  Measurement of certain enzymes in the urine has recently been of some value in
diagnosing specific aspects of renal toxicities, since they show selective increases de-
pending on whether the glomeruli or tubules have been damaged. These enzymes
include lactate dehydrogenase(LDH), alkaline phosphatase, aspartate amino-trans-
ferase,  creatine phosphokinase, catalase,  /3-glucuronidase, JV-acetyl-/J-glucosa-
minase, renin, and muramidase. The more widespread use of techniques of measur-
ing enzymuria is prevented by the lack of sensitivity of existing methods, the presence
of inhibitors and bacterial enzymes in urine, the lack of organ specificity for many
enzymes and problems related to the manner of data expression. Moreover, isoenzy-
matic patterns  may be a more specific  index of renal tissue damage than are total
urinary enzyme levels. For example, the isoenzymatic  pattern for LDH may be of
more diagnostic value in differentiating between a renal and a hepatic lesion.
  In other procedures, test substances  are administered and the renal handling of
these compounds indicates the functional status of specific regions of the nephron.
Such procedures generally measure either the renal clearance of the test substance
using plasma concentration, urine concentration and urinary secretion rate, or the
maximum ability of the kidney to reabsorb or secrete the test substance. Clearance
experiments indicate the virtual volume of plasma that would be completely cleared
of the test substance in one minute; the specific renal function that is measured is de-
termined by the choice of test substance. For example, with inulin or polyethylene
glycol (PEG), substances that are filtered but not reabsorbed or secreted, the clear-
ance value is equal to the glomerular  filtration rate;  with p-aminohippuric acid
(PAH), which is filtered and strongly secreted, the clearance value is roughly equal to
the renal plasma flow, being an indicator of both blood flow and proximal tubule
function.
  These in vivo tests tend to be technically simple and they can provide nearly con-
tinuous monitoring of test animals over relatively long periods of time. Their major
problem is one of specificity, since altered blood or urine chemistry is not necessarily
an indicator of impaired renal function. Indeed, most nephrotoxic chemicals clearly
affect more than one organ system and  the observed effects on body fluids must be
considered to be a result of action at all target  organs.  Although these in vivo tests
may be of only limited value when used alone, their usefulness increases many-fold
when they are considered  in conjunction with more specific in vitro tests.
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In Vitro Techniques
  Each of the groups of techniques discussed below can be used for biochemical,
physiological and, in some cases, morphological studies of normal and impaired re-
nal function. In general, pathological changes in biochemical and physiological pa-
rameters tend to precede changes in morphology (seen at light or electron micro-
scope levels), so the  focus of this  section will be  on the functional rather than the
structural assessment of renal damage. Since so much of renal function is related di-
rectly  to membrane transport and cellular energy metabolism, probes of these
processes will be emphasized.

Isolated Perfused Kidney—
  With perfused kidney preparations, a renal artery of an anesthetized animal is can-
nulated, and physiological saline, usually with glucose, amino acids, and bovine al-
bumin, is pumped in at constant pressure (for review, seeMaack, 1980). Urine is col-
lected  and the clearance of substances added to the perfusate is determined. This is a
simple procedure which allows changes  in perfusion pressure (roughly comparable
to a change in filtration pressure) and in the composition of the perfusate to be made,
and the effects of these changes on overall renal function to be determined. Thus, sys-
temic influences on renal function can be eliminated experimentally. Ideally, direct
toxic effects can be assessed after chronic or acute in vivo dosing or by addition of
toxicant to  the perfusate. Unfortunately, when renal function data from perfused
kidneys are  compared to corresponding whole-animal data, one finds altered hemo-
dynamic characteristics, reduced  concentration and diluting ability and abnormal
distal tubule function. Since glomerular and proximal tubule functions are remark-
ably well preserved, the preparation appears to be most useful for the study of these
regions of the nephron.

Micropuncture—
   This methodology has been historically useful in localizing transport functions to
distinct regions of the nephron and, more recently, in studying specific mechanisms
of transport (see review by Gottschalk and Lassiter, 1973). One or more micropipets
are inserted into the  lumen of a surface nephron of an intact anesthetized animal and
either  tubular fluid is sampled  at a given location or the changes that occur in the
composition of the fluid as it passes from one sampling location to another are deter-
mined. Without involved surgical procedures, the sites available for micropuncture
are generally surface glomeruli (which give data on single nephron glomerular filtra-
tion  rate, GFR) and portions of the proximal and distal segments; only limited in-
formation on tubular function can be obtained. Except for the anesthetic, the con-
ditions are generally physiologically realistic with tissue geometry being preserved.
Micropuncture experiments require a great deal of technical skill on the part of the
investigator as well as much specialized equipment for taking and analyzing nanoliter
(10~9 1) volumes of fluid.

Isolated Tubular Segments—
  Over the last twenty years, procedures have become available for isolating tubular
fragments, perfusing them with solutions of known composition and measuring the
transfer of substances from the perfusate (luminal) to the bathing medium (peritu-
bular)  and vice versa (Burg and Orloff, 1973). Tubules can be dissected consistently
by hand from slices  of rabbit renal tissue, but care is required to avoid damaging
them.  When a suitable length of tubule  has been isolated, the ends are drawn into
pipets  by suction and the tubule is sealed in the pipets. Additional concentric pipets
are inserted  into the lumen of the tubule, through which perfusion fluid is introduced.
Data on reabsorptive and secretory transport of fluid, electrolytes, and organic com-
pounds have been obtained for perfused segments from nearly every distinct tubular
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region of the nephron, segments which would otherwise be inaccessible. The micro-
dissection procedures tend to be tedious and rigid control of perfusion pressures,
flow rates, and temperature are critical. At present, the low data output (a few tu-
bules perfused per day) and the relatively large variation in transport data from one
preparation to the next limit the use of the technique.

Cells in Culture—
  In the past few years, cell culture techniques have been utilized to a significant de-
gree for studies of renal cell function (see Handler et al., 1980). Epithelial cells from
renal tissue form monolayers with similar orientations when grown in artificial me-
dium on  a suitable support; their basal-lateral surfaces face the support and apical
(luminal) surfaces face the media. Many of these polar cultures transport water and
solutes and respond to hormones in much the same manner as the epithelial tissue
from which they are derived. Tissue culture offers potentially unique advantages,
since it could provide large amounts of material from individual cell types for long-
term in vitro studies. New mutant cell lines and renal cell cultures from a wide range
of species could be developed and maintained.  There are two major drawbacks,
however, limiting immediate use. First, epithelial cultures currently contain several
types of epithelial cells which represent  multiple segments of the nephron, as well as
non-epithelial cells. Second, the changes in cellular physiology that occur when renal
cells are grown in culture have not been documented thoroughly. The first problem
is largely one of technology and it will  probably be solved in time; the second is of
major concern, since it bears directly on the usefulness of cell culture techniques in re-
nal toxicology.

Tissue Slices—
  The preparation and experimental use of tissue slices is technically the simplest of
the in vitro procedures available for the study of renal function (for review,  see
Berndt, 1976). When thin slices from grossly defined regions of the kidney, such as
the cortex, are incubated in a well-defined physiological saline, cellular metabolism,
ion and volume regulation, and transport of specific substrates can be studied con-
veniently in the absence of factors affecting renal hemodynamics and GFR. Since
many slices can be obtained from a single animal, the effects of many in vitro treat-
ments can be determined and compared directly to paired controls. The limitations
of slice procedures are numerous. First, slices must be cut less than about  0.5 mm
thick in order to provide for adequate oxygenation of cells through their entire thick-
ness. Since the rate of penetration of substrate, nutrients, and toxicants is dependent
on slice thickness, slices must be uniform to give consistent data. Second, renal mor-
phology  is complex and slices cut at any level of the cortex or medulla will  contain
tissue from several tubular segments, blood vessels, and interstitial cells. Careful se-
lection of the process to be studied may allow evaluation of the function of one type
of tubular cell. For example, the secretion of organic anions, such as p-aminohippu-
ric acid (PAH), has been localized by micropuncture and microperfusion studies in
the proximal segment. Accumulation in proximal cells  is dependent on oxidative
metabolism; therefore, PAH uptake by cortical slices can be used to determine the
functional status of these cells. Third, since cross-sections of tubular tissue are usual-
ly obtained and tubular lumens appear to be closed, luminal or transepithelial trans-
port events cannot be studied. Indeed, most investigators consider the transport
properties of slice preparations to be primarily indicative of the characteristics of the
basal and lateral tubular cell membranes. Because of these limitations, the prepara-
tion is most appropriate for the study of secretory rather than reabsorptive transport,
where only the bath-to-cell step in the transport process is expressed.
  Some of the problems found with mammalian renal slices can be bypassed  by care-
ful choice of a simpler model from among the lower vertebrates. In certain species of
                                      103

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teleost fish, like flounder, the nephron contains few glomeruli, no loop of Henle and
little, if any, recognizable distal segment. Most of the tubular tissue appears to be
morphologically and functionally identical with the mammalian proximal segment.
The tubules are embedded in a matrix of friable hematopoetic tissue and thus can be
teased free easily; they show open lumens and seal off at the broken ends, forming a
cyst. Because of their long viability at the lower physiological temperatures of these
animals, teased tubular masses from teleost kidneys have provided a useful in vitro
preparation for the study of secretory transport in the proximal tubule (Pritchard
and  Miller, 1980).

Subcellular Fractions—
  Since the early 1960s, procedures have been available for separating nuclei, mito-
chondria, plasma membranes, microsomes and cytosol from homogenates of renal
tissue by differential centrifugation (Hopfer, 1977). In some cases, as with mitochon-
dria, both functional and morphological integrity is preserved and isolated mito-
chondrial preparations are clearly of value for studying inhibitors of oxidative me-
tabolism.
  New procedures in the field of membrane biology now permit the isolation of mem-
brane fragments from distinct regions of the plasma membranes of polar epithelial
cells, such as the brush border membrane or basal-lateral membrane of proximal
tubule cells. M ore importantly, it is now possible to study transport in vesicles which
form spontaneously from those membrane fragments; such  vesicles are essentially
free  of metabolic activity associated with cytoplasmic or mitochondria! enzymes.
These preparations have been instrumental in characterizing fundamental membrane
transport mechanisms, especially in cases where complex interrelationships among
several transport processes and cellular metabolism had previously confounded the
situation.  Vesicle  procedures permit the functional isolation and the study of
discrete transport events in specific membranes, and thus provide a powerful tool for
rapid testing  of pollutant effects.
  As with any technique, there are limitations which should be considered. Subcellu-
lar fractions should be well characterized with regard to  specific  marker enzymes
and, if appropriate, ultrastructure. Unstated in every tissue fractionation scheme is
the assumption that the methods used do not substantially change the biochemical
and physiological characteristics of the subcellular fractions obtained; this may not
be true of all cases. Because of damage caused by tissue homogenization or by chem-
icals used in the isolation procedure, mitochondria or membrane vesicles may lose
labile enzymes. In addition, assay media tend to be nonphysiological, and activating
factors, such as hormones or secondary messengers, are generally absent. Moreover,
drug treatment in vivo may affect the physical and biochemical characteristics of the
subcellular elements and thus  modify the fractions  collected, as  well as the  size,
shape, surface charge and "sidedness"(that is, inside out versus right side out), of the
membrane  vesicle populations. These relationships must  be further explored.

Tissue Enzymes—
  Measurement of enzyme activities or substrate metabolism in whole-tissue homo-
genates or subcellular fractions is a convenient way to obtain potentially important
data on renal cell function and on subcellular sites of toxicant action. Normal renal
metabolism clearly varies qualitatively and quantitatively with the segment of the
nephron under study. In most segments, overall metabolic rate, as measured by 02
consumption or  lactate production (depending on whether aerobic or anaerobic
metabolism predominates  there),  is directly related to major transport functions,
such as NaCl transport in the proximal tubule and thick  ascending limb, and can
provide a reasonable but relatively nonspecific index of transport capacity. In con-
trast, caution should be exercised in assuming that changes in activities of single en-
                                    104

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zymes, measured in vitro, reflect impaired renal function in vivo. Most in vitro en-
zyme  assays  measure activities under  maximum velocity (V  max)  conditions.
Enzyme activities in situ are dependent on actual intracellular concentrations of sub-
strates, cofactors, and regulators, which  may be different from those in the in vilro
assay  medium. In addition, some enzymes, such as carbonic anhydrase, are present
in cells in great excess and a large fraction of enzyme activity must usually be inhib-
ited before any physiological effect is observed. Other enzyme activities, notably
those  of ATPase, appear to be directly  correlated with specific transport events.
Na.K-ATPase appears to be a pivotal transport-related enzyme in the renal tubule,
driving  transepithelial  salt and water  transport  directly and  reabsorptive and
secretory transport indirectly. This enzyme also plays a major role in cell volume and
ion regulation, thus maintaining conditions that are optimal for overall cell function.
Na.K-ATPase activity provides a potentially important indicator of renal effects af-
ter in vivo exposures to toxicants. However, caution should be exercised in the use of
this or any other enzyme assay  as an in  vitro screen,  since exposure conditions in
homogenates (both surfaces of membrane fragments exposed to toxicant in artificial
medium) may result in inhibitory effects  that are not possible in intact cells. More-
over, the use of any enzyme assay for in vitro screening should be supported by data
showing that the enzyme used is, indeed, a primary target of toxicant action in vivo.

FUTURE DIRECTIONS FOR RESEARCH

In Vitro Screening Procedures
  In the past, little attention was focused on the development of widely applicable,
short-term tests for renal toxicity. We believe that certain of the procedures discussed
in the preceding section could provide a methodological framework for such test
protocols. Because no single group of related pathological processes underly the ef-
fects of nephrotoxic chemicals,  it is unlikely that any simple screening procedure
would by universally predictive.  Indeed, a battery of several simple procedures may
prove to be of greatest value.
  The regions of the nephron that can be studied using each of the previously dis-
cussed i/i vilro procedures are shown in Table 3. Because of technical considerations,
perfused kidney, micropuncture and perfused tubule procedures do not lend them-
selves well to screening protocols; slices are limited  in applicability to only the prox-
imal and, possibly, distal segments. The two remaining types of preparations, cells in
culture and isolated organelles, offer the greatest promise for success as specific, rap-
id screening procedures at the cellular and subcellular levels, respectively. However,
considering the present state of knowledge, significant methodological refinements
are needed before either group of procedures can be used for routine screening. For
both cases, parallel problems exist in obtaining relatively pure and functionally re-
plete preparations of cells or organelles (for example, membrane vesicles) from spe-
cific regions of the nephron.

Developmental Renal Toxicology
  Young organisms require special attention with regard to pollutant toxicity. First,
additional routes of toxicant exposure must be considered. Fetal exposure is a re-
flection of both maternal body burden and current exposure levels since the placenta
is not an absolute barrier to toxicants. After birth, nursing neonates may be exposed
to high levels of lipid-soluble toxicants that have accumulated in breast milk. After
weaning, growing animals may  encounter toxicants in food or water. In addition,
lead poisoning due to the ingestion of nonfood items (pica) is a significant problem
in children; other nephrotoxic substances could conceivably be ingested at this time.
Second, when compared to other organ systems, kidney function develops late, with
changes still occurring weeks after birth, so that renal lesions which occur as a result
of exposure early in life may not be expressed until weeks later. Third, our under-
                                     105

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Table 3.  Portions of the Nephron that can be Studied by the Use of Avail-
           able In  Vitro Procedures.
Procedure
Perfused kidney
Micropuncture
Perfused tubular
fragments
Tissue culture
Slice
Subcellular fraction/
membrane vesicle
Glomerulus
X
X


X



Proximal
tubule
X
X

X
X
X

X
Loop of
Henle



X
X


X
Distal
tubule

X

X
X


X
Collecting
duct



X
X


X
 standing of the changes that normally occur in renal function during development is
 largely incomplete. Additional studies are clearly needed to identify critical periods
 before toxic effects in young animals can be understood completely and suitable test
 procedures can be devised. This problem is further confounded by the fact that renal
 systems in different species develop at different rates, so no single animal model com-
 pletely correlates  with man.
                                REFERENCES

 Bellies, R.P.  Metals. In  Toxicologv, edited by L.J. Casarett and J. Doull, Macmillan, New
    York, pp. 454-502, 1975.
 Berndt, W.O. Use of the tissue slice technique for evaluation of renal transport processes.
    Environ. Health Perspect. 75:73-88, 1976
 Brenner, B.M., and F.C. Rector, Jr. The Kidney, Vol. 1, W.B. Saunders, Philadelphia, 1976.
 Burg, M.B.,and J.Orloff. Perfusionof isolated renal tubules. Handb. Physiol Sect. 8:145-159,
    1973.
 Dinesen, I. Seven Gothic Tales, Vintage Books, New York, 1972.
 Foulkes, E.G. Mechanism of renal excretion of environmental agents. Handb. Phvsiol. Sect.
    9:495-502, 1977.
 Foulkes, E.G., and P.B.  Hammond. Toxicology of the kidney. In Toxicology, edited by L.J.
    Casarett and J. Doull, Macmillan, New York,  pp. 190-200, 1975.
 Friberg, L., M. Piscator, and G. Norgber.  Cadmium in the Environment, CRC Press, Cleve-
    land, Ohio, 1971.
 Gottschalk, C.W., and W.E.  Lassiter. Micropuncture metholology. Handb. Phvsiol. Sect. 8'.
    129-143, 1973.
 Guarino, A.M., D.S. Miller,  S.T.  Arnold,  M.A. Urbanek, M.K. Wolpert, and M.P. Hacker.
    Approaches to studies of platinate toxicities. In Cisplatin-Current Status and New Devel-
    opments, edited by A.W.  Prestayko, S.K. Carter, and S.T. Crooke, Academic Press, New
    York, pp. 237-248, 1980.
 Guyton, A.C.  Textbook of Medical Physiology, W.B. Saunders, Philadelphia,  1976.
 Handler, J.S., F.M. Perkins, and J.P. Johnson. Studies of renal cell function using cell culture
    techniques. Am. J. Physiol. 238: F1-F9, 1980.
 Hopfer, U. Isolated membrane vesicles as tools for analysis of epithelial transport. Am. J.
    Physio/. 233: E445-E449,  1977.
 Maack, T. Physiological evaluation of the isolated  perfused  rat kidney. Am. J. Phvsiol. 238:
    F71-F78,  1980.
 Magos, L., and T.W. Clarkson. Renal injury and urinary excretion. Handb. Phvsiol. Seel. 9:
    503-512, 1977.
 Maher, J.F. Toxic nephropathy. In The Kidnev, edited by  B.M. Brenner and F C Rector,
    W.B. Saunders, Philadelphia, pp. 1355-1595, 1976.
                                        106

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Oppelt,  W.W. Toxicity from exposure to  solvents. In Laboratory  Diagnosis of Diseases
   Caused by Toxic Agents, edited by F.W. Sunderman and F.W.  Sunderman, Jr., W.H.
   Green, St. Louis, pp. 296-304, 1970.
Orloff, J., and R.W. Berliner (eds.). Renal Physiology, Handb. Physiol. Seel. S:1082 p., 1973.
Pritchard, J.B., and D.S.  Miller. Teleost kidney in evaluation of xenobiotic toxicity and
   elimination. Fed Proc. 39:3207-3212, 1980.
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   EARLY  WARNING  SYSTEMS  FOR BEHAVIORAL
                            TOXICOLOGY

                             Bernard Weiss

INTRODUCTION
  Traditional definitions of toxicity epitomized the tangible criteria of death and tis-
sue damage, but newer public concerns have enlarged their scope. Now toxicity in-
cludes the consequences of low-level, lifetime exposures and impairment of function
rather than outright poisoning and gross pathology. The role assigned to behavioral
measures is consonant with this new emphasis. Behavioral toxicology is the discipline
that emerged in response (Weiss and  Laties,  1975).

Why  Choose  Behavior?
  Behavior and the nervous system mediate our exchanges with the environment,
and their capacities determine how we adapt to its challenges. Behavioral measures,
moreover, do not invade  or destroy tissue so that they permit repeated testing, al-
lowing us to follow the progression of an intoxication and to evaluate cumulative or
reversible effects. Behavior, finally, may be the only visible consequence of toxicity.
Consider the parallel with schizophrenia. Despite the nearly universal belief that it
arises  from a chemical  substrate and despite a  massive research  effort, a cogent
chemical basis  for it has yet to be defined. It remains a behavioral disorder.

Agents  Associated with Behavioral Toxicity
  Behavior is the most multifaceted aspect of biology, spanning an immense array of
structures, systems, mechanisms, and processes. It should not surprise us that many
chemicals can be singled out as sources of behavioral disturbances. Heavy metals,
such as lead and mercury, have been recognized since antiquity as origins of neuro-
logical and behavioral toxicity. Lead remains troublesome because of its wide dis-
persal  in the environment and its threat to the developing nervous system. At high
exposure levels it can cause brain damage, while at lower levels, without provoking
any visible symptoms, it may be  responsible for depressed school performance and
for disturbed conduct. Mercury  is recognized as a powerful central nervous system
poison, and one of its organic forms, methylmercury, has been responsible for several
serious epidemics of poisoning.  It is capable of causing severe, irreversible brain
damage and of disrupting the pattern of normal brain development. Table 1 surveys
the array of neurobehavioral consequences ascribed to excessive exposure to various
metals. Volatile solvents and fuels can also engender severe behavioral toxicity.
Toluene, trichloroethylene,  dichloromethane and carbon disulfide can produce
behavioral actions ranging from  mild impairment of alertness to frank intoxication
like that  produced by alcohol.
The Author: Bernard Weiss is on the staff of Ihe Division of Toxicology and Environmental Health Sciences
Center. Department of Radiation Biology and Biophysics, School of Medicine and Dentistry University of
Rochester,  Rochester, New York.
                                     108

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Table 1.  Adverse Neurobehavioral Responses Ascribed to Metals (Weiss,
           1979).'
E
D
C
'E
D
<
Antimony
Arsenic
1
o
m
Cadmium
•o
CD
0>
_J
Manganes*
Mercury

-------
Examining Behavior in Different Settings
  The flexibility and variety of behavior yield both an immense luxury of choice and
a bewildering expanse of possibilities for tests.

The Natural Environment—
  Behavior need not be brought into the laboratory to observe and quantify it. We
can, for example, place trained observers in the classroom to record, by standardized
procedures, the behavior of individual children. Parents can complete rating scales,
which require estimates of the magnitude, frequency or applicability of specified be-
havioral characteristics, and workers can select, from a list of adjectives, those that
describe their current moods, feelings, or complaints.

Laboratory  Testing—
  Sensory processing, learning and memory, and motor coordination in humans are
best studied in a controlled environment. Certain issues demand the precision af-
forded by the laboratory, especially if the alternative may be an improperly high or
low exposure standard  with its consequent economic and health implications. Non-
human species, however, dominate in toxicology because we not only need  to
maximize control over exposure conditions and ancillary factors such as diet, but
also over behavioral history. Moreover, the issues arising out of delayed effects and
fetal exposure cannot await resolution by epidemiology that may require decades for
completion.

HISTORICAL PERSPECTIVE
  Behavioral toxicology  now describes an increasingly coherent discipline, but its
importance had been recognized earlier. Industrial hygienists have long viewed atyp-
ical behavior as an important  guide  to  adverse effects, and  took  account  of
behavioral aberrations in establishing exposure standards. In a more formal manner,
the USSR, guided by its scientific tradition, elevated behavior and central nervous
system function into pre-eminent roles in toxicity assessment. Exposure standards
derived from such criteria spurred the United States and other Western countries to
examine  behavior more carefully as a toxic end point.
  A century of experimental psychology and a surgent neuroscience provided the
basic tools for behavioral toxicology, while behavioral pharmacology offered the
most direct guidance. The advent of potent drugs, especially antipsychotic agents,
for treating behavioral  disorders  inaugurated this new discipline.  It emphasized be-
havior as the focus of drug action and aimed its resources at three problems: screen-
ing for new drugs, the behavioral mechanisms of drug action, and the relationships
between brain chemistry  and behavior. It was an easy sidestep to toxicology.

PRESENT  STATE  OF BEHAVIORAL TOXICOLOGY
  Behavioral toxicology began in response to questions about the health risks from
agents already recognized as hazards. What was needed for both resolution and doc-
umentation was the extent of the threat. For example,  how much methylmercury
was safe to ingest? What blood levels of lead in children can be considered nontoxic?
  With the passage of legislation  such as the Toxic Substances Control Act of 1976,
the impact of such issues widened to include new agents, but also  posed a dual set of
questions. One set stems from the  need to detect whether a new or previously unstud-
ied agent has potential  for behavioral and neurological impairment. The other de-
rives from the need to estimate the exposure levels of a known toxic agent that might
pose a health threat. The first requirement might be met by a collection of tests pre-
viously shown to be sensitive to neurotoxic agents. Those of the  second ordinarily
will have to be met by more refined and specific tests. These dual responsibilities are
                                     110

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reflected by two distinct definitions of what constitutes an "Early Warning System"
In one instance, it comprises identification. In the other, it signifies quantification.
The efforts of the past ten years reflect the dominance of the latter — the risk assess-
ment — in behavioral toxicology.

Evaluation of Sensory Function
  Information about our immediate environment reaches the brain through a com-
plex sequence of steps.  The process begins with the response of a receptor or sense
organ. The eye, for example, converts variations in light into nerve messages, and the
ear translates fluctuations in sound pressure. Defects in the receptor or in the nerve
pathways  that transmit and process this information impair our ability to  react to
our surroundings. Perhaps because they act as sentinels themselves, sensory systems
often  reflect the early impact of toxic processes.
  No  sensory system is immune. Deficits in the skin senses,  impaired hearing, and
tunnel vision, can all result from methylmercury poisoning, which damages pertinent
brain  areas.  Methanol (wood alcohol), often proposed as a fuel or fuel additive, in-
jures the optic nerve. Certain organic  solvents damage  peripheral nerves and mar
sensitivity to touch. Cadmium may injure the sense of smell. Such widespread sus-
ceptibility argues for the inclusion of sensory tests as measures of toxicity.
Reflex Assessment of Sensory Function—
  Simple reflex testing  is usually carried out during the  early phases of toxicity as-
sessment and is commonplace in the drug industry. Rodents may be examined for re-
sponsiveness to a sharp hand clap, or their paw pads may be stroked to gauge skin
sensitivity. Beyond identifying gross toxicity, such  testing is not a useful guide to
hazard identification or mechanisms of action. More precise, instrumented tech-
niques are preferred.
  A simple, instrumented test is the startle response. A sudden loud sound elicits
both in humans and in animals a stereotyped posture (like a sharp wince) and associ-
ated physiological activity.  By placing a rat on a force-sensitive platform, we can
measure the amplitude of its startle response. Hoffman and Ison at Bryn Mawr and
Rochester, respectively, found that the amplitude of the startle response is exquisitely
sensitive to accessory stimuli during or preceding the one evoking startle (Hoffman
and Ison,  1980). The startle amplitude can be enhanced or depressed by an amount
determined by the amplitude of these accessory stimuli. Presume an interval, say, of
100 milliseconds between the modifying and the startle stimuli. A loud or bright or
other  intense modulating stimulus reduces the startle amplitude markedly.  A
barely detectable modulating stimulus reduces amplitude by a small amount. During
a 30-minute test period, a wide range of modulating stimulus intensities can  be
presented  and a function subsequently plotted.  Triethyl  tin damages the fatty
(myelin) sheaths of nerves. To define its toxic end points further, Squibb, Carmichael,
and Tilson (1980) administered the compound to rats and measured the startle re-
sponse both to a loud sound and to an air puff to the rat's face. Responses  to both
stimuli were reduced by triethyl tin poisoning, but the rats were severely impaired in
many other  ways as well; for example, they sharply decreased their food intake.

Psychophysical Methods—
  Psychophysics, the oldest branch of psychology, studies the relationship between
specified physical dimensions of stimuli and the responses they evoke, typically rely-
ing on cooperative humans or highly trained animals. The subject indicates if a stim-
ulus has been detected, or assigns some index of its magnitude. Psychophysical meth-
ods usually are not viewed as elements of a preliminary test battery, but as alterna-
tives when questions arise about the earliest, subtlest consequences of toxic exposure.
  Psychophysical studies of vision can provide precise information for toxicity as-
sessment; I will discuss two such studies which have been concerned with the toxic ef-
                                     111

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 fects of methylmercury.  Both experiments  used monkeys because  their visual
 systems and visual capacities correspond to those of humans. The retina, located at
 the rear of the eye, is the screen upon which visual scenes are displayed. But the ret-
 ina, and its representation in the brain, is not homogeneous in structure or function.
 Its central area, the fovea, is comprised of closely packed elements termed cones.
 Functions such as the delicate discriminations of reading and distinguishing colors
 are served by the cones, which require at least moderate levels of illumination. The
 more peripheral areas of the retina are served by  the rods, which, because of their
 sensitivity and interconnections, can be thought of as light-gathering instruments.
 We depend on the rods for night vision. Damage to central vision can be produced
 by methanol,  whose metabolic products cause optic nerve lesions due to swelling
 (edema). Quinine damages mostly the peripheral retina. Methylmercury selectively
 damages neurons in areas of the cerebral cortex that subserve the peripheral visual
 fields.
   Methylmercury became an important concern in North America in 1970 with the
 discovery of contaminated fish in the Great Lakes and the high body burdens of oce-
 anic fish such as tuna and swordfish. Episodes of methylmercury poisoning in Japan
 — Minamata  in the 1950s and Niigata in the  1960s — cogently documented earlier
 reports that vision provided an early warning system. Evans, Laties and Weiss (1975)
 trained monkeys to discriminate geometric forms (circle, square, triangle) projected
 onto translucent response keys that the monkey pushed to indicate its choice. Cor-
 rect choices yielded a few drops of fruit drink from a nearby spout. After prolonged
 treatment with methylmercury, the monkeys began to show decreases in accuracy,
 especially when the stimuli were illuminated with very dim light. This deficit, con-
 gruent with the night blindness complained of by some human victims, was consis-
 tent with  the pattern  of damage to the  brain (Weiss,  1978).
   Another approach is to plot the visual fields, a technique that opthalmologists call
 perimetry. With the center of gaze fixed, stimuli are presented in various parts of the
 field, and the  patient indicates whether or not these have been seen. The resulting
 chart shows those portions of the field unresponsive to stimuli. This test was recently
 adapted to monkeys (Merigan and Weiss, 1980). It depends on their insatiable ap-
 petite for marshmallows. The monkey sits in a special chair, facing a screen contain-
 ing a central aperture of variable diameter (Fig. 1). With the monkey's gaze fixed at
 the center of the aperture, a white marshmallow on another rod is moved to the inner
 border of the test aperture while the monkey's gaze is monitored. The monkey's alert-
 ness is maintained by allowing it to eat the  stimuli. Central apertures of different di-
 ameters allow  the peripheral fields to be mapped with surprising consistency. A sin-
 gle monkey can be mapped in about 30  minutes by two observers. The bottom
 portion of  Fig.  1  shows the aftermath of  methylmercury  poisioning—severe
 constriction of the range of vision. Small depressions in the boundary, however, ap-
 pear early in the course of intoxication and at that point are reversible.

 Elect rophysiology—
  The activities of nerve cells can be measured  as electrical signals. The nervous sys-
tem, in fact, is in continuous electrical flux, a feature  that has made it possible for
clinicians to rely  upon "brain waves" for diagnosis. Epilepsy, for instance, is charac-
terized by  massive coordinated discharges. Electrical activity is detectable by placing
electrodes (such as small metal cups) on the skull, amplifying the signals, and record-
ing the output to give an electroencephalogram (EEG). In animals, stiff wire elec-
trodes, buried  directly in brain tissue, furnish clearer electrical signals than surface
electrodes and  allow the local activity of different brain areas to be compared. Most
investigators have access to devices that convert the EEG to a representation of fre-
quency against amplitude (a power spectrum).
  The incessant electricial activity of the brain, however, except in unique situations
such as epilepsy, does  not afford a distinct index of abnormal conditions, since too
                                     112

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                                                                           Left eye
Right eye
                                                                                   No. 605  Control
                                                                           Left eye               Right eye
                                                                                   No. 82 MeHg
Figure 1.  Procedure for plotting visual fields in monkeys. Top: The visual subtense of this aperture can be changed in 10 degree steps
          from 10 to 70 degrees. The experimenter places a marshmallow or small piece of fruit on a thin rod at the fixation point, and
          observes the monkey through his hole. While the monkey gazes at the central target, a 1 -cm white marshmallow is moved
          inside the aperture at one of the light test points located at 45 degree intervals. If the monkey glances toward the second
          marshmallow, a detection is marked at the appropriate location on the visual field chart. Only one eye is tested at a time. The
          other eye is occluded with an opaque contact lens. Bottom: The visual field boundaries of a normal monkey, No. 605, extend
          both vertically and horizontally much farther than the boundaries of a methyl-mercury (MeHg)-poisoned monkey. No. 82. The
          latter monkey, long since recovered from the peak of the intoxication, shows tunnel vision. (The second boundary drawn on its
          left eye plot shows the results of a second determination.) This kind of visual loss also appears in human victims. Incipient
          visual field contraction seems to be the earliest adverse sign in monkeys  (Merigan and Weiss, 1980).

-------
many things are happening at once. Yet, there are underlying regularities, some of
which are detectable as rhythmic fluctuations in amplitude. An example is the indi-
cation that organophosphate pesticides may change the shape of the power spectrum
in monkeys and humans (Duffy and Burchfiel, 1980). Although extensively applied
to central nervous system drug research, such representations have  not wielded
much of an impact in toxicology.
  One way to reduce the electrical chaos is to sharpen the focus of action by trig-
gering a particular population of nerve cells into synchronous discharge, and sepa-
rating their unique pulse from its incoherent background. The outcome is the evoked
potential. Its use was clearly demonstrated, again, when methylmercury poisoning
swept through Niigata Prefecture, Japan, in 1963-65. Ophthalmologists were among
the first clinicians to be alerted because of the previous experience at Minamata 10
years earlier. Among the many diagnostic methods they applied was the visually
evoked cortical response. One of their stimulus conditions consisted of a flickering
light presented at various frequencies. A normal control subject (Fig. 2) evinced
well-defined fluctuations in the amplitude of the cortical electrical response at fre-
quencies as high as 42 Hertz, but the patient record showed disorderly fluctuations
even at 28 Hertz. These data corresponded to reports by patients of the frequency at
which  the flickering light appeared  steady, indicating that electrophysiological
measures may be candidates for toxicity assessment in similar visual disturbances.

Assessing Motor Dysfunction
  Almost every class  of chemical agents induces characteristic deficits in coordi-
nated movement.  Mercury vapor's unique toxic action is to cause tremor. Children
poisoned by methylmercury show disorders of coordination resembling cerebral pal-
sy. Volatile solvents impair the ability to execute delicate movements such as those
required to assemble small mechanical parts, and manganese dust and fumes induce
a syndrome  resembling Parkinson's disease.

Motor Reflexes and Simple Instrumented Tasks—
  Preliminary pharmacologic and toxicologic screens typically assay several simple
reflexes. The righting reflex, for example, is the tendency of animals to return to a
normal position after being turned on their backs. Placing reactions refer to the way
an animal extends its paws when it is brought forward to approach the edge of a sur-
face. The best-known instrumental technique for measuring reflexes is the rotarod
(Fig. 3). The animal is placed on a grooved, rotating rod to which it clings in order to
avoid a fall. At high rates of revolution, clinging for an extended time is impossible.
The number of revolutions usually is  counted automatically, with the subject's fall
tripping a switch that stops the counter. Kaplan and Murphy (1972) used the rotarod
to study the neurotoxic effects of acrylamide, a highly reactive vinyl monomer, used
since the 1950s to produce various polymers, to strengthen paper products, to sepa-
rate solids in water, and as a grouting agent. Acrylamide damages both sensory and
motor nerves in a pattern characterized as "dying-back" neuropathy. The nerves first
begin to  degenerate at their distal ends (farthest from the cell body), a process that
spreads gradually inwards. Rats treated by moderate doses of acrylamide began to
show deficits in performance after about four days of treatment. Visible loss of coor-
dination did not appear until about day seven. Edwards and Parker (1977) soughta
simpler method. When a mouse or rat is dropped from a height, it extends its paws in
a characteristic posture which can be measured by inking the paws  beforehand.
Splaying of the hind legs was exaggerated by acrylamide and proved about as sensi-
tive as the rotarod. Later, investigators at the National Institute of Environmental
Health Sciences in North Carolina developed a technique to measure forelimb and
hmdhmb strength;  the  hindlimb  response  proved  more sensitive to acrylamide
(Tilson, Mitchell and Cabe, 1979). In all three studies, the treated animals lost weight
compared to controls,  a confounding problem.
                                    114

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    Patient
    Flicker
      22Hz
       VER
      28Hz
       VER
'VWVWWWXAAA
    Normal
       VER
 Figure 2. The response of a patient with Minamata Disease and a normal control. The
         patient VER (visual evoked response) showed inability to follow the light
         fluctuations even at relatively low frequencies, a marked contrast with the
         control subject.
Advanced Methods—
  We should not expect the comparatively simple acts assayed by preliminary toxic
screens to predict the subtle deficits incurred by human victims after moderate ex-
posures.  For example, excessive tremor accompanies  many types of neurological
disorders, including those induced by toxic agents such as mercury vapor. It was one
of the earliest signs of toxicity in workers exposed to the organochlorine pesticide,
Kepone, in a Virginia plant. Wood, Weiss and Weiss (1973) followed the progress of
workers who has been poisoned by mercury vapor (Fig. 4). The  patient inserted a
finger in a lucite trough attached to a force sensor whose output was amplified and
processed by a computer. Tracings showed marked tremor at the time the patients
first came to the hospital, and a gradual decline over a period of months without fur-
ther exposure. Quantitative measures of tremor revealed more subtle changes, espe-
cially the presence of multiple peaks in the frequency spectrum. These cannot be ob-
                                  115

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 Figure 3.  Mice being tested on a rotarod. They are placed on the grooved rod, which
           then begins to turn. Control circuitry gradually increases the rotation speed.
           When a mouse falls, it strikes a paddle that turns off the counter beneath it.
           The counter is incremented once with each revolution.
served clinically, which is why industry has now turned to such a system to monitor
exposed workers in the chlor-alkali industry, where mercury electrodes are used to
convert brine into chlorine and caustic soda.

Elect rophysiological Methods—
  Nerve conduction velocity is the most widely studied electrophysiological index of
toxicity, and is measured by electrically stimulating first one site on the nerve and re-
cording the electrical response at the muscle, and then stimulating another site and
recording the response again. To measure the conduction velocity of the ulnar nerve,
for example, stimulating electrodes are placed at the wrist and elbow on the inner
forearm, and recording electrodes are placed on the muscle near the base of the little
finger. A short duration stimulus  pulse (about 100 milliseconds) propagates an im-
pulse along the nerve and causes muscle contraction. The time elapsing between the
stimulus pulse and the onset  of contraction represents the latency of the response.
The difference in latency between elbow and wrist electrode positions is  used to cal-
culate the  velocity of the impulse along the nerve. If the latency from the elbow posi-
tion is  six milliseconds, that from the wrist electrode is three milliseconds, and the
distance between electrodes is 21 cm, the conduction velocity is calculated as 70 me-
ters per second.
  Lead-exposed humans and animals suffer depressed motor nerve conduction ve-
locities. Solvents such as methylbutyl ketone also reduce conduction velocity. The
test does not seem to bean especially sensitive measure, however. For individual di-
agnosis, the range of normal values is so wide that only the presence of ancillary signs
and symptoms is definitive. Temperature must be carefully controlled because low-
                                     116

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                                                               4-9-70
100-

 70:

 40
     10
                                                              1-14-71
                                      -1 5 sec.
Figure 4.  A system designed to measure fine motor control. The patient inserts a finger
          into the slot and tries to maintain a steady force between the specified limits
          (such as 10 and 40 grams) Excessively high or  low forces are signalled by
          lights on the nearby display box. The tracings show the improvement in
          amplitude of tremor and accuracy of motor control between the time when
          mercury vapor poisoning was first diagnosed in this patient and nine months
          without further exposure.
ered limb temperatures decrease velocity. The young and the elderly show reduced
velocities.  Empirically, such measures have  not proven fruitful in animal studies
because other indices seem able to detect toxicity at earlier times or lower exposure
levels. Seppalainen (1974) in Helsinki surveyed a group of workers in storage bat-
tery plants who had been exposed to lead, but who revealed no clinical signs of in-
                                     117

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toxication.  They  displayed  about  six  percent slower  maximal motor con-
duction velocity (MCV) of the median and ulnar nerves than an unexposed control
group. Since the MCV only reveals damage to large diameter nerve fibers, the inves-
tigators also tested the conduction velocity of narrow diameter, slower motor fibers
(CVSF) of the ulnar nerve. They found significant slowing due to lead (Fig. 5).
Complex Behaviors
  Since the natural environment is complex, the patterns of behavior by which or-
ganisms  respond to  and  manipulate the  environment must  be  correspondingly
complex. Consider what is involved when humans exposed to a  potentially toxic
agent complain of memory difficulties, apathy, inability to concentrate, and similar,
often vague symptoms.

Naturalistic Behaviors—
  Even rather complex, extended behavioral processes may be controlled largely by
hereditary mechanisms. Despite their often stereotyped expression, they represent
the outcome of many component systems and processes, giving them the potential to
reveal adverse consequences that might be overlooked in a survey  of simpler mech-
anisms.

  Reproductive processes—Mating and maternal care are central to species surviv-
al, a role consistent with the elaborate mechanisms that have evolved for their sup-
port. Flawed mating behavior may reflect disorders of endocrine function, of neuro-
chemistry, of central nervous system structure, of sensory function, or of other, per-
haps less direct influences. Madlafousek, Hlinak, and Parizek (1971) asked whether
cadmium-induced testicular atrophy and  necrosis were correlated with sexual
behavior in male rats. They placed male and sexually active female rats into a spe-
cial  enclosure  and  recorded  quantitative  measures of the  males' copulatory
performance (Fig. 6). After about nine days of treatment, most of the males began to
show incomplete mounts  and  failure to copulate. To determine  whether this be-
havioral  deficit arose from the destruction of testicular endocrine tissue, one group
was treated  with the male hormone, testosterone. Seventy days after hormone
treatment, most of the  rats had recovered copulatory function except for a pro-
longed postejaculatory interval. Testicular tissue also had  regenerated, confirming
the correlation between morphology, endocrinology and behavior.

  Activity—Many experimenters have tried to use activity as a quick and inexpen-
sive index of total function. Rats and mice adapt to many different kinds of enclo-
sures ranging from simple boxes to circular alleys. A simple technique is to mount
photocell beams in an enclosure and count the number of interruptions during a
specified time. The residential maze, a more recent type,  is designed like a figure
eight, and contains food and water in specific locations, and photobeams positioned
at several points. Since,  however, such techniques detect mostly coordinated, gross
movement, newer methods have been introduced  that provide measures of smaller
amplitudes. Some rely on disturbances of an electromagnetic field, and are especially
appropriate for measuring the activity of neonatal rodents not yet capable of walk-
ing.
  Animals typically display a daily rhythm to their activity. Robins are most active
in the morning and at dusk, and become even more active when they consume PCBs
(Ulfstrand, Sodergren and Rabol, 1971). Rodents are more active within the hours
of darkness. Running wheels attached to living cages are one traditional means of
monitoring daily activity.  Most rodents  will run several thousand revolutions per
night, and females run more during oestrus than at other times during their repro-
ductive cycle.
                                     118

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Figure 5. These charts plot conduction velocities, for two worker groups, of the slowe
motor fibers (CVSF) of the ulnar nerve. The group on the left, labelled Lead
Poisoning, consisted of 32 workers who displayed clinical symptoms or
markedly elevated blood  lead concentrations. Each worker's reading is
represented by a black dot. Age-matched controls, with no lead exposures,
showed higher and more normal values. The horizontal lines represent the
means. Note the clusters of three lead-poisoned workers at the bottom. The
charts to the right compare 28 lead-exposed workers who were clinically
normal with another group of age-matched controls. Even here, the difference
between the groups is greater than would be expected by chance.
(Seppalainen, 1974).
                            119

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 Figure 6.   Scheme for testing copulatory performance in male rats. The male is
            introduced at the first arrow and the female at the second. Intervals are (1)
            intromission latency (the time from the entrance of the female into the
            testing boxtothefirst intromission); (2)ejaculation latency(thetimefromthe
            first intromission to the appearance of the ejaculatory behavior pattern);
            (3) mean interval between intromissions (that is, between two consecutive
            complete mounts); and (4) post-ejaculatory interval (the time from the
            ejaculatory behavior pattern to the next complete mount). The investigators
            also measured :  (5) intromission frequency (the  number of intromissions
            preceding an ejaculatory behavior pattern); (6) incomplete mount frequency
            (the number of incomplete mounts between the first intromission and an
            ejaculatory behavior pattern); and (7) ratio of incomplete mount frequency to
            intromission frequency (Madlafousek et a/., 1971).
   Reasoning that air pollutants might hamper physical activity, Konigsberg and
 Bachman (1970) measured spontaneous running in rats exposed to ozone. Ozone is
 the principal oxidant in photochemical smog, producing systemic as well as local ef-
 fects on the  lung. Even at ozone concentrations that appear frequently in urban
 centers such as Los Angeles and Denver, activity fell off. This reduced inclination to
 run has its human counterpart in runners poisoned by increased levels of ozone in the
 course of exercise.
   Different techniques for assessing activity yield different conclusions, probably
 because they feature different acts. In addition, minute variations in enclosure design
 and  the environment can produce enormous variations in results. No one method is
 demonstrably superior (Reiter and MacPhail,  1979).

 Learned Behaviors—
   Many complex functions depend on individual histories and experience. They are
 learned. The  dominant role of learning and learned behaviors  in humans led experi-
 mental psychology to feature learning as one of its stoutest pillars. Impaired learning
 ability in humans has such devastating consequences that learning is an almost uni-
 versal component of proposed test batteries.
   A  situation used extensively in behavioral pharmacology is  avoidance condition-
 ing. Most typically, a stimulus such as a light or a tone serves as a conditioned (warn-
 ing) stimulus, providing several seconds delay before a painful electric shock circuit
 is closed. A specified response by the subject allows it to avoid the shock. Bignami
 (1975) relied  on avoidance performance as an index of toxicity for a class of com-
 pounds  called anticholinesterase  agents (for example, organophosphate pesticides
and nerve gases), which block an enzyme crucial to the function of the neurotrans-
 mitter, acetylcholine. He trained rats  to cross from one section to another of the ex-
perimental chamber at the appearance of a light or the sound  of a tone to avoid an
                                     120

-------
electric shock delivered several seconds later. His most important finding was the
confirmation of the rapid tolerance that develops to such agents. The first few in-
jections of the agents disrupted avoidance reactions markedly. Later, the compounds
exerted far less effect even though brain levels of the enzyme fell to about 10 percent
of normal. The implications of such a discrepancy pose a significant problem, be-
cause the long-term consequences  of such chronic reductions on human health are
unknown.
   More elegant analyses can be built on what are called Schedules of Reinforce-
ment. This term designates a scheme for manipulating operant behavior, or behavior
controlled by its consequences. In nature, the actions of organisms affect their en-
vironment, and determine its reactions. A laboratory model is exemplified by a rat in
an experimental chamber who is kept alert to the possibilities for food by mainte-
nance on a diet that holds its body  weight at about 80 percent of the free-feeding val-
ue. It quickly learns to press a small lever which discharges a pellet of food. Food is
the reinforcer and the events of food delivery comprise reinforcement. Food need not
be delivered after each response, but instead can be provided intermittently. The way
in which food delivery is governed is  called the Schedule of Reinforcement. For
example, a Fixed-Interval Schedule provides reinforcement for the first response
after a specified lapse of time, usually  measured from the previous reinforcement.
Responses occurring before  the interval ends are usually recorded but are not re-
quired for reinforcement.
   Willes, Rice and Truelove (1980) found Fixed-Interval performance to be a sen-
sitive index of lead exposure. They fed infant monkeys, from birth to 200 days, with
a milk formula to  which they added lead in various concentrations. One group of
four animals, treated each weekday with 500 micrograms for each kilogram of body
weight, attained blood lead concentrations in the upper range of urban American
children. After the treatment period, the monkeys were trained to press a lever for
juice delivery on an eight-minute Fixed-Interval Schedule of Reinforcement. During
the first 20 to 30 sessions, treated monkeys pressed much more frequently than con-
trols. They also tended to respond in bursts, in contrast to the controls, who showed
the typical pause after reinforcement followed by an increasing rate of response as
the opportunity for juice delivery drew nearer. Later, the average response rates for
the lead group declined to that of the controls, but the bursting pattern persisted.
These findings, coupled  with earlier  rat data,  suggest that  the  period before
performance stabilizes may be a sensitive index of aberrant function, with the con-
comitant advantage of not requiring extended training.


Homeostatic Functions
   Disturbances in  homeostasis frequently appear during the incipient stages of a
toxic process. Weight loss may be detected even before subtle neurological signs ap-
pear, as in acrylamide and methylmercury poisoning in rodents. Loss of appetite is a
common complaint among victims of toxic exposure. Metabolic disturbances might
be reflected even more quickly than disruption of body temperature regulation. A
web of simple reflex adjustments maintains central body temperature within rather
narrow limits by continuous changes in blood flow to the skin, metabolic rate and
sweating. These are microadjustments,  however. The major contribution to temper-
ature regulation is  behavior, for instance, wearing appropriate clothing during the
winter. Hormonal  disturbances and nutritional deficiencies that impair metabolic
heat production can be detected by measures of behavioral thermoregulation. Rats
deprived  of essential vitamins or lacking full thyroid function work for external heat
to replace the heat they cannot produce metabolically (Weiss and  Laties, 1961).
   Although microwaves heat tissue, some investigators have claimed that there are
behavioral effects at intensities too low to elevate deep body temperature. Stern and
his coworkers (1979) trained shaved rats to press a lever for a burst of heat from a
                                    121

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heat lamp, a task rats readily perform at low temperatures (Fig. 7). They then com-
pared alternating 15-minute periods with and without microwave exposure. During
exposures as low as five milliwatts per square centimeter of surface (half the current
human standard) the rats reduced their frequency of responses. This experiment is
also a useful example of how it is possible to measure presumably subjective states
(feeling cold) by behavioral methods.

Measures  of Aversion and Attraction
  The bilious miasma that clings to certain cities and the choking fumes emitted by
some industrial processes are not simply unaesthetic. Smarting eyes and raw throats
imply toxic actions. The aversive potencies of such irritants need to be established in-
dependently  of overt tissue damage.  Especially with new  chemicals, we cannot
always adopt the easy solution of asking human subjects to evaluate or estimate irri-
tant potency. We turn to animals, instead.
  Estimates of respiratory irritation obtained from various parameters of lung func-
tion are limited by the number of feasible measures and by reactions outside the di-
rect scope of the lung. Tests relying on the response of the whole animal have been
adopted for this reason. Mice or rats are put into a sealed cylinder, and variations in
breathing are recorded as pressure variations. Irritant gases, fumes, or vapors intro-
duced into the cylinder shift the pattern of respiration. Surveying a range of concen-
trations allows the experimenter to calculate relative indices of irritant potency. This
technique, like other reflex measures, has the virtue of not requiring extended train-
ing, although it could sometimes  be misleading if animals respond to low concentra-
tions because they have been conditioned to the smell of the agent. It also is not in-
trinsically a measure of aversiveness,  which is gauged by escape or avoidance re-
sponse (Wood, 1979). Such a measure is obtained in the same way that we estimate
the aversiveness of electric shock. In this case, a noxious gas or aerosol is injected
into a chamber and the subject makes a defined response so that the gas is replaced
by a stream of clean air (Fig. 8 A). The technique has been used  to measure the aver-
sive properties of ammonia. At low concentrations, the mice waited 20 to 30 seconds
before turning off the flow, but, at high concentrations, they waited only about five
seconds (Fig. 8B).

Behavioral Teratology
  There is  a  growing awareness  of the special, enhanced vulnerability of the fetus
and neonate, particulary its nervous system. What is especially troublesome is that
toxic substances may cause derangement of central nervous system development
which will be manifested, not as visible deformities (teratologies), but as behavioral
disorders later in life. Environmental contaminants are of particular concern be-
cause they may be stored in the female's body long before conception  and because
explicit exposure sources may not be known. Most studies in behavioral teratology
attempt some assessments in peri nates and neonates, seeking not just evidence of ab-
normalities, but of delayed development. The progression in Table 2 shows the mat-
urational stages in mice often used as markers of development.
  However, the earliest periods of development may not reflect the impact of a toxic
process. Damage in human infants may go unnoticed because of the neonate's undif-
ferentiated  behavior. Only later,  when the child fails to progress normally, does the
damage become apparent. The same problems arise in laboratory animals. In fact,
certain kinds of damage may not  manifest themselves until well into maturity, when
the reserve capacity and compensatory mechanisms of the brain, reduced because of
the original deficit, are no longer adequate to  cope with the  progressive erosion of
function that accompanies aging.
  One of the most significant studies in the past decade was carried out by Crarimer
(1975) after suspicions about the elevated sensitivity of the fetus to methylmercury
had been stirred by events at Minamata. Cranmer treated pregnant mice withmeth-
                                     122

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                        Blower
Figure 7.  System for evaluating the effect of microwave exposure on thermo-
          regulatory behavior (adapted from Stern et a/., 1979). The rat was shaved
          before the session, then placed in the chamber, which  was constructed
          largely of materials transparent to microwaves. The ambient temperature
          was about 4-5° C. Each press of the lever by the rat turned on the heat lamp
          for two seconds. Fifteen-minute periods without microwaves alternated with
          15-minute periods during which the rat was exposed to microwave radiation
          from a feeder horn. During the microwave exposures, which ranged from a
          power density of 5 to 20 milliwatts per square centimeter, the rat turned on
          the heat lamp less often.
ylmercury at various times before they gave birth. Some of the offspring died before
weaning; others showed reduced weight gain, and even those prenatally exposed off-
spring who were grossly indistinguishable from controls showed behavioral devia-
tions. At 30 days of age, they were tested in an open-field device where they not only
proved less venturesome than controls but also tended to take backward steps, an
unusual occurrence. They also swam in peculiar ways, showing frequent episodes of
incoordination and motionless floating.  Even more significant, as the treated ani-
mals aged, they began to show generalized  debilitation, evidence of immune defi-
ciencies, and neuromuscular lesions. Disorders that earlier had required tests to be
disclosed erupted into clinical disease in old age. Later research showed that even
much lower dose levels can produce later behavioral abnormalities.
                                     123

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(A)
                                                                                        (B)
                                                                    Irritant
                                                                                             60
                                                                                             50
                                                                                             40
                                                                                         1   30
                                                                                         u_

                                                                                         'o
                                                                                         c
                                                                                         o

                                                                                             20
                                                                                             10-
                                                                                              0-,
                                                                                                         i
  Figure 8.
                                                                                   0.0     .05     .10     .15


                                                                                         NH3 Concentration (%)




Irritant potency of airborne substances is measured behaviorallv with a response that terminates irritant flow (A) Room air

flowing through the exposure chamber evokes no response by the mouse (left). When room air is replaced by an aversive

concentration of ammonia (right), the mouse inserts its snout into one of the attached cones, an action that interrupts the

photobeam. Selection of the correct cone is programmed to pour clean air into the chamber and through the cone The other cone

is used to evaluate response specificity; responses are counted, but do not stop the flow of ammonia  (B) The higher the

concentration of ammonia, the more quickly the mouse responds to replace it with air (Wood, 1979).
                                                                                                                               .20

-------
Table 2.  Developmental Progress in Mice (adapted from Fox. 1965).*
      Period
                        Duration, Days
                     Main reflexological criteria
1.  Perinatal

2.  Neonatal
3.   Postnatal
    Transition
4.  Postnatal
    infantile
    (orPre-juvenile)

5.  Juvenile
Birth - 3

3 -9


9- 15
                       15-26
26 - onward
until sexual
maturity
Weak rooting, righting, geotaxis
and limb placing.
Strong and stereotyped reflexes and
limb placing reactions, pivoting and
circling locomotion.
Primitive reflexes disappearing (for
example, rooting). Adult locomotor
activities appear and organs of
special sense are functional at the
end of this period. Hyperkinesis
disappears. Overgeneralized sensory
responses seen.
Refinement of locomotor abilities
and sensory capacities. Over-
generalized responses to sensory
stimuli disappear.
Further refinement of activities
and sensory responses as the over-
generalized responsiveness is no
longer seen and manipulative
abilities are adult-like.
'Characteristic behavior patterns appear (and fade) within relatively circumscribed peri-
 ods, which are labelled on the basis of time since birth. Workers in behavioral teratology
 typically select a restricted subsample of these criteria for assessment.
   Such studies supplement the growing epidemiologic evidence of the developing
 nervous  system's enhanced sensitivity and emphasize the critical role that longi-
 tudinal studies, extending into old age, must play in behavioral teratologic research.
 Data from the 1971-72 epidemic of methyl mercury poisoning in Iraq now suggest
 that the fetus is at least 10 times more sensitive than the adult to this agent and that
 increasingly subtle deficits will appear as the children mature (Marsh et al., 1980).
   These studies have a counterpart in the debate about what constitutes a safe ex-
 posure to lead. In children, lead concentrations in blood are the traditional indices,
 but blood lead is a labile measure, reflecting only recent exposure, and cannot reveal
 effects on school performance of excessive exposure during earlier periods of life.
 Needleman (1980) therefore measured lead in deciduous teeth collected from over
 3000 asymptomatic  children in the first and second grades. Teeth, like bone, store
 lead so that the amount in the dentine of baby teeth serves as an integrated measure
 of early exposure. He also collected performance rating scale  measures from the
 teachers. In addition, he examined 58 children with the highest and 100 children with
 the lowest tooth lead concentrations by means of various psychological tests. Even
 in clinically asymptomatic children, differences in lead levels correlated with differ-
 ences in classroom behavior (Table 3) and IQ scores (Table 4). Perhaps the most in-
 triguing finding was the clear relationship between tooth lead and scores of class-
 room behavior based on a teachers' rating scale, a finding that makes the assumption
 of a threshold questionable.
                                      125

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TableS.  Comparison of Children with High and Low Tooth Lead Concen-
           trations on a Teacher Rating Scale (adapted from Needleman,
           1980).*
                              Low Lead         High Lead
                                                                PValuef
Distractible
Not persistent
Dependent
Disorganized
Hyperactive
Impulsive
Easily frustrated
Daydreamer
Does not follow:
Simple directions
Sequence of directions
Low overall functioning
14
9
10
10
6
9
11
15

4
12
8
36
21
23
20
16
25
25
34

14
34
26
0.003
0.05
0.05
0.14
0.08
0.01
0.04
0.01

0.05
0.003
0.003
*The percentage of each group receiving a negative evaluation is tallied here.
fine probability that these differences could have occurred by chance.
Table 4.   Intelligence Test Scores of Children with High and Low Tooth
           Lead Concentrations (adapted from Needleman, 1980).*

Full-scale IQ
(normalized)
Verbal IQ
Information
Vocabulary
Digit span
Arithmetic
Comprehension
Similarities
Performance IQ
Picture completion
Picture arrangement
Block design
Object assembly
Coding
Mazes
Low Lead
(Mean)
106.6

103.9
10.5
11.0
10.6
10.4
11.0
10.8
108.7
12.2
11.3
11.0
10.9
11.0
10.6
High Lead
(Mean)
102.1

99.3
9.4
10.0
9.3
10.1
10.2
10.3
104.9
11.3
10.8
10.3
10.6
10.9
10.1
P Valuef
0.03

0.03
0.04
0.05
0.02
0.49
0.08
0.36
0.08
0.03
0.38
0.15
0.54
0.90
0.37
"The test employed was the Wechsler Intelligence Scale for Children (Revised), which
 yields,  besides a total (full-scale) IQ  score, individual scores for the component
 subtests. The latter are divided into two categories: Verbal and Performance.
tjhe probability that these differences could have occurred by chance.
                                    126

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OUTLOOK FOR THE FUTURE
  Behavioral toxicology, having achieved recognition as an essential component of
toxicity evaluation, now has to mature as a discipline. In growing as a science, it has
drawn from psychology, psychiatry, epidemiology and the vast spectrum of neuro-
science. It cannot afford, however, simply to graft the methods of these more estab-
lished disciplines onto toxicologic issues. Toxicologic questions may not respond to
tactics  designed for other  problems.  Much  of physiological  psychology,  for
example, was aimed at resolving relationships between brain and behavior by trying
to localize specific brain functions. A frequent approach was to damage an area sur-
gically or electrically, and then determine the consequences for a specific behavior. A
poison, however, is likely to  act on a wide array of structures and chemical systems
within the brain, to damage peripheral nerves as well, and even to impair the func-
tion of other organ systems.  Lack of specificity, coupled with the emphasis of envi-
ronmental toxicology on early warning signs,  mandates new approaches. This is
where the challenge lies.

Choices and  Strategies
  There are a galaxy of possibilities from which  we can select approaches and specif-
ic tests and then formulate a strategy. For hazard detection, two alternatives can be
pursued. One is to devise a cluster (or battery)  of specific tests affording a compre-
hensive assessment of critical functions such as sensory  processes, coordination and
strength, learning and memory, activity and other naturalistic behaviors, and  apply
this approach in all instances. Such a standardized vehicle is helpful to both manu-
facturers and regulatory agencies because it sets boundaries to disagreement, but it
may waste  resources. Not every  agent or question demands a full treatment. The
other alternative is to proceed in a branching sequence, like a computer program-
mer's flow chart, beginning with simple assays  of behavior, then assimilating more
complex or specific ones at successive steps dictated by the earlier data.
  The Environmental Protection Agency (1980) recognizes the virtues of combining
both approaches. Once a hazard is identified by screening tests, the sequential scheme
could then pursue the questions posed by the preliminary data, conducting detailed,
intensive surveys of selected functions.  The  coming decade will also continue
another kind of scenario. Problems will arise that are a legacy of past neglect, such as
chemical dumps.  For example, at least 50, and probably many more, of the sub-
stances identified in the Love  Canal are neurotoxic. The  Health and Human Services
Committee's report (1980) on the potential health effects of toxic chemical dumps
recognizes that "Clinical assessment of the presence  of disease in individuals  is less
efficient than comparisons of quantitative health  measures in populations."It also
recognized  that we lack techniques for detecting incipient toxicity.
  If behavioral toxicology is to thrive and to fulfill its potential, it must evolve effi-
cient, feasible and sensitive testing strategies that can reflect the impact of chemicals
on  the highest levels of human function.


Development of Methods
  Techniques should be selected on  the basis of their congruence with function. A
behavioral analysis should determine the selection of tests, not the reverse. The most
critical task is not to evaluate many  different tests, but to evolve a logic or  strategy
of methods development. Each of the components offers its own problems and pos-
sibilities.

Sensory Processes—

  Reflex testing—Quick and simple tests of sensory function  are among the most
eagerly sought technologies in behavioral toxicology. A promising technique,  reflex
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modulation, has been developed during the past few years. In mature humans, audi-
bility has been tested by means of the "startle" response (a puff of air to the eyelid, for
example). Sounds presented as modulating stimuli change the amplitude of the re-
sponse in accordance with their loudness. Such audibility functions closely parallel
verbal reports, giving us reason to rely on these data. Therefore, these tests are use-
ful for organisms that cannot report to us: namely, infants, stroke victims, retardates
and animals.

  Psychophysics— Even with refinements in reflex modulation, reflex methods are
unlikely to yield subtle details of dysfunction. Although objections frequently are
lodged against psychophysical procedures because of their apparent cost and com-
plexity, occasions are certain to arise for which there is  no alternative. No matter
how many simple tests are performed, with no matter how many animals, they still
will fail to provide an adequate guide to human parameters. In the end, the costs of
studying a few  highly trained primates may be far less than any alternative proce-
dure. Further, the precision with which psychophysical  studies  can  be conducted
and the stability that can be achieved in individual animals mean that from each
animal a large harvest of information is obtained. During the next decade, some of
these procedures may be shortened, or at least made less expensive, by exploiting the
possibilities of automation derived from advances in computer technology. For ex-
ample, the visual fields of monkeys,  as described under  Psychophysical methods,
can be plotted by many of the same procedures now employed for automated testing
in humans which rely on devices that can sense the direction of gaze.

  Naturalistic behaviors—The hope is often voiced that behavioral toxicology can
bypass  complex studies   of  learned  behavior and  investments  in  complex
instrumentation by observing the behavior of animals under natural or quasi-natural
conditions. But this route offers problems of its own in deciding which behaviors are
to be observed and how they are to be specified (Reiter and MacPhail, 1979). Control
over important sources of variation is often impossible, especially when (1) animals
are sampled from a population composed of several distinct subpopulations(forex-
ample, dominant and subordinate animals); (2) numerous behavioral measurements
are recorded on each subject such as frequency counts of behaviors exhibited during
agonistic interactions (Aspey and Blankenship,  1977); and  (3) behaviors also are
composed of sequences, and only rarely are these transition relationships analyzed
adequately and reproducibly.
  Naturalistic behaviors are not a shortcut. In the absence of standardized instru-
mentation, highly trained observers and repeated checks of observer consistency are
needed to achieve repeatability from one laboratory to another. Behavioral lexicol-
ogists cannot neglect the potential of ethological approaches, however. But this will
first  require refinement of behavioral taxonomies for different species in different
situations, and also familiarity with time series analysis and multivariate statistics.
Multivariate techniques are especially  critical because several dimensions of be-
havior are inevitably measured concurrently (Aspey and Blankenship, 1977). The
relationships among these measures and the ability to compress many  measures into
a smaller number are provided  by such techniques. They also can help to define
clusters of individuals, an especially important role when confronted by subpopu-
lations of susceptible individuals.

  Complex  learned  processes—Enhanced sensitivity to what is appropriate for
ethological observations should enhance sensitivity to what is appropriate for studies
of complex learned behaviors. The main difference between them is that experimen-
tally  oriented behavioral research  focuses on a  single dependent variable while
holding extrinsic sources of variation constant (Aspey and Blankenship, 1977). They
converge in such questions as what governs the way in which animals distribute their
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foraging behavior? Baum (1981) points out that "laboratory experiments on food-
producing behavior ought  to relate to foraging for food in the wild." Foraging
theory deals with how an animal makes transitions from one source of food to the
other, but the same kind of analysis can be applied to behavior allocated to different
Schedules (payoffs) of Reinforcement. Schedules of Reinforcement, because they
specify some relationship between behavior and its consequences, are a means for
modulating the distribution of behavior in time and among alternatives. The proto-
type experiment is one in which the organism responds on one of two alternative re-
sponse devices, each having a different frequency or probability of paying off with a
reinforcer such as food. Comparing the rates of responding on the two yields a ratio
that can be related mathematically to the frequency of reinforcement associated with
each (Hursh,  1980). This  situation has  the  potential  to  be  an elegant tool in
behavioral toxicology and mimics an  important  human activity.
  Behavioral toxicology can also exploit more fully the possibilities for microanal-
ysis made available by digital computer technology (Weiss,  1970).  A relatively
simple extension, such as recording the intervals between successive responses, per-
mits more  subtle and  comprehensive analyses than more  typical measures; for
example, the application of time series measures (Weiss, 1981; Weiss and Gott, 1972).
The drug amphetamine, a stimulant, changed the pattern of responding in pigeons
that pecked a key to gain occasional brief access to grain. The drug increased the in-
cidence of aborted responses, that is, responses that failed to strike the key or that
struck it with insufficient force to close the contacts. Microanalysis plays an obvious
role in behavioral toxicology because of our interest in subtle modifications of be-
havior, especially those that render the  organism less able to adj ust to the shifting de-
mands of the environment.

Behavioral Teratology—

  Specific contributions of  behavioral teratology to environmental toxicology are
still rather modest. Behavioral teratologic research is intrinsically limited by a num-
ber of different problems. One, noted by Dews and Wenger (1979), is that each sub-
ject can only contribute one  piece of data, which can be swamped in a mass of varia-
bility. Structural deformities occur only occasionally; most individuals are at least
grossly normal, even after exposure to potent teratogens. Unless prenatal and ne-
onatal exposures have affected an array of mechanisms in a substantial fraction of
the exposed  organisms, only a huge number of subjects and rather precise tests can
detect the aftermath of a toxic exposure.
  Even so, current procedures could be enhanced. The immature organism is capable
of more complex behavior than typically is assessed in screening. Deficits in integra-
tive functions rather than reflexes may be better clues to problems as the organism
matures. The ten-day-old rat, guided by smell, is capable, for example, of orienting
toward and finding a clump  of bedding from its own nest (Leon, 1978). Infant mam-
mals also seek heat as early  as one day after birth, a behavior far more effective in
temperature regulation than reflexes (Satinoff, 1980) and easily assessed. Neonates
can also learn to suck an instrumented nipple. Newborn dogs, for example, respond
appropriately when milk delivery is governed by intermittent reinforcement sched-
ules (Stanley,  1970). Behavioral teratologic assessment optimally extends  to  the
organism's lifetime. Longitudinal studies are important because, as the brain ages, it
undergoes numerous   structural  and  functional  losses.  If  such  changes  are
accelerated by environmental chemicals, these exposures represent a hazard rather
inadequately weighted.  Only a restricted number of agents can be studied in this way
because of the cost, but certain agents and questions require such depth.
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Behavioral Epidemiology—
  Behavioral assessment in human populations poses special problems. Consider the
episode of polybrominated biphenyl (PBB) poisoning that took place several years
ago in Michigan. Dairy cattle and other farm animals were fed highly toxic PBBs (a
fire retardant formulation) instead of the nutritional supplement with a similar
name. Over thirty thousand contaminated dairy cattle were slaughtered as a result.
Many of the  farm families that consumed the tainted milk and meat complain of
nonspecific symptoms, such as depression, memory loss, lethargy, and insomnia,
which are unaccompanied by  clear clinical  indications of disease. Simple, direct
questionnaires and conventional clinical medical examinations are intrinsically un-
satisfactory.  More suitable psychometric techniques such as forced-choice inven-
tories may be used to refine subjective complaints. These typically are  made up of
multiple choice items, none of whose alternatives are clearly preferable to the others,
so that  subjects cannot choose answers designed to convey a specific impression.
  Multivariate techniques will again play a central role. Studies at the Institute of
Occupational Hygiene in Helsinki of workers in battery plants where they are ex-
posed to lead and in rayon manufacturing where they are exposed to carbon disulfide
support the potential of multivariate analyses (Seppalainen, 1974). Any one test
singly may provide  only  the most tentative discrimination between an exposed
group of workers and controls, but a group of tests is much more effective in re-
vealing differences among  populations with different exposure histories (Hanninen,
1979).

Ancillary Measures—
  Ancillary measures of central nervous system status, such as electrophysiology,
neurochemistry, and histopathology, also play crucial roles  in toxicologic assess-
ment. If behavioral deficits appear, it is certain that some underlying system has been
disturbed. But even if by behavioral criteria the organism seems fit, it is no guarantee
that adverse consequences have  not been  imposed on one  or more ancillary
mechanisms. The nervous system possesses impressive reserve capacity, both chem-
ically and morphologically, and also compensatory mechanisms that exploit that
capacity. During methylmercury intoxication, for example, nerve cells in the brain
may be destroyed in limited quantity so that the consequences do not erupt into
overt neurological or behavioral disturbances, a phenomenon that has led to the
concept of "silent damage." Both to weigh such damage and to provide correlative
measures with  behavior, other sources of information about the organism's status
need to be tapped.

  Electrophysiology—The time and costs  required to train animals for the precise
assessment of  sensor capacity and  other  complex functions  have  encouraged
speculation that  electrophysiological techniques may prove to be quicker and
cheaper as well as  a source  of additional  information. The  averaged response
method has been proposed as  an attractive  alternative, and the digital computer
makes this an easy tactic. Averaging depends on a time-locked event such as a light
flash. After each flash, subsequent electrical activity is recorded from the scalp or
deep brain electrodes for 500 milliseconds.  This period is divided into 500 one-milli-
second intervals represented by 500 computer memory locations.  The amplitude of
the electrical response at each millisecond is added to the corresponding memory lo-
cation. Although the tracing of any single response to the stimulus event is unlikely to
be distinctive, the average  of, for example, 100 flashes may yield a clear waveform
because the random, uncorrelated activity ("noise") is cancelled out. The averaged
response represents a profile of stimulus-triggered electrical activity. In theory, this
technique bypasses adaptation and training and permits direct evaluation of a par-
ticular sensory system. The method has some drawbacks. Suppose the intent is to
measure the functional capacity of the visual system. In typical experiments, the trig-


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gering stimulus is a brief, bright flash of light. Such a stimulus may not reveal much
information about incipient toxicity: early methylmercury intoxication seems to be
characterized by localized dysfunction of small areas at the boundaries of the visual
field. Illumination of the entire visual  field by a light flash will not produce changes
detectable in  the evoked potential.
  Evoked response methods are also burdened by complex problems of variability.
Estimation of background "noise" is essential before assumptions can be made about
waveform shape (Glaser and  Ruchkin, 1976). Tests of the homogeneity  or consist-
ency of waveforms can be conducted  with relatively little cost and computer time.
Simple tests of statistical significance,  not restricted by assumptions about the noise
statistics and easily programmed, are also available (Lowy and Weiss, 1968). Prob-
lems of variability are not  confined to waveform analysis but also extend to compar-
isons among subjects (Snyder, Becak, and Dustman, 1979). By addressing these is-
sues, toxicology could advance electrophysiology in parallel with its own concerns.

  Histopathology— Morphology is the best entrenched criterion of toxicity. Wheth-
er the pathology conventionally practiced in toxicology is adequate  for the nervous
system might be questioned,  however. For example, to discern depletion of a nerve
cell population in a particular area of the brain, 30 percent of the cells would have to
have been destroyed. Moreover, deranged morphology may be expressed in other,
more subtle ways. Suppose that a toxic substance administered during early devel-
opment reduces the number of nerve cell processes (dendritic spines) receiving con-
nections from other cells. Even if the number of cells remains intact, the functional
potential of the brain will have been compromised. Such a flaw in development
might not be manifested behaviorally until advanced age or disease or trauma, when
the reserve capacity of the brain has  been depleted.
  Quantitative neuroanatomy will augment conventional histopathology. Such as-
says rely  on the phenomenon that certain silver compounds penetrate into selective
nerve cells and outline them  with remarkable clarity. With proper optics and elec-
tronics, the nerve cell image can be converted into computer-readable form, and
then be subjected to pattern-recognition routines to quantify the length and number
of branches (Coleman, West and Wyss,  1973).
  Pathology often is also  a question of interpretation as exemplified by the contro-
versy over brain damage from monosodium glutamate  (MSG), the source  of the
Chinese Restaurant Syndrome. It has now been established that MSG kills cells in
the developing brain (Olney,  1980), at least in mice and rats, which is why it has been
removed from baby foods. Olney's early results were at first greeted with skepticism
but have  been upheld by later studies. The disagreement arose because specialized
cells in the brain, the glia,  consumed the debris left by the dead neurons and because
the locus of destruction was confined  to a small area on the borders of the internal
fluid system (the ventricles) of the brain and spinal cord. In addition, the critics used
a traditional cell stain that is relatively insensitive to this kind of damage.  By turning
to special techniques that differentially mark individual neurotransmitter systems
and by conducting coordinated assays of brain chemistry, the breadth  of damage
from  MSG  and allied neurotoxic amino acids has  been demonstrated (Kizer,
Nemeroff and Youngblood, 1978). With such data as guides, it was found that lower
doses alter the levels of certain critical hormones without producing overtly detect-
able cell pathology.


  Neurochemistry—Like its  morphology, the chemistry of the brain is remarkably
heterogeneous, and, like its interconnected web of structures, its chemical entities act
as components of an integrated, precisely orchestrated network. It is inevitable that
some environmental  contaminants will interfere with one or more of the myriad
processes  and  mechanisms  through  which the brain's chemical functions are
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conducted. Consider a few of the possible steps at which such agents might interfere
with normal function: (1) dietary uptake and transport to the brain of required nu-
trients such as amino acids; (2) enzymatic conversion, by one or more steps, of amino
acid precursors into functional neurotransmitters; (3) storage of neurotransmitters
at specific sites within the nerve cell; (4) release of the neurotransmitter into the syn-
apse when the nerve is stimulated; (5) metabolism and  removal of the transmitter
from the synapse; and (6) excitation responses of the postsynaptic neuron. Multiply
these actions by the 40 or so identified neurotransmitters and neuromodulators in
the central nervous system (Lipton, DiMascio and Killam, 1978) and the result is
almost as staggering an array of possibilities as that provided by behavior itself. Un-
less some specific information is available about the impact of a given chemical, the
neurochemist will have to proceed by systematic search, perhaps focusing on those
chemical constituents and  processes extensively studied in psychopharmacology.
Metabolism and synthesis of the transmitter serotonin, for example, is increased by
DDT, and its concentration in the brain decreased by nickel, manganese and perhaps
cadmium as well (Rastogi, Merali, and Singhal, 1977).
   Neurochemical methods, however, may find their widest application through test
systems that rely on special preparations of brain tissue.  Nerve cell cultures, clumps
of nerve endings, brain minces  and slices, and excised  nerve-muscle preparations
might prove especially apt for the phase of early hazard screening and its emphasis
on identification.

   Nonspecific measures—Behavioral toxicology legitimately focuses on the central
nervous system as the source of the mechanisms that determine behavior. Suchafo-
cus, however, should not block awareness that other systems within the body make
significant contributions to behavior through indirect actions on the central nervous
system. Pronounced  falls in body weight, which are common when toxic exposures
occur, have been noted in association with lead, methylmercury, acrylamide, carbon
disulfide, and triethyl tin poisoning. Disturbances of energy regulation profoundly
influence many bodily processes and inevitably will be reflected in behavior. For ex-
ample, undernutrition affects brain chemistry (Michaelson, 1980). Any report of
neurobehavioral  deficits  accompanied  by  significant  changes in body-weight
regulation or food intake must be tempered with that realization, and should move
the investigators to establish the independence and significance of their behavioral
observations apart from a profound interference with the  body's vital economy.
   The impact of other organ systems cannot be ignored either. A poison whose pri-
mary effect is impairment of liver function can easily exert profound effects on be-
havior; the neurological consequences of "hepatic coma" have been recognized since
antiquity. Liver impairment is associated with increased brain synthesis of the neuro-
transmitter, serotonin,  probably because  its precursor amino  acid,  tryptophan,
enters the brain more readily (Nutrition Reviews, 1980).  Clinical descriptions of the
aftermath of hepatic coma feature a variety  of nervous system disorders such as
tremor, grimacing, slurring of speech, incoordination, and impairment of intellectu-
al function (Victor, Adams and  Cole, 1965). Behavioral  and neurological disorders
have often been noted in clinical descriptions  of uremia and other kidney diseases.


SHORT-TERM TESTS: SOURCES AND DEVELOPMENT
  The ideal test is short, sensitive, easily quantified, simple to perform, and cheap.
Most of all, it is a valid predictor of long-term consequences to humans. Such a test,
given such an aim, is a self-contradiction. There are too many barriers to such an
ideal blending. Behavior and the central nervous  system are viewed as especially irk-
some targets of chronic exposure because the cumulative impact of a toxic agent is so
difficult to distinguish from other influences, especially  if the objective is to detect
incipient  damage and to arrest its course. Chronic and  acute exposures may also
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foster qualitatively different consequences. If specifications call for chronic evalua-
tion, why focus on short-term tests obliquely related to the main  issues?
  A question typically buried in the search for short-term assays is their reliability,
or reproducibility. Techniques that seem optimal for short-term tests, such as nat-
uralistic observation and activity measures, are often enormously variable from one
laboratory to another and from one occasion to the next (Bornschein, Pearson and
Reiter, 1980). Many investigations of lead-induced changes in motor activity were
spurred by somewhat insubstantial reports that the childhood syndrome of "Hyper-
activity" was significantly associated with the body burden of lead. Such a simplistic
translation neglects the most salient features of the clinical syndrome now called
"Attention  Deficit Disorder" by the  American Psychiatric Association. Given the
vastly different techniques of measurement and dosage regimens, the association
between  motor activity and lead resists generalization. Even more bothersome is
what the survey by Bornschein, Pearson and Reiter (1980) could not tell us. First, it
could not estimate the reproducibility of the measure because  the reports rarely
provide a quantitative  estimate of consistency from one occasion to the next. If the
measures are highly variable, what is their predictive value?  A second problem is
consistency from one laboratory to another. Activity measures are sensitive to many
variables. How many of these must be uniform to establish  consistency among lab-
oratories? Further, if automated activity measures are subject to so many extraneous
influences, can naturalistic observations, which multiply these influences by varia-
tions among observers, show any greater consistency?
   With more  complex measures, reproducibility  can be  enhanced  enormously.
Fixed-Interval Operant performance in Boston looks no different than Fixed-Inter-
val performance in Rome, and it is equally responsive to drugs. Individual animals
are also consistent. The same features of stability hold for psychophysics, which is
why we can study individual animals and make sense of the effects. To claim that
simple, "short-term" tests are cheaper than more complex procedures is specious.
What is the ultimate cost of a false positive or false negative? How many subjects
have to be studied and  how much time has to be involved in order to reach a believ-
able conclusion? Perceptive and accurate cost  accounting  should guide our
decisions.
   Short-term tests are obligatory for initial hazard identification because invest-
ments in detailed evaluation are  unlikely unless some preliminary guides are avail-
able (Tilson, Mitchell and Cabe,  1979). They also offer estimates of the appropriate
range of exposure levels. Given these limitations of role, is there any way to improve
the sensitivity and selectivity of short-term test methods? Dews and Wenger (1979)
believe that enough information may be extracted from lethality determinations and
measures of spontaneous locomotory activity (and I would add energy regulation) to
make additional increments of information unreasonably expensive. Such a point of
view does not conflict with that voiced  by Tilson, Mitchell and  Cabe (1979). In
essence, find out, with minimal resources, if the job needs to be done (hazard identi-
fication). Then attack,  with the best available measures, the main, crucial, enduring
issue: What is the risk?
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Weiss, B. Behavior as a sentry of metal toxicity. In Trace Metals Exposure and Health Effects,
   edited  by E. Di Ferrante, Pergamon Press,  Oxford, pp. 185-198, 1979.
Weiss, B.  Microproperties of operant behavior as aspects of toxicity. In Quantification of
   Steady-State Operant Behavior, edited by C.M. Bradshaw, E. Szabadi, and  C.F. Lowe,
   Elsevier, Amsterdam, pp. 249-265, 1981.
Weiss, B.,and T. Gott. A microanalysis of drug effects on fixed-ratio performance in pigeons.
   J. Pharmacol. Exp.  Ther.  ISO: 189-202,  1972.
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   timore, pp. 213-240, 1980.
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   7:67-72, 1979.
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   ganic mercury. Arch. Environ. Health 26:249-252, 1973.
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  THE  ROLE OF THE ENVIRONMENT IN CHRONIC

                 CARDIOVASCULAR DISEASE

              C. Richard Minick and Carl G. Becker


INTRODUCTION
  Cardiovascular diseases account for over one-half of all deaths in the  United
States and are of similar importance in other developed Western countries. More
than 80 percent of these deaths can be attributed to complications of atherosclerosis.
Atherosclerosis is a change in the walls of musculoelastic and large muscular arteries
characterized by lipid-rich, fibrocellular thickening of the innermost or intimal coat
of the arterial wall. Atherosclerosis produces its effects by encroachment of the lu-
mens of arteries as a consequence of intimal thickening, thereby compromising
blood flow and decreasing the supply of oxygen and nutrients to organs or tissue.
The resulting lack of oxygen, or ischemia, may lead to death of tissue or infarction.
Atherosclerosis may also result in severe damage to the medial coat of arteries with
the  consequent loss of elasticity or compliance of the arterial wall and formation of
areas of weakness or aneurysms. In many instances, the diminished blood supply
due to intimal thickening is acutely accentuated by sudden occlusion of the artery by
formation of a blood clot or thrombus on the altered luminal  surface.
  Atherosclerosis tends to involve preferentially certain arterial beds, coronary ar-
teries, cerebral arteries and arteries of the lower extremities with the result that the
effects are particularly striking in the organs and tissues supplied by these vessels.
The related clinical manifestations include angina pectoris due to ischemia of the
heart, death of myocardial tissue or myocardial infarct and sudden death or heart at-
tack, stroke or cerebral infarction, and loss of blood supply to the lower extremities
resulting in gangrene. In 1975, atherosclerosis resulted in 643,000 deaths from coro-
nary heart disease, 155,000 deaths from cerebrovascular disease and  55,000 deaths
from other diseases of the arteries. In the same year, the direct and indirect cost of
cardiovascular disease mostly due to atherosclerosis was estimated to be 50.4 billion
dollars—16.0 billion in direct costs, and 34.4 billion in indirect costs  due to loss of
productivity as a result  of either illness (8.7 billion) or death (25.7 billion).

RISK FACTORS IN ATHEROSCLEROSIS
  The causes of atherosclerosis are poorly understood. Although atherosclerosis
has been known to occur since antiquity and is found in all populations, manifesta-
tions  of atherosclerosis have reached epidemic  proportions  in  the Western
industrialized societies in the twentieth century. Investigators have attempted to cor-
relate changes in the environment of particularly susceptible populations with the
increased incidence of the disease. These environmental risk factors can generally be
divided, albeit somewhat artificially, into changes in the personal environment and
changes in the overall environment. Alteration in the fat content of the diet which
The A uihors: C. Richard Minick and Carl G. Becker are staff members of the Department of Pathology, The
New York Hospital-Cornell Medical Center, New York, New York.
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may result in hyperlipemia is an example of change in personal environment while
changes in water hardness or availability of trace minerals are examples of changes
in the overall environment.
  In general, these two categories of change also require a different approach to
their control. Personal risk factors, that is, diet-induced hypercholesterolemia, cig-
arette smoking and hypertension, may at least in part be modified by individual ac-
tion. Alterations in the general environment, such as air pollution, water hardness,
toxic chemicals, are more likely to require legislative action for their control. Thus
far, the environmental factors shown to enhance risk significantly are all personal
ones; they are diabetes mellitus, maleness, postmenopausal state, familial or genetic
factors and stress or "coronary prone" behavior  patterns in addition to the ones
mentioned above. Obesity and physical inactivity are alo suspected risk factors.


Personal Risk  Factors
  High concentrations of blood lipid,  particularly cholesterol, have been shown to
be significantly correlated with the increased risk of ischemic heart disease (IHD) in
comparisons between populations with a low incidence of arteriosclerotic heart dis-
ease and those with a high incidence and also in studies within populations that com-
pared those persons with increased risk to the remaining population (Keys, 1970;
Kannel et al., 1971). Serum cholesterol levels have been found to be related to intake
of  animal  protein  and saturated  fat (Connor  and Connor,  1972). Increased
cholesterol and cholesterol ester constitute the major lipid component of athero-
sclerotic plaques, and there is incontrovertible evidence that increased serum cho-
lesterol concentration contributes to the development of atherosclerosis in experi-
mental animals. Cholesterol carried in certain blood proteins which transport cho-
lesterol and other blood lipids are also correlated with IHD. The quantity of low-den-
sity-lipoprotein cholesterol correlates positively with the incidence of IHD in a pop-
ulation. In contrast, the quantity of high-density-lipoprotein cholesterol correlates
inversely with the incidence of IHD (Miller and Miller, 1975; Gordon et al., 1977).
Finally, studies in nonhuman primates suggest that low-density lipoproteins are
heterogeneous and that a high molecular weight low-density lipoprotein is particu-
larly associated with increased risk of coronary atherosclerosis (Rudel et al., 1979;
1981). Other studies, however, indicate that differences in serum cholesterol cannot
entirely account for increased risk either between or within populations (Gordon et
al., 1974; Garcia-Palmieri,  1973). Further, dietary and other intervention studies
have not had the anticipated effect in reducing mortality from IHD (Dayton et al.,
1969; Oliver, 1980). High blood pressure is also significantly correlated with risk of
development of IHD and the risk increases directly with blood pressure levels even in
the so-called normal range. Blood pressure was found to be one of the strongest
factors in the Framingham Study with almost a ten-fold range of IHD morbidity
over the measured range of systolic blood pressure  (Kannel et al., 1976).
  Cigarette smoking is associated with an increased risk of IHD, including Sudden
Death; data from the  International Atherosclerosis Project indicate  a relationship
between coronary artery-raised lesions and a history of cigarette smoking (Strong et
al., 1969). The risk of death for men at all ages and for women below the age of sixty-
five is directly correlated to the number of cigarettes smoked and is about 60 to 70
percent greater for  cigarette  smokers than nonsmokers. The  younger smoker  is
generally at greater risk than are older smokers. The excess mortality for cigarette
smokers  decreases when smoking is  stopped, and the mortality  rate gradually
returns toward the normal rate over a number of years.
  Several complications resulting from atherosclerosis including coronary heart
disease, sudden death, peripheral vascular disease and stroke occur more commonly
among diabetics than nondiabetics, and the difference appears particularly striking
in younger age groups. The association with peripheral artery disease is a particularly
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strong one and the vast majority of patients with this disease can be shown to be
overtly diabetic, have abnormal glucose tolerance tests or to be cigarette smokers.
Although diabetes is often associated with increased incidence  of hyperlipidemia,
hypertension, and obesity, the changes in these factors are not believed to be of suffi-
cient magnitude to explain the increased incidence of cardiovascular morbidity and
mortality. It has been suggested that increased mortality may be mediated by other
factors, perhaps involving arterial wall metabolism (Wolinsky, Goldfischer et al.,
1978).
  Other factors have been associated with increased risk of arteriosclerotic heart dis-
ease. Among these are sex, family history, personality type and physical inactivity.
Premenopausal women have a significantly lower incidence and severity of coronary
artery disease than women of the same age with artificial menopause. The use of oral
contraceptives has been found to increase the risk of IHD  disease, particularly in
young women (Hennekens and MacMahon, 1977; Lancet Editorial, 1977). This risk
is further increased when associated with  cigarette  smoking (Jain, 1976).
  Inactivity has been receiving increased attention as a risk factor particularly be-
cause of recent findings which indicate that physical activity, specifically long-dis-
tance running, is associated with a proportional increase in the high-density lipopro-
tein fraction, lipoproteins that we previously noted were inversely correlated with
increased risk of atherosclerosis. In addition, there is a decrease in total serum cho-
lesterol (Paffenbarger and Hyde, 1980). Obesity is also a risk factor although many of
its effects may be mediated through other risk factors such as  hyperlipidemia, hyper-
tension and diabetes.  Obese  individuals  also have lower levels of high-density lipo-
protein and are less physically active.
  Finally, there are families  in which heart attacks occur prematurely and with in-
creased frequency. In some  instances, familial predisposition is clearly associated
with readily identifiable factors such as various  types of familial hyperlipoprotein-
emia (Frederickson and Ferrans,  1978).  However, in others, it is difficult to be cer-
tain what the relative contribution of life style, environment and dietary habits are as
compared to genetic factors.  It is also uncertain in some instances how these genetic
factors operate although it is becoming increasingly apparent that there are hered-
itary components to a number of risk factors including hyperlipoproteinemia, hy-
pertension and  diabetes mellitus  (Department of  Health, Education and  Wel-
fare,   1971).   Mathews  (1975)  has  suggested  a  relationship  between
population frequencies of the histocompatibility antigen HLA-8 and haplotypes 1-8
and the frequency of IHD.

Environmental Risk Factors
  Although certain personal factors have been shown to be correlated with increased
risk of ischemic heart disease, these factors are believed to account for less than 50
percent of the increased risk in certain Western  populations (Stamler and Epstein,
1972; Gordon et al., 1974; McGill, 1978). Large numbers of individuals have compli-
cations of coronary artery  disease even though  known  risk factors are lacking.
Severe atherosclerosis and its complications also occur in a few individuals within
low-risk populations who share the characteristics leading to decreased risk in that
population. Taken together, these findings suggest that there are other risk factors,
acting either alone or in synergy with the known ones. There may be legitimate con-
cern, therefore, about the effects of substances that are encountered either occupa-
tionally or in our general living situation. The recognition that some environmental
factors may be important in initiation or growth of neoplasms has further strength-
ened  concern with  regard to their influence on other chronic disease processes.
However, it is important to point out that, even in the instance of neoplasia, personal
factors are believed to contribute much more  to increased risk than those in the
general environment (Waugh, 1979). General environmental factors that have been
considered to be of possible  importance include carbon monoxide, water hardness
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and intake of trace metals, occupational exposure to certain inhalants, noise, radio-
frequency irradiation, and physical and psychophysical stress (Rosenman,  1979;
Harlanet al., 1981).

Carbon Monoxide—
  Carbon monoxide (CO) is one of the more common pollutants in most industri-
alized societies. Worldwide CO production from technological and natural sources
is estimated to be at least 250 million tons per year (Astrup and Kjeldsen, 1974). Al-
though fuel consumption by motor vehicles is a major source of carbon monoxide,
the most frequent source of inhaled CO is in tobacco smoke. Cigarette smoke con-
tains between 2.7 and 6 percent CO and during inhalation and dilution with air aver-
ages about 440 ppm of CO, a level eight times greater than the maximum level per-
mitted in industry. Consumption of one to two packs of cigarettes daily may lead to
alveolar CO concentrations of approximately 50 ppm which, at equilibrium  with
blood, equals a carboxyhemoglobin (COHb) concentration of about seven percent
(Ball and Turner, 1974). Ayres et al. (1969) demonstrated COHb concentrations in
nonsmokers of 0.5 percent as compared to concentration in smokers of one or two
packs  of 1 and 3 percent, respectively. Certain occupational groups such as fire
fighters and vehicular tunnel workers also experience elevated CO exposure (Ayres
et al.,  1969; Sammons and Coleman, 1974).
   Increasing concentrations of carboxyhemoglobin (COHb) have been found to in-
fluence myocardial oxygen utilization in patients with ostensibly normal hearts as
well as those with IHD when patients are subjected to exercise. Vogel and Gleser
(1972) found that exposure to 225 ppm CO, resulting in COHb saturations of  18 to
21 percent, had little effect on normal resting myocardial oxygen consumption but
resulted in a decrease in total oxygen consumption of 23 percent at maximum exer-
cise. Increased COHb saturation to 9 percent in patients with  no evidence of coro-
nary heart disease was found to result in increased coronary blood flow and decreased
myocardial oxygen extraction (Ayres etal., 1969). In contrast, in patients with coro-
nary heart disease, a rapid increase in COHb did not result in a significant increase in
coronary blood flow indicating that they have decreased ability to  compensate for
increasing COHb concentrations (Ayres et al., 1970;  1973).
   As might be expected from these findings, Anderson etal. (1973) found that adult
men with stable angina pectoris had significantly shorter treadmill exercise times be-
fore onset of pain and that the pain lasted for a longer period of time when exposed
to CO and stressed with exercise. EKG changes also occurred earlier and lasted for
longer periods. Similarly, Aronowetal. (1972) demonstrated that exposure to vehic-
ular CO in heavy automobile traffic resulted in a significant decrease in exercise per-
formance before the onset of  angina pectoris and  EKG  changes.  Breathing
increasing concentrations of CO has also been found to shorten exercise tolerance in
patients with significant iliofemoral occlusive arterial disease (Aronow et al., 1974).
   A study in Copenhagen indicated that increased levels of COHb correlated with
increased tendency to development of atherosclerosis; those with  COHb levels of
five percent or more were found to be 21 times more likely to be affected than matched
controls with COHb below  three percent (Wald et al., 1973). Studies  of Cohen,
Deane and Goldsmith (1969) have indicated increased fatality rate from myocardial
infarction in areas of high pollution during periods of relatively increased CO con-
centration. However, others have attempted unsuccessfully to correlate the number
of myocardial infarcts with levels of ambient  CO including those  associated  with
sudden death (Kuller et al.,  1975). Exposure to increased atmospheric concentra-
tions of CO has also been found to increase myocardial irritability in humans and to
lead to myocardial fibrosis in rabbits (Kjeldsen et al., 1974).
   Unfortunately, after many years of experimentation, the findings with respect to
atherosclerosis are unclear. Astrup (1974) and Kjeldsen etal.(1968, 1972)found that
exposure to increased concentrations of CO led to increased deposition of lipid in
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aortas of rabbits fed cholesterol-supplemented diets. Interpretation of the results of
these experiments is complicated by the finding that serum cholesterol concentra-
tions in animals exposed to CO were significantly higher than diet-fed controls.
Further, the same investigators were unable to demonstrate an effect in rabbits with
normal  blood cholesterol levels, a result which was at variance with their earlier ex-
periments (Hugod et al., 1978). In nonhuman primates, exposure to increased CO
has been found to result in anatomical damage to coronary arteries (Thomsen and
Kjeldsen,  1974).
  Increased concentrations of CO in the atmosphere has also been found to have an
effect  on  fetal  development,  vascular permeability  and  cerebral  function
(McFarland et al., 1944; Lilienthal and Fugitt, 1945; Schulte,  1961, 1963; Siggard-
Andersen et al., 1967; Pauli et al., 1968; Siggard-Andersen etal., 1968; Astrupetal.,
1972; Parving et al., 1972). Impairment of cerebral function occurs at the levels of
COHb found in the smoking population.
  The results of the above studies do not support the concept of a safe threshold level
of COHb. In the presence of regional vascular insufficiency, even the lowest levels of
COHb may critically interfere with adequate oxygen delivery. A safe level of CO ex-
posure appears to be the lowest level which  can  be achieved (Turino, 1981).
Water Hardness and Trace Metals—
  Since the original observations of Kobayoshi (1957) noting a relatively high death
rate from stroke among Japanese with soft water supplies, many investigators have
attempted to correlate water hardness with mortality from cardiovascular diseases,
particularly arteriosclerosis. Water hardness is defined as a condition when the con-
centration of calcium carbonate is equivalent to the dissolved calcium and magne-
sium present (Sharrett, 1981). Data with respect to water hardness may be confound-
ed with trace mineral intake.  However, overall, none of the trace elements is consist-
ently correlated with water hardness, but in some areas at least the concentration of
magnesium appears to be independently associated (Sharrett, 1981). Several large
studies made in England, Wales, Canada, North America and an International study
have failed to resolve the question of a consistent correlation between water hardness
and mortality rates from cardiovascular disease in general  and atherosclerotic
cardiovascular disease in particular (Comstock, 1979). The association, a weak one
at best, tends to become insignificant when smaller included areas are examined
separately or when other related circumstances such as climate or rainfall are con-
sidered (Sharrett, 1981).
  Magnesium is often found in relatively high concentrations in hard water, and
intake from drinking water may account for as much as 10 to 20 percent of the total
magnesium ingestion (Sharrett, 1977). There is experimental and clinical evidence to
indicate that magnesium deficiency can cause  disturbances of cardiac rhythm
(Polimeniand Page, 1973; Iseri et al., 1975) and epidemiologic evidence that at least
some  increased cardiovascular mortality associated with soft water may be due to
Sudden Death (Peterson et al.,  1970; Anderson and LeRiche, 1971; Crawford etal.,
1977). Taken together with the finding of low myocardial magnesium levels in acci-
dent victims in these areas (Anderson et al., 1975) and in association with Sudden
Death (Behrand Burton, 1973; Anderson etal., 1975; Chipperfield and Chipperfield,
1978), this latter finding has been interpreted as  evidence that inadequate magne-
sium is a causal factor in cardiovascular disease, possibly through its effect on cardi-
ac rhythm. However, an independent association has not been established. Some
British studies indicate insignificant or trivial associations with magnesium content
of drinking water even though cardiovascular disease is significantly associated with
water hardness in the same areas (Morris et al., 1961; Crawford etal., 1968;Elwood
et al.,  1977). Further studies on magnesium metabolism and magnesium require-
ments in man would be useful (Sharrett,  1981).
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  Other investigators have attempted to correlate changes in incidence of athero-
sclerosis with trace minerals and not necessarily with those minerals that determine
water hardness although in many studies the two are confounded (Borhani, 1981).
Clinicopathologic, epidemiologic and experimental data suggest that excessive ex-
posure to several trace elements or insufficient dietary intake may be associated with
changes in the cardiovascular system which may lead to coronary artery disease. Al-
though an effect of industrial exposure has yet to be documented, exposure to cobalt
in beer has been shown to lead to a cardiomyopathy similar to that resulting from
thiamine deficiency, presumably by interfering with mitochondrial energy metabo-
lism (Alexander,  1969). Changes in the electrocardiogram have been noted following
exposure to antimony (Brieger et al., 1954), and copper smelter workers exposed to
arsenic have been reported to have increased cardiovascular mortality (Axelson et
al., 1978).
  Increased lead  concentrations have been observed in tissues and blood of patients
with hypertension (Schroeder and  Kraemer,  1974; Wedeen et al., 1975; Beevers,
Erskine et al., 1976) and in the aortas of patients with coronary heart disease (Voors
et al., 1973). Increased cadmium concentrations have also been found in tissue and
blood of some patients with hypertension (Schroeder and Buchman, 1962; Axelson
and Priscator, 1966; Glauser et al., 1976) although others have not been able to con-
firm these findings (Beevers, Campbell et al.,  1976).
  Increased  zinc to copper ratios have been reported in association with coronary
heart disease (Klevay, 1975), and there has been speculation that decreases in zinc,
with a decreased  zinc to copper ratio, may be part of the protective effect that is seen
with physical exercise and exposure to hard water. It is also suggested that increased
ischemic heart disease in patients with hypertension is due to a change in zinc to cop-
per ratios as a result of lowered copper concentrations following increased urinary
excretion  of copper.
  Decreased concentrations of chromium  have also been  observed in aortas of
patients dying of arteriosclerotic cardiovascular disease as compared to those dying
of accidents  or other diseases (Schroeder et al., 1970). Chromium concentrations in
aortas of North Americans were found to be low and declined with age (Schroeder,
1968; Schroeder et al., 1970) whereas those of African or Oriental populations were
much higher. Aortic tissue of Thailanders had more chromium than any other groups
and they have very little aortic atherosclerosis (Schroeder, 1958). Chromium defi-
ciency has also been associated with abnormal  glucose tolerance and elevated serum
cholesterol  concentrations (Schroeder  and  Balassa,   1965;  Schroeder,  1966).
Abraham and co-workers (1980a) were unable to establish a relationship between
serum chromium levels and ischemic heart disease or abnormal blood glucose levels.
However, they did observe an elevation in patients with acute myocardial infarction
suggesting the possibility that large amounts of chromium may be liberated acutely
from myocardial tissue following injury. These same investigators obtained data to
suggest that intraperitoneal injections of chromium salts may lead to enhanced
regression of diet-induced aortic atherosclerosis in rabbits (Abraham  et al., 19806).
A cause-and-effect relationship has  not been established, however.
  In conclusion, the evidence for implication  of zinc, copper, lead, cadmium and
chromium in coronary artery disease is perhaps more plausible than that of other
elements, but in no case has a strong or consistent association been established. Even
assuming a consistent association, their role as independent risk factors has not been
established nor has the mechanism of action of these elements in normal biological
processes and in cardiovascular disease been determined.

Inhalants—
  With respect to coronary artery disease, only two occupational inhalants have
been found to have a definite effect. A connection has been reported between an in-
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creased incidence of ischemic heart disease and exposure to carbon disulphide in the
viscose rayon industry (Nurminen, 1976) and glycerol nitric acid esters in the explo-
sives manufacturing industry (Weill, 1981). In the latter instance, the risks appear to
be greatest following cessation of exposure suggesting that the mechanisms may re-
sult from a rebound phenomenon perhaps with vessel spasm.
  Halogenated  hydrocarbons  and  fluoro-alkanes  have  been implicated  in
myocardial irritability and cardiac toxicity (Lehnert et al., 1974; Ravens et al., 1974;
Flowers et al.,  1975; Speizer et al., 1975). The overuse of bronchodilators for the
treatment of asthma has also  been cited in cardiovascular disease. In addition,
benzene  derivatives, pesticides, the organosilicon monomer methyl methacrylate,
butyl isocyanate, cadmium, nitrogen oxide, cobalt  and arsenic have all been sug-
gested as cardiotoxins in individual cases following occupational exposure. None-
theless, only carbon disulfide and glycerol nitrates have been well documented as oc-
cupational inhalants  that have a decided effect in  cardiovascular disease (Weill,
1981).

Physical Agents—
  Vibratory stress is a well-known environmental hazard for those using pneumatic
drills, chain saws, hand and pedestal grinding and copper swaging. Stress associated
with continued vibration leads to transient paling and paraesthesis of the fingers. If
exposure is continued, eventually persistent paleness of the finger, the so-called vi-
bration white finger, results due to permanent changes in blood supply and vessels
(Walton, 1974; Department of Health, Education and Welfare, 1977).
  Moderate increases in blood pressure have been observed in response to high or
low noise levels (Ortiz et al., 1974). Increases in adrenal secretion, particularly in
medullary  hormones  (Ortiz et al., 1974), as well as increases in serum cholesterol
(Friedman et al., 1967; Ortiz, 1974) and platelet aggregation (Maas, 1973) have also
been  noted in experimental animals following exposure to noise. Considering the
prolonged exposure of workers in various industries to increased noise levels, further
studies appear especially warranted. Exposure to radiofrequency radiation has also
been  suggested to have possible acute and chronic effects in the cardiovascular
system (Resnekov, 1981). Unfortunately, most of the available data is far from con-
clusive, and the mechanism of action of these environmental stimuli are unknown
(Harlan, 1981).

Psychological and Social Stress—
  Certain personality or behavioral patterns have been shown in several studies to be
related independently to the development of coronary heart disease (Brand et al.,
1976;  Jenkins, 1976; Haynes, Feinleibetal., 1978; Haynes, Levine etal., 1978; Weiss,
1981). Although the results of these studies are  highly interesting, they are compli-
cated  by  a lack of clear definitions of the relative contribution of behavior patterns
and psychological stress on the cardiovascular system.
  Changes in sociocultural factors have also been cited as being important in cardio-
vascular  disease, primarily high blood pressure and IHD.  Changes in incidence of
these  diseases are particularly striking for groups moving between societies or cul-
tures  but have also been observed in association with cultural changes following
moves within a society or country (Gampel et al., 1962; Page et al., 1974).  It is not
clear  to what degree the effect of these moves may be directly attributed to social
stress as compared to that portion of the increased risk that is contributed by other
environmental changes such as diet, physical activity and level of health care.
  The role of occupational stress in cardiovascular disease is also difficult to assess.
Even  though there are differences in cardiovascular mortality and morbidity in dif-
ferent occupations, there is no direct evidence that links these conditions to job-re-
lated  stress. Further, the assignment of stress levels to various job classifications is
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externally perceived and may have no validity with respect to stress on various organ
systems.
  Overall, the role of psychosocial stress in cardiovascular disease is an important
area for future investigations, especially since the interaction between stress and be-
havioral or personality traits, if shown to be important, may be amenable to various
types of modification.
  In summary, general environmental risk factors may be potentially important be-
cause of (1) the need to explain the increase in atherosclerosis and related diseases in
Western industrialized societies; (2) the magnitude of exposure to many of these
general environmental factors; (3) preliminary data available concerning the con-
tribution of some factors; and (4) the particular pertinence of some of these factors to
societies at high risk. However, for the most part the available epidemiologic data is
at best only suggestive. As is emphasized above, there is need for additional rigorous
studies to investigate these  areas further. Since evidence to implicate any of these
factors is so weak, it would be inappropriate at this time to suggest legislative action
except in those few areas where the data are persuasive.

MECHANISMS OF DEVELOPMENT OF  ATHEROSCLEROSIS
  It is important to understand more about the manner by which well-established
risk factors may contribute to the genesis and progression of atherosclerosis. An
understanding of the mechanism(s) may suggest possible interventions in lesion ini-
tiation and development even if the particular risk factor responsible cannot be en-
tirely eliminated or significantly modified. For example, evidence that platelets  may
be a factor in atherogenesis  by causing smooth muscle cell proliferation and intimal
thickening had led several  investigators to attempt to inhibit the development of
atherosclerosis by interfering with platelet function either by platelet depletion or by
pharmacologic inhibition of platelet function.Inhibition of platelet reaction with the
vessel wall has been found to decrease significantly the extent of intimal thickening
induced in experimental animals by mechanical injury, chemical injury associated
with homocystinemia, and immunologic injury due to graft rejection  (Kincaid-
Smith, 1969;  HarkeretaL,  1974; 1976;  Friedman etal., 1977; Lurieetal., 1981). In
the instance of immunologic injury due  to graft rejection, these findings may be  par-
ticularly pertinent since they suggest that  graft-induced  atherosclerosis  in organ
transplants may be almost completely  eliminated by pharmacologic inhibition of
platelet function. In this regard, it is well to remember that accentuated atheroscle-
rosis induced in allografts has been found to be a primary long-term complication of
organ transplantation in humans,  particularly heart transplants (Rider et al., 1975).
In subsequent discussions we will consider how some of the above risk factors  may
accentuate arteriosclerotic  cardiovascular disease  either  by interaction with  cur-
rently accepted mechanisms of atherogenesis or by other mechanisms.
  There are at least three major hypotheses that are considered to be important in ex-
plaining the pathogenesis of atherosclerosis: the lipid filtration hypothesis, the arte-
rial injury hypothesis, and the recently  proposed monoclonal hypothesis.  Each has
potentially important implications with respect to our environment. The lipid hy-
pothesis is  environmentally important with respect to diet and other environmental
factors that may elevate blood lipids, in particular cholesterol. The injury hypothesis
relates importantly to the environment through factors that injure arteries and  may
predispose to atherosclerosis. Finally, chemical or viral agents in the environment
may lead to mutation of proliferating smooth muscle cells resulting in focal areas of
intimal thickening—arteriosclerotic plaques—that are monotypic.  Obviously these
hypotheses are not mutually exclusive. Hypercholesterolemia could result in arterial
injury, and the response of the arterial  wall is probably modified by hyperlipemia.
Further injury could result in proliferation of a monotypic population of smooth
muscle cells through a process of selection.
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  According to the lipid filtration hypothesis, lipid accumulation is a result of filtra-
tion of increased quantities of cholesterol-bearing lipoprotein across the endothelial
lining of the arterial wall as a consequence of increased serum levels. This increased
filtration leads to lipid accumulation in the arterial wall and secondarily to intimal
proliferation and atherosclerosis. If this hypo'thesis were the sole explanation, one
would anticipate that lipid accumulation would occur in a short period of time fol-
lowing the onset of hypercholesterolemia, and atherosclerosis would be similar in all
arterial beds and within all portions of an arterial bed. However, it is well established
that certain portions of the arterial tree are more prone to lipid accumulation and
atherosclerosis than other portions. In experimental animals, it has also been repeat-
edly observed that lipid  accumulation and lesion development are not initiated im-
mediately following  the onset of hypercholesterolemia but may have their onset
several weeks later, suggesting that hyperlipemia leads to other events in the arterial
wall that are necessary for the deposition of lipid.
  Studies of human autopsies and of naturally occurring and experimentally in-
duced atherosclerosis in animals indicate that injury to arteries and the reaction of
the arterial wall are fundamental processes in the pathogenesis of atherosclerosis.
Evidence obtained during the last two decades has led to a reformulation of the arte-
rial injury hypothesis.  These findings include the observation that endothelial
damage is an important common feature of many injuries including those resulting
from mechanical, chemical and immunological stimuli (Ross et al., 1974; Minick,
1976; Ross and Harker,  1976).  Further, the blood and blood elements have been
shown to contain mitogens that stimulate smooth muscle cell proliferation. Impor-
tant mitogens are contained within the alpha granule of the platelet, the mononu-
clear cell and low-density lipoproteins in hypercholesterolemic serum (Ross et al.,
1974; Fisher-Dzoga et al., 1974; Ross and Vogel, 1978;  Martin etal, 1981). Thus, in
its modern form, the injury hypothesis proposes that endothelial injury and desqua-
mation lead to  a reaction of platelets and other blood elements with the damaged
arterial wall. Platelets and perhaps also macrophages then release mitogens that
stimulate smooth muscle cell proliferation and deposition of connective tissue re-
sulting in thickening of the innermost or intimal coat of the artery. Loss of the barrier
function of the  endothelium enhances the filtration  of macromolecules such as
cholesterol-rich lipoproteins into the wall resulting in  intimal liquid accumulation
and atherosclerosis.
  The concept that injury and reactive changes in the arterial wall may lead to ather-
osclerosis is not new. Virchow hypothesized that inflammation in arteries led to the
development of atherosclerosis and suggested that injury to the innermost coat in-
cited this inflammation  (Virchow, 1858;  Long, 1967; Saphir, 1967). The fact that
arteriosclerosis  shares important features with inflammation continues to be at-
tractive to  pathologists (Joris and Majno, 1978).
  Rokitansky (1856) and, later, Duguid (1949) suggested that encrustration  of
blood components on the lining of blood vessels at sites of injury, that is, formation
and organization of thrombi, resulted in the development of arteriosclerotic plaques.
Thus, although the injury and thrombogenic hypothesis may be viewed as different,
they in fact do share important features. Moreover, a large body of observations in-
dicates that events leading to inflammation and to coagulation of blood are often the
same or at least cooperative. This relationship becomes especially apparent when in-
flammation is induced via mechanisms related to immune reactions, both humoral
and cellular. Recent studies have implicated lipoproteins in changes in function of
components of inflammation and blood coagulation. Very low-density, intermedi-
ate, and high-density lipoprotein were found to stimulate a procoagulant activity by
blood monocytes; low density lipoprotein suppressed this stimulation  (Schwartz et
al., 1981).
  Many environmental  substances may contribute to arterial injury  and result in
atherosclerosis. In the instance of immunological injury, antigens in foreign pro-
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teins, drugs or microbes, ingested as foodstuffs or inhaled, as in tobacco smoke,
could lead to immunologic injury to blood vessels. Although we have a better under-
standing of these mechanisms today in molecular terms and have clearly demon-
strated that immunologic injury to arteries and reactive changes can lead to athero-
sclerosis, the concept that immunologic injury to vessel walls might contribute to
atherosclerosis was appreciated nearly 50 years ago. Autopsy studies of patients who
had experienced multiple attacks of rheumatic  fever indicated that  some had
developed precocious arteriosclerosis which was especially apparent in coronary
arteries (Zeek, 1932a, b). Fahr (1921) had drawn attention to the resemblance be-
tween the arterial lesions of rheumatic fever, polyarteritis nodosa and dermatomyo-
sitis. Similar arterial lesions involving the intima, media and adventitia of blood ves-
sels were also described in tissues of patients suffering from serum sickness following
injection of horse antipneumococcal serum (Klinge, 1933;Schmitt, 1935-1936; Rich,
1946; Rich and Gregory, 1947). These studies also commented on the predilection of
coronary arteries for acute immunologically mediated injury.
  More recently, precocious development of atherosclerosis has been described in
association with systemic lupus erythematosus and rheumatoid arthritis (Bywaters,
1957; Tsakraklides  et al., 1974), both diseases mediated at least in part by deposition
of circulating antigen-antibody complexes. Precocious atherosclerosis  in cardiac
and renal  homografts is presumed to be mediated by cellular or perhaps humoral
immune reactions (Rider et al., 1975).
  Taken together,  these studies of human autopsy material indicated that: (1) dis-
eases mediated by immunologic mechanisms caused arterial injury characterized by
inflammation of the walls of arteries and often thrombus formation; (2) these sites of
injury could acquire lipid and evolve into atherosclerotic plaques or into primarily
proliferative lesions containing little lipid;  and (3) the evolution of these lesions
could be rather rapid, perhaps because of protracted or recurrent episodes of injury,
leading to the precocious development of atherosclerosis.
  Early studies of experimental serum sickness indicated that arteries were targets
of injury (Klinge, 1933; Schmitt, 1935-1936;  Rich, 1946; Rich and Gregory, 1947). It
was later demonstrated that formation of antigen-antibody complexes in slight to
moderate  antigen excess in the  circulation and their subsequent  deposition in the
walls of blood vessels led to the development of arteritis (Dixon et al., 1958). Such
depositon in rabbits could be inhibited by administration  of antagonists of hista-
mine or serotonin or by depletion of platelets (Kniker and  Cochrane, 1968). It was
further demonstrated that release of histamine from platelets  could be mediated by
complement components or by a mechanism requiring IgE and basophilic leuko-
cytes. Study of the latter phenomenon revealed  that when  antigen reacted with
specific IgE antibodies on the surface of basophils or mast cells the release of hista-
mine was  triggered. In addition, the stimulated cells also released a lipid initially
called platelet activating factor (PAF) and recently identified as acetyl glyceryl ether
phyophorylcholine which  caused platelets to aggregate transiently and to release
vasoactive amines (Benveniste,  1974; McManus et al., 1980). Release of PAF also
causes transient disappearance of platelets and  leukoytes  from the circulation
(Pinckard et al., 1977). Further, PAF-induced aggregation of platelets can cause
accumulation  of platelets at sites of immune  complex deposition (Kravis and
Henson, 1977). In addition, it has been shown that challenge with antigen of animals
preferentially synthesizing IgE antibodies to that antigen also results in activation of
the clotting system (Pinckard et al., 1975).  In this connection it  has been demon-
strated that challenge of sensitized basophils also results in release of enzymes capa-
ble of cleaving and activating prekallikrein or Fletcher factor,  which in turn, can ini-
tiate activation of the intrinsic pathway of coagulation leading to clot formation,
fibrinolysis, and the generation  of bradykinin, thus potentiating the inflammatory
response and  increasing vessel permeability  (Newball, Berninger et  al.,  1979;
                                     145

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Newball, Talamo et al., 1979). This set of reactions might also enhance permeability
of the vessel wall to  other  macromolecules not involved in the  initial process,
including plasma lipoproteins.
  It  must be pointed  out that exact mechanisms are unknown  by which IgE-
mediated release of vasoactive  amines enhances deposition of circulating immune
complexes. It is conceivable that release of vasoactive amines and/ or generation of
bradykinin stimulates endothelial contraction as occurs in venules (Majno et al.,
1969), but arterial endothelial contraction has not been demonstrated unequivocally
though the endothelium of systemic arteries contains contractile protein (Becker and
Murphy, 1969; Becker and Nachman, 1973), and cultured umbilical vein endothelial
cells contain smooth muscle myosin (Moore et al., 1977). Further, it is not known if
immune complex deposition  is  potentiated in all arteries by these mechanisms. For
example, coronary arteries are commonly injured  in experimental  serum sickness
whereas cerebral vessels rarely are.
  The surfaces of endothelial cells themselves may play a role in localization of im-
mune complexes. There is some disagreement as to whether normal endothelial cells
have receptors for the Fc portion of immunoglobulin molecules or for complement
components. Immunoglobins bind to cells with Fc receptors which are avid for the
Fc of the immunoglobulin molecule. This site is exposed after reaction with the
specific  antigen. A product  of cleavage of the third component of complement,
C3b binds to specific receptors and mediates activation of other components of com-
plement. One report indicates  that these  receptors are present (Shadforth et al.,
1979). Other investigators could not demonstrate Fc or C3b receptors on normal
human pulmonary and umbilical vein endothelial cells in culture (Ryan et al., 1980;
Lyss et al., 1980). However, when these cells were infected with Herpes simplex virus
(Type 1), they synthesized Fc and C3 receptors  (Lyss et al., 1980).
  Synthesis of Fc and C3 receptors may be an adaptive function serving to enhance
the clearance of foreign antigen associated with infection. However, it may be adap-
tation at a price, increasing the chances of vascular injury, especially if the quantity
of circulating complexes is large. These findings may be especially pertinent to de-
generative vascular disease since virus infections have recently been shown to lead to
atherosclerosis (Fabricant et al., 1978; Minick et al., 1979).
  Whether immune complexes  will deposit in the walls of blood vessels is also influ-
enced by the size of the complex (those weighing 106 daltons or more are likely to de-
posit), and by the  quality of antibody, that is, its avidity for antigen (Cochrane and
Hawkins, 1968; Germuth et  al., 1972).
  The quality of antigen may also be important. The high avidity of DN A for vascu-
lar collagen may play a role in the deposition of DNA-antiDNA complexes in vessel
walls in patients with systemic lupus erythematosus. Furthermore, it has been dem-
onstrated that a glycoprotein antigen present in cured tobacco leaves and cigarette
smoke is not only an allergen, but because of its polyphenol hapten groups is capable
of activating the factor XII- or Hageman factor-dependent pathways of coagulation,
fibrinolysis, and kinin generation, events which might enhance focal inflammation
(Becker and Dubin, 1977). In this connection, it was found that activation of factor
XII— dependent pathways by intravenous injection of ellagic acid or rutin (known
polyphenol activators of factor  XII) results in inflammation in the vessel of the gall-
bladder and to a lesser extent of the lung, but not in vessels of other  viscera (Figs. 1
and 2). These latter findings  emphasize the selectivity of various types of injury for
certain vascular beds.
  Although it is not precisely known how circulating immune complexes deposit in
or on vessel walls,  depletion of complement prevents all but extremely mild changes
in artery walls in animals injected with foreign protein (Henson, 1977). Circulating
immune  complexes activate the complement  cascade and  certain  components
become associated with the complex. Chemotactic factors derived from complement
components, especially C5a and C567 complex, attract neutrophils to sites of com-
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 Figure 1.  Blood vessels in the muscularis of the gallbladder of a dog injected
           intravenously with ellagic acid. The walls and veins of an artery are
           necrotic (arrowheads) and infiltrated by polymorphonuclear neutrophils
           (hematoxylin and eosin; X500). (From Becker et al., 1980).

 Figure 2.  Section of lung treated in a similar fashion to that illustrated in Fig  1
           Increased numbers of polymorphonuclear neutrophils (arrowheads) are
           present in alveolar capillaries that are focally injured, allowing extravasa-
           tion of erythrocytes (arrows) (hematoxylin and eosin, XI,250). (From Becker
           et al., 1 980.) Similar changes are often seen in lungs of rabbits following
           inhalation of tobacco glycoprotein.
plement activation (Henson and Oades, 1975; Fehr and Jacob, 1977). Neutrophils
are then induced by C5a to produce toxic oxygen radicals which may be injurious to
vascular endothelium (Sacks et al., 1978). Furthermore, when the attracted neutro-
phils attempt to phagocytize deposited immune complexes, exocytosis occurs along
the stimulated portion of cell membrane, resulting in the release of enzymic constit-
uents, such as elastase and collagenase, destructive to vessel wall constituents. In ad-
dition, C3a and C5a, the anaphylatoxic components, of complement can cause dis-
charge of inflammatory mediators from basophils and mast cells also contributing
to intensification of the vascular lesion (Henson, 1972; Becker and Henson,  1973).
  Activation of the alternative pathway of complement which can be achieved by a
wide variety of environmental and endogenous substances, including cobra venom,
components of microorganisms, and aggregated immunoglobulins, can presumably
trigger the same events without the need for antibody. Furthermore, complement
components accelerate thrombin-induced  aggregation  of  platelets (Polley and
Nachman, 1978). This induced aggregation may play an important role in normal
hemostasis, but it might also potentiate platelet accumulation at sites of vessel in-
jury. The role of complement may not be limited to immunologically or viral-in-
                                     147

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duced atherosclerosis. Severe atherosclerotic changes that characteristically develop
in the aortas of rabbits fed cholesterol are very much milder than in rabbits geneti-
cally deficient in the sixth component of complement, indicating that activation of
the complement cascade and subsequent events described above may play a role in
the pathogenesis of diet-induced as well as immunologically mediated atherosclero-
sis (Geertinger and Sorensen, 1975).
  The attraction and adherence of polymorphonuclear neutrophils, monocytesand
platelets  to sites of immune  complex  deposition either  because of complement
components,  Fc receptors on these cells, or participation of the clotting system
through  either  the extrinsic  system  via  release of tissue factor or the  intrinsic
pathway can also result in acute vascular injury. Indeed, the various pathways may
act  synergistically.  However, the attraction of platelets and monocytes to the site of
injury influences more than just the development of acute injury. Both platelets and
monocytes have been demonstrated to release mitogens capable of stimulating the
proliferation  of vascular smooth muscle  cells and fibroblasts, and  in the case of
monocyte-derived mitogen, endothelial cells as well (Ross et al., 1974; Polverini et
al.,  1977; Ross and Vogel, 1978; Martin et al.,  1981). Since these mitogens lead to
proliferation of mesenchymal cells, they contribute to the healing of the injury but
also may stimulate the local proliferation of cells that results in the atherosclerotic
plaque.
  Experiments were conducted over the last several years in our laboratory to test
the  hypothesis that immunologically induced injury of blood vessels can act syner-
gistically with diet-induced hyperlipidemia to induce atherosclerosis. Arterial injury
was induced in rabbits by repeated intravenous injection of foreign serum approxi-
mately every four to eight weeks. Certain rabbits were fed a cholesterol-poor, lipid-
rich diet  for as  long as 17 months; this elevated serum cholesterol levels from the
normal range of 40 to 80 mg/dl to between  150 and 400 mg/dl, a range comparable to
that of adult humans in developed Western countries. Changes that developed in the
arteries  of these rabbits which strikingly  resemble  those alterations  in chronic
human atherosclerosis with respect to distribution and morphologic characteristics
in the arterial tree (Figs. 3,4,5 and 6). Earlier experiments in which rabbits were fed a
cholesterol-supplemented diet and subjected to repeated immunologic challenge
over a shorter period  of time resulted in less chronic lesions  (Minick et al., 1966).
  In other experiments, sites of injury induced immunologically as above were al-
lowed to  heal for 40 to  80 days prior to induction of hypercholesterolemia. It was ob-
served that lipid accumulation occurred preferentially at sites of intimal thickening,
that is, the sites of previous injury (Figs. 7 and 8). These findings indicate that sites of
intimal thickening may have an increased  avidity for lipid  long after the initial
injury, permitting them to evolve  into atherosclerotic plaques. In humans sites of
intimal thickening resulting from arterial injury induced many months or even years
previously may preferentially accumulate lipid and evolve as atherosclerosis later in
life  when plasma lipids increase.
  In recent experiments, vasectomized monkeys with circulating immune com-
plexes consisting of sperm-derived antigens and antibodies also were observed to
develop accentuated atherosclerosis in the presence of hyperlipidemia as compared
to sham-operated controls (Alexander and  Clarkson, 1978). Subsequently, it was
shown that vasectomy resulted in accentuated atherosclerosis even in monkeys fed a
monkey-chow diet low in lipid (Clarkson and Alexander, 1980). It has been observed
that thrombotic and obliterative changes occur in medium and small myocardial
arteries leading to myocardial necrosis in the hearts of mice with lupus-like syn-
dromes (Accinni and Dixon, 1979). In this connection, restriction of caloric intake in
these mice has  been shown to increase their life span apparently by reducing the
number of circulating  immune complexes (Fernandes et al., 1978). Campbell et al.
(1978) also found that, in mice infected with Coxsackie virus, persistence of viral
antigen in aortas and  aortic injury occurred only in  hyperlipemic mice.
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 Figure 3.  Atherosclerosis of the righl     nary artery of a 35-year-old man with
           marked coronary atheroscle     , old and recent myocardial infarcts,
           and aortic and mitral steno:     3tal cholesterol in serum one month
           before death was 279 mg per dl  Lumen of artery is narrowed by
           musculoelastic intimal thickening characterized by fatty-proliferative
           changes with pooling of lipid and very little elastic tissue (intima identi-
           fied by bar). Note similar changes in the rabbit atherosclerosis shown
           in Fig 4 (Weigert's hematoxylin and eosin, X25). (From Minick and
           Murphy, 1973).

 Figure 4.  Atherosclerosis of the right coronary artery of rabbit that received
           semisynthetic lipid-rich diet and seven injections of horse serum over a
           period of nine months. Average total cholesterol in  serum, 157 mg per
           dl. Atheromatous  intimal thickening with straightening, fragmentation,
           and reduplication  of internal elastic membrane (intima identified by
           bar).  Fatty-proliferative changes with very little elastic tissue overlie a
           layer of musculoelastic intimal thickening Note marked similarity to the
           human atherosclerosis shown  in Fig.  3. In the right lower quarter  of
           the picture, the atheromatous intima overlies a thinned media, as
           commonly occurs in human atherosclerosis The atheromatous change
           seen here  did not occur in coronary arteries of control rabbits that
           received semisynthetic lipid-rich diets without injections of foreign
           serum protein (Weigert's hematoxylin and eosin, X75). (From Minick
           and Murphy, 1973)
  Clinico-pathologic observations and experimental results indicate that immuno-
logically mediated vascular injury leading to atherosclerosis can also be induced in
transplanted tissue perhaps by cellular mechanisms.  This alteration has  been ob-
served clinically in the unexpectedly severe and rapidly developing atherosclerosis
found to occur in some human cardiac homografts (Thompson, 1969; Rider et al.,
1975). Coronary atherosclerosis in association with graft rejection  has been investi-
                                      I49

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 Figure 5.   Atherosclerosis of the left coronary artery of a 72-year-old man who
           died with disseminated rheumatoid disease. Serum cholesterol
           concentration not known. Fatty-hyaline intimal thickening with fibro-
           muscular cap and deep intimal deposits of calcium is associated with
           clusters of  foam cells. Note the striking similarity to the rabbit athero-
           sclerosis shown in Fig. 6 (hematoxylin and eosin, X105). (From Minick
           and  Murphy, 1973).
 Figure 6.   Atherosclerosis of mesenteric artery of rabbit that received semisyn-
            thetic, lipid-rich diet and six intravenous injections of bovine serum
            albumin over a period of one year. Average total cholesterol in serum,
            280 mg per dl. Fatty-hyaline intimal thickening with fibromuscular cap
            (C) and deep intimal deposits of calcium (arrowheads) is associated
            with foam cells (arrows). Note the striking similarity to the human
            atherosclerosis shown in Fig. 5 (hematoxylin and eosin, X165). (From
            Minick and Murphy, 1973).
gated in experimental animals. In one study, heterotopic cardiac homotransplants
were performed in the necks of 60 rabbits. Addition of cholesterol to the diet accel-
erated and intensified the development of atherosclerosis in coronary and myocar-
dial arteries and the segment  of aorta attached to the transplanted heart. Similar
atherosclerotic lesions were found in the arteries in hearts transplanted to rabbits
which received no lipid supplement, and no lesions were seen in the vessels of graft
recipients (Figs. 9 and 10). Thus, both humorally and cellularly mediated immune
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 Figure 7.  Lumen of rabbit mesenteric artery is narrowed by musculoelastic
           intimal thickening (bar) resembling diffuse intimal thickening in man.
           Arterial change characteristic of those seen in rabbits fed diets low in
           lipid, injected with foreign serum protein, and sacrificed 40 to 80 days
           after the  last  injection (Weigert's hematoxylin and eosin, X95). (From
           Hardin et al.,  1973).
 Figure 8.  Atherosclerosis of rabbit artery representative of that induced in rabbits
           repeatedly injected with foreign  serum protein and subsequently fed a
           cholesterol-supplemented diet 40 to 80 days after last injection.
           Morphologic and statistical evidence indicates that areas of fibromus-
           cular intimal thickening (bar) like those illustrated in Fig. 7 preferen-
           tially accumulated lipid and evolved as atherosclerosis like that
           illustrated in this artery (hematoxylin and eosin, X60). (From Hardin et
           al., 1973).
reactions may lead to atherosclerosis. In both instances endothelial injury appears to
be an important early change (Figs. 15 and 16).
  Cellular immune mechanisms may be involved in the development of atheroscler-
osis associated with viral infection. Infection of genetically appropriate strains of
specific pathogen-free chickens with Marek's disease herpes virus will lead to occlu-
sive atherosclerosis even in animals with normal levels of serum cholesterol (Fabricant
et al., 1978; Minick et al.,  1979). Although these lesions evolve in a short period of
time,  they bear  a striking resemblance to chronic atherosclerosis in man (Figs.
11,12,13 and  14). Results of these experiments may have implications with respect
to  human atherosclerosis since man is  persistently infected with  up to five  herpes
viruses.
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 Figure 9.  Cardiac allograft and recipient's heart and aorta from rabbit fed a
           cholesterol-supplemented diet and treated with immunosuppressives.
           Rabbit was sacrificed 92 days after transplantation. Note marked
           atherosclerosis of transplanted aortic valve (arrows) and ascending
           aorta (*). Atherosclerotic change ends abruptly at line of anastomosis
           with recipient's common carotid artery (C). (From Alonso et al., 1977).
 Figure 10.   Proximal left main coronary artery of cardiac allograft that functioned
             for approximately two weeks. The lumen is markedly narrowed by
             atheromatous intimal thickening (bar). Cellular cap covers lipid
             deposits deep in intima. Recipient rabbit was fed cholesterol-supple-
             mented diet and received no immunosuppressive agents (Weigert's
             hematoxylin and eosin, X35). (From Alonso et al., 1977).

 INSET      Serial section of artery illustrated in Fig. 10.  Atheromatous gruel
             containing cholesterol clefts (arrowheads)  is  present deep in intima
             (Weigert's hematoxylin and eosin, X38). (From Alonso et al., 1977).
  Since it has been demonstrated that human endothelial cells display histocompat-
ibility antigens and are susceptible to infection with a number of viruses, it is con-
ceivable that these cells can become the target of cell-mediated cytotoxic reactions in
much the same way that  infected lymphocytes became target cells for uninfected
lymphocytes bearing the  same histocompatibility determinants (Zinkernagel and
Doherty,  1979). Finally, it is known that monocytes release tissue factor in delayed
hypersensitivity reactions  resulting in activation of the extrinsic coagulation path-
way possibly worsening vascular injury (Edwards et al., 1979). As noted above in
some instances release of tissue factor by macrophages may be regulated by various
lipoproteins (Schwartz et  al., 1981).
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 Figure 11.  Atheromatous change in gastric artery of normocholesterolemic
            chicken infected with Marek's disease herpesvirus and fed a diet low
            in cholesterol. Lumen (*) is occluded by thickened intima. Athero-
            matous change is present deep in intima and media (arrowheads)
            (Weigert-van Gieson, X40)  (From  Fabricant et al., 1978).

 Figure 12.  Higher magnification of artery shown in Fig. 11. Note foam cells,
            extracellular lipid, and cholesterol clefts (arrowheads) in media
            (Weigert-van Gieson, X190). (From Fabricant et al., 1978).

  It must be pointed out that once an arteriosclerotic plaque forms the endothelial
surface may no longer be normal, characteristics of flow are certainly altered, and
evidence has been presented indicating that secretion by and consumption of blood
platelets is enhanced. Thus some  of the same pathways that are involved in the ini-
tiation of atherosclerotic plaques  may be involved in its continued growth. On the
other hand, additional  pathogenic mechanisms may play a part in lesion progression.
  Recently, a new and  very different theory about the initiation and growth of arte-
riosclerotic plaques has been put  forth by Benditt (Benditt  and Benditt,  I973;
Benditt, 1976; Pearson et al., 1977; Benditt and  Gown, 1980). This theory, now
called the monoclonal  theory of plaque formation, is derived from a highly innova-
tive set of experiments in which small tissue samples of individual artenosclerotic
plaques from the aortas of women  who were heterozygous for isoenzymes of glucose-
6-phosphate dehydrogenase  (G6PD), a sex-linked gene, were analyzed clectro-
phoretically for their content of specific G6PD variant. The results indicated that
plaques contained one or another variant (A  or B), but seldom  both. Analysis  of
uterine leiomyoma, a benign proliferation of smooth muscle cells, in the same exper-
imental design have yielded similar  data (Under and Gartler, 1965). The following
interpretation of these  data has been constructed: (1) uterine leiomyomata are con-
sidered benign neoplasms and are apparently monotypic or monoclonal; (2) arterio-
sclerotic plaques appear to be monotypic or monoclonal; (3) therefore, arteriosclerot-
                                    153

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 (13)
 Figure 13.   Cross-section of heart of a chicken infected with Marek's disease
             herpesvirus and fed cholesterol-supplemented diet for 15 weeks. The
             lumens of major coronary arteries are occluded by atherosclerotic
             change as seen in the two arteries in the upper portion of this photo-
             graph (arrowheads) (X2). (From Minick et al., 1979).

 Figure 14.   Major coronary artery of chicken infected with Marek's disease
             herpesvirus and fed a cholesterol-supplemented diet for 15 weeks.
             Cholesterol clefts (arrows) are present in atheromatous intima (bar)
             adjacent to the media. The subjacent media is markedly thinned.
             There is no cellular reaction in adventitia and little adventitial thick-
             ening (Weigert-van Gieson, X38). (From Minick et al., 1979).

ic plaques are also benign neoplasms; and (4) if so, then the arteriosclerotic plaque
has arisen as a consequence of neoplastic transformation of smooth muscle cells.
  This construction has very important implications for our understanding of the
pathogenesis of atherosclerosis especially in relation to the nature of environmental
factors which may contribute to  it. This theory raises the possibility that a large
number of environmental mutagens, such as those in cigarette smoke or tryptophan
derivatives in cooked  meat and an unidentified host of promoting substances, may
contribute to the initiation  and continued growth of arteriosclerotic plaques.  It is
also implied  that the mechanisms described  above, immunologic, inflammatory,
and  thrombogenic factors,  may  have something to do with intimal thickening in
arteries, but not with the formation of the arteriosclerotic plaque. However the data
leading to this conclusion are reproducible in some but not all laboratories (Pearson
et al., 1977; Thomas et al., 1979). The interpretation can be challenged.
  The question is whether evidence of monoclonality or monotypia necessarily im-
plies mutation or transformation.  In other systems, the answer is no. Immunization
of rabbits with streptococcal teichoic acid stimulates the production of monoclonal
antibody to teichoic acid in a significant number of recipients (Osterland et al., 1966).
The cells producing the monoclonal antibody do not constitute clones of  neoplastic
                                     154

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            (15)
            (16)
 Figure 15.  Photomicrograph of endothelial surface of rabbit that received one
            injection of horse serum and was sacrificed 15 days later Endothe-
            lium is partially denuded and red cells and platelets adhere to the
            surface (arrowheads). Note rolled edge of endothelial cell where it is
            separating from the aortic  surface (arrows) (XI,290). (From Minick et
            al.,  1978)

 Figure 16.  Lummal surface of large coronary artery of transplanted rabbit heart
            that functioned for 18 days. There is complete loss of endothelium
            with platelets adherent to  subendothehal basement membrane deep
            within the media (arrowheads) (X7.600)  (From Alonso et al.,  1977)

plasma cells. They are cells which differ from neighboring lymphocytes and plasma
cells only in terms of their capacity to multiply and secrete a unique product in re-
sponse to a certain stimulus. The responders are selected in response to the stimulus.
The question of selection as applied to  the monotypic character ol certain arterio-
sclerotic plaques has been raised by Thomas and colleagues (I979).
  Further, suggesting that mutational events account for the monotypic  character
of certain plaques appears to ignore the evidence indicating that areas ol diffuse in-
timal thickening evolve into arteriosclerotic plaques. Data of Thomas et  al. (1979)
indicate that such areas are ditypic. Evidence cited earlier indicates that such diffuse
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intimal thickening induced experimentally by immune injury can, in the presence of
diet-induced hyperlipidemia, evolve into atherosclerotic plaques closely resembling
those in humans (Hardin et al.,  1973). On the other hand, areas of intense cellular
proliferation may be areas in which mutational events become more likely, leading to
the emergence of cancer cells in ulcerative colitis and from a pool of atypical, but not
malignant cells in the lungs of cigarette smokers.
  Assuming, however, that some or perhaps even many plaques are monotypic, is it
necessary to invoke neoplastic transformation as an explanation? Scanning electron
microscopic studies of the aorta in rabbits made serum sick by injection of foreign
protein indicate that in some instances sites of injury of the endothelium are very
small, involving only a few cells per site (Minick,  1976; Sharma and Geer, 1977).
Such injury also involves focal damage to the underlying intima and media. If we can
assume that at the site of injury some smooth muscle cells may be killed outright and
that another small number are capable of responding to mitogenic stimuli, then it is
possible for the population of smooth  muscle cells at this site to be skewed such that
the evolving plaque is monotypic. This explanation does not require selective ad-
vantage of one monotypic cell over the other.
  Finally, it must be pointed out that neither the monoclonal hypothesis interpreted
as representing  neoplastic transformation nor  the injury  hypothesis is exclusive.
Perhaps, from the standpoint of examining the effect of environmental factors on
the pathogenesis of atherosclerosis it is best at the present time to consider both
hypotheses.

PATHOGENESIS OF ATHEROSCLEROSIS AND  RISK FACTORS
  The next question to be considered is how theories of the pathogenesis of athero-
sclerosis relate to known risk factors  for death from IHD.

Hyperlipemia
  Data from epidemiologic studies, chemical analyses of diseased tissue and animal
experiments substantiate the importance of plasma lipids, particularly cholesterol in
the pathogenesis of the atherosclerotic lesion.  Despite the importance of hyper-
lipemia, the mechanism by which it leads to atherosclerosis is not known. Serum
lipids and lipoproteins may be the primary factor. It is possible that, in the presence
of increased  plasma concentrations, increased filtration  of lipoprotein  into the
arterial wall may lead to proliferation of smooth muscle cells and enhanced  con-
nective tissue synthesis resulting  in formation of a fibrous plaque. Low-density lipo-
protein from hyperlipemic sera has been shown to be mitogenic for smooth muscle
cells  (Fischer-Dzoga et al., 1974). However, this hypothesis does not explain the
distribution of atherosclerosis within the arterial tree and its segmental localization
in a particular artery.
  Experiments completed over the past several years have delineated an elaborate
physiologic system important to  cholesterol metabolism of the entire organism as it
relates to the cholesterol economy of the cell. These findings have provided interest-
ing insights into the control of concentrations of low-density lipoproteins particu-
larly with  regard to the possible interaction between genetic and  dietary factors.
However, they have yet to furnish more specific insights into lipid transport and ac-
cumulation in the arterial wall other than to function as a scavenger in response to
increased concentration of low-density lipoproteins (Brown etal., 1981). Recent ex-
periments  of Davies (1981) have  provided interesting new insights into the influence
of growth  on endocytosis and of platelet-derived growth factor on high-affinity re-
ceptors for low-density lipoprotein.
  On the other hand, it is possible that many substances in the environment lead to
injury to the arterial wall, selectively damaging certain portions of the arterial bed
more than others. Depending on the environment and the injurious stimulus, these
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sites of injury in the arterial wall will evolve either as fibrocellular atherosclerotic
lesions or as areas of fibromuscular intimal thickening. In the presence of increased
serum lipids, fibromuscular intimal lesions will accumulate various quantities of cel-
lular and extracellular lipid. In especially severe and sustained injury, appreciable
lipid may accumulate even in the presence of normal serum lipid levels. Alternatively,
sites of injury evolving in the presence of normal serum lipid levels may evolve as
areas of fibromuscular intimal thickening resembling diffuse intimal thickening in
human arteries. Such areas can be shown to contain increased lipid as compared to
uninvolved arteries (Falcone et al., 1980).
  It is widely accepted that injury favors lipid accumulation within the arterial wall
by altering the barrier function of endothelium, thus allowing increased quantities of
cholesteryl ester-rich  low-density lipoprotein to gain access to the cells. Although
appealing in its simplicity, this theory does not explain the preferential lipid accumu-
lation in sites of fibromuscular intimal thickening that have evolved from previous
injury (Hardin et al., 1973). Our experiments indicate that lipid accumulates prefer-
entially in areas of fibrocellular intimal thickening covered by endothelium and not
in such areas lacking endothelium, as would have been anticipated if lipid accumula-
tion were simply a function of the presence or absence of the endothelial barrier
(Minicketal., 1979; Falcone etal., 1980). We also demonstrated cholesterol accumu-
lation in an injured re-endothelialized aorta was correlated with serum cholesterol
concentration; in contrast, there was no correlation over a range of 900 mg per dl in
areas  devoid of endothelium (Falcone et al., 1980).
  Our experiments indicate that there is greater accumulation of radiolabeled lipo-
protein in re-endothelialized areas of an injured aorta as compared to adjacent de-
endothelialized areas (Falcone et al., 1981). This may result from the trapping of
lipoprotein there since the re-endothelialized neointima also accumulates increased
quantities of glycosaminoglycan (Minick et al., 1977; Wight et al., 1979),  and cer-
tain glycosaminoglycans bind to low-density lipoproteins (Iverius, 1972). Finally,
results of other experiments indicate that injury leads to an alteration of a number of
cellular enzymes responsible for the synthesis and degradation  of cholesteryl esters,
and that the endothelium modulates those activities (Hajjar et al., 1981). In  sum-
mary, it appears that in this model system injury leads to alterations in pro'teoglycan
metabolism that favor accumulation of cholesterol and cholesteryl ester-rich lipo-
proteins in the previously injured re-endothelialized aorta. At the same time, other
metabolic perturbations in the enzymes responsible for cholesteryl ester degradation
further enhance its accumulation in this area.
   Lipids and lipoproteins by themselves may be an important source of vascular in-
jury and may damage the endothelium. Both low-density lipoproteins and hypertri-
glyceridemic very low-density lipoproteins have been shown to damage endothelial
cells in vitro (Hendrikson et al., 1979; Gianturco et al., 1980). Experiments of Ross
and Harker (1976) indicate that modest diet-induced hypercholesterolemia in non-
human  primates will lead to endothelial injury and denudation. Others have not
been able to demonstrate this (Goode et al., 1977; Taylor  et al., 1978). However, it
should be emphasized that the denudation is an extreme form of endothelial injury.
Small areas of the endothelial desquamation rapidly re-endothelialize primarily due
to migration of adjacent endothelial cells (Reidy and Schwartz, 1981). The possibil-
ity exists that hyperlipemia may induce more subtle forms  of endothelial injury that
lead to endothelial desquamation without denudation.
   The response of the arterial wall to injury may result in increased exposure to var-
ious mitogens, which may be important in uptake of lipoproteins and lipid  accumu-
lation. Experiments of Daviesand colleagues (Davies and  Ross, 1978; 1980; Davies
et al., 1980; Davies, 1981) and Vlodavsky et al. (1978) indicate that in  some in-
stances cell growth stimulated by platelet-derived growth factor and other mitogens
may increase bulk phase endocytosis in smooth muscle cells and endothelial cells. In
addition, platelet-derived growth factor stimulated high affinity binding, uptake and
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degradation of low-density lipoprotein by smooth muscle cells and binding by endo-
thelial cells that was independent of its effect on cell growth.

Cigarette Smoking
  Cigarette smoking is one of the best defined risk factors for atherosclerosis and
heart attack. It not only is a major risk factor itself, but it acts synergistically with
others such as hyperlipemia. For reasons that are unclear, the younger smoker is
very much more at risk of heart attack than the older smoker. Recent experimental
data suggest that nicotine can injure endothelial cells of umbilical arteries ex vivo, as
assessed by decreased production of prostacyclin (Stoel et al., 1980), and also may
lead to endothelial injury in vivo (Booyse et al., 1981). Carbon monoxide has also
been shown to potentiate the deposition of lipid in arterial walls, perhaps by damage
to vascular endothelium (Thomsen,  1974). However, the experimental data  are
equivocal. Moreover, the animal  experiments that were designed to measure the ef-
fect of carbon monoxide on vessels were of relatively short duration compared to the
number of pack-years of the average smoker.
  Recently, a highly allergenic glycoprotein has been isolated from cured tobacco
leaves and from cigarette smoke  to which approximately one-third of individuals,
smokers and nonsmokers, exhibit immediate cutaneous hypersensitivity. Repetitive
immunologic challenge with tobacco antigen might contribute to arterial injury and
the development of atherosclerosis (Becker et al., 1976; Becker and Dubin, 1977).
The same antigen also contains polyphenol haptens which enable it to activate  the
coagulation factor XII  or  Hageman factor-dependent pathways  of coagulation,
fibrinolysis, and kinin generation. Intravenous injection of these polyphenols can
also result in vascular injury and thrombosis (Figs. 1 and 2). Both allergically medi-
ated reactions and factor  XH-dependent reactions  might act synergistically to
induce vascular damage.
  It is also important to point out that the heart in the hypersensitive host is a target
organ for anaphylactic reactions  and responds to antigenic challenge with a fall in
contractility and  coronary  artery perfusion and the development  of a variety of
arrhythymias,. including ventricular fibrillation (Capurro  and  Levi, 1975). These
changes have been induced experimentally with tobacco antigen raising the possibil-
ity that Sudden Cardiac Death in some smokers may be mediated by allergic mech-
anisms (Levi et al., 1982). These data may explain the apparently greater risk of cig-
arette smoking for the younger smoker, that is, this group includes  those hypersen-
sitive to constituents of smoke. If they die of heart attacks, they are no longer repre-
sented in the population and  smoking decreases as a risk factor.

Hypertension
  With regard to hypertension,  mechanisms of increased risk are also unclear.
Experimental evidence from hypertensive animals indicates that the walls of arteries
have increased permeability to several plasma proteins (Duncan et al., 1965; Weiner
et al., 1969; Bretherton et al., 1976), which may be important in explaining both the
intimal  thickening and  lipid accumulation that are accentuated by hypertension.
The mechanism of the increased permeability is not clear and experimental data are
conflicting. It has been  attributed to increased quantities of angiotensin II (Giese,
1964; Robertson  and Khairallah, 1972;  Giacomelli et al., 1976).  Some observers
have noted that the incidence of cardiovascular complications is higher in hyperten-
sive patients with high plasma renin concentrations than in those with low plasma
renin concentrations (Brunner et  al., 1972; 1973), but the statistical evidence for  this
has been challenged (Doyle et al., 1973; Mroczek et al., 1973; Genestetal, 1974;
Kaplan,  1975). Further, Gabbiani et al. (1979) were unable to confirm a relationship
between high circulating renin levels and endothelial permeability. Overturf et al.
(1981)  recently observed that the amount of atherosclerosis  induced by low renin
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and high renin hypertension was not significantly different in rabbits fed a cholester-
ol supplemented diet.
  Altered hemodynamic factors have also been considered to be of importance
(Stehbens, 1975). It has been suggested that hypertension leads to endothelial dam-
age and increased cell turnover (Schwartz and Benditt, 1977). On the other hand, in-
creased replication could represent an attempt to adapt to dilatation and increased
vessel size resulting from hypertension (Wolinsky,  1972). In this regard, Schwartz
and Standaert (1982) found that endothelial replication increased following acute
increases in blood pressure but was not sustained as the blood pressure elevation be-
came chronic. Endothelial turnover may be used as an indirect measure of injury but
this finding suggests that it is associated with acute change in blood pressure and not
with chronic hypertension. Finally,  activities  of  several  lysosomal degradative
enzymes within the aortic wall have been shown to change following the onset of ex-
perimental hypertension in rats (Wolinsky, Capron et al., 1978). It may seem diffi-
cult to reconcile an increase in a lysosomal degradative enzyme with the increased
cholesteryl ester accumulation seen in atherosclerosis   due to hypertension.
However, it should be emphasized that the increase  in activity may not be sufficient
to degrade the increased quantities  of lipoprotein entering the wall. As a result, cho-
lesteryl esters may accumulate in association with hypertension. We have observed
similar phenomena with regard to arterial injury, lipid accumulation, and choles-
teryl ester hydrolase  activity (Hajjar et al., 1981).

Diabetes Mellitus
  Even though studies in humans may clearly identify diabetes mellitus as one of the
risk factors associated with atherosclerosis (Garcia et  al.,  1974; Ostrander et al.,
1965) the explanation(s) for the increased risk is not  clearly defined. Several lipopro-
tein abnormalities  have been identified in diabetes (Stout,  Bierman and Brunzell,
1975), but they generally  are  considered to be insufficient to account  for the
magnitude of increased risk.
  The diabetic patient also  has a well-defined abnormality of the microvasculature,
characterized by thickening of the basement membranes(Maueretal.,  1976). More-
over, several of the glycoproteins in basement membranes may be excessively glyco-
sylated (Spiroand Spiro, 1971; Biesswengerand  Spiro, 1973). Abnormalities associ-
ated with changes in  the microvasculature include diabetic retinopathy, glomerulo-
sclerosis and diabetic cardiomyopathy. Based on observations of decreased in vitro
life spans of diabetic as compared to nondiabetic fibroblasts, Vracko and Benditt
(1974) have suggested that the basis for the basement membrane thickening is an in-
creased susceptibility of the endothelium of diabetics to injury,  and, with increased
endothelial turnover  in vessels, each new population of endothelial cells contributes
its layer of basement  membrane to the overall thickness of the blood vessel. It is not
known whether similar endothelial damage and abnormalities of glycoprotein and
basement membrane synthesis or  degradation may contribute to the accentuated
atherosclerosis seen in larger blood vessels.
  Results of recent experiments of Wolinsky and his colleagues indicate that there
are abnormalities of the activity of catabolic enzymes in the arterial wall of animals
with experimentally induced diabetes; they were able to show that there is a decrease
of several lysosomal enzymes,  including acid cholesteryl ester hydrolase, in both
streptozotocin- and alloxan-induced diabetes in rats. Abnormalities  of lysosomal
enzymes were restored to normal following administration  of insulin. They specu-
late the decrease in these catabolic enzymes may account for some lipid accumu-
lation in the wall of the diabetic human artery, especially considering the possibility
of a need for capacity to degrade increased amounts of cholesteryl ester bearing lipo-
protein in the diabetic (Wolinsky,  Capron et al., 1978; Wolinsky, Goldfisher et al.,
1978).  Further experiments revealed that the increased activity of acid  cholesteryl
ester hydrolase seen in arteries of hypertensive rats was decreased in the presence of
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experimental diabetes, suggesting that in the presence of hypertension and possible
increased filtration of lipoprotein the ability to degrade cholesteryl ester is decreased
(Wolinsky, Capron et al., 1978). However, these findings should be interpreted with
caution since, as the authors point out, experimental models of diabetes in rats with
severe insulin deficiency, glucagon excess, and absent ketosis are not a faithful
counterpart of the complicated metabolic abnormalities in human diabetes.
  Stout, Bierman, and Ross (1975) have suggested that the relative excess of insulin
seen in many diabetics may make important contributions to increased risk. This
hypothesis is based on the finding that insulin enhances growth of vascular smooth
muscle cells in vitro  and hence could be of importance in proliferation of smooth
muscle cells and  intimal thickening in vivo. Finally, the association of diabetes with
autoimmune diseases and virus infections suggests intriguing possibilities with re-
gard to vessel injury  in diabetes and the interaction of viral, immune and immuno-
genetic factors.


Sex,  Age, and  Physical Activity
  Being a male also appears to predispose one to heart attacks. The mechanisms un-
derlying this association are  unclear, and a large number of experimental and clinical
studies have not  solved this puzzle (Lancet Editorial, 1977). In fact, administration
of estrogen potentiates heart attacks in males and birth control pills can apparently
precipitate thrombotic and ischemic heart disease events in females (Hennekens and
MacMahon,  1977). As noted  earlier, smoking has been shown to interact signifi-
cantly with use of birth control pills (Jain, 1976). Recent findings demonstrating the
presence of androgen receptors in hearts and aortas of male baboons may provide an
interesting lead for further  studies (Lin et al., 1980; McGill et al., 1979).
  The likelihood of heart attack, aortic  aneurysm and clinical symptoms of periph-
eral vascular disease all increase with age. The most obvious explanation for this is
that arteries become progressively narrowed to the point that ischemia occurs, or to
the point that a clot or thrombus can occlude the vessel. Changes in the patterns of
blood flow over  plaques may perturb blood platelets, and also potentiate further
endothelial cell injury. Also, a wide variety of substances which are not demonstrably
thrombogenic when injected or absorbed into the blood stream become so in the pre-
sence of stasis which makes  their weak capacity to activate clotting manifest. The re-
action of other body systems that may bear on atherosclerosis may change with ag-
ing, for example, the immune system, and many older people have increased levels of
circulating immune complexes.
  On the other  hand, every pathologist has seen examples of atherosclerotic but
ectatic coronary  arteries in hearts of older patients. A similar phenomenon is partic-
ularly striking in the  Masai in Africa. Masai have a low incidence of ischemic heart
disease although coronary artery intimal thickness is as great as that of men in the
United States (Mann et al., 1972). Bond  and co-workers (Bond, Adams and Bullock,
1981; Bond, Adams, Kaduck  et al., 1981) showed that the coronary arteries  of cy-
nomologus monkeys (crab-eating macaques) fed lipid-rich diets developed arterio-
sclerotic plaques. However, in some of  these macaques  the lumen was larger, even
though the intimal surface area involved by disease was increased. This is explained
by the finding that the overall size of the artery is increased. Reasons for the ectatic
atherosclerotic arteries seen in man are obscure. They are possibly genetically deter-
mined since longevity is familial.
  There is also evidence that physical inactivity may lead to increased cardiovascular
mortality, particularly these deaths associated with ischemic heart disease. Epidemi-
ological studies of London  transport workers and British civil servants, San Fran-
cisco longshoremen and Harvard University alumni have all indicated that regular
exercise can promote health and cardiovascular fitness  (Morris et al., 1966; 1973;
Paffenbargeretal., 1977; Chave et al., 1978; Paffenbarger et al., 1978). This effect is
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partly independent of other risk factors such as cigarette smoking, hypertension,
obesity, and genetic factors implicated in coronary heart disease. There are several
possible explanations. Exercise  has been found to increase fibrinolytic activity in-
duced by experimental venous occlusion (Williams et al., 1980). The implication of
this finding seems obvious since thrombus formation appears to be important in the
pathophysiology of myocardial infarction, stroke and pulmonary embolism. Exer-
cise has also been found to lower plasma triglyceride, low- and very low-density lipo-
protein cholesterol  while increasing the proportion of high-density lipoprotein cho-
lesterol. The mechanisms leading to these changes is poorly understood; however,
they appear to be related to the level of exertion and not entirely related to weight re-
duction (Huttenen  et al., 1979; Wood and Haskell,  1979; Hartung et al.,  1980).
  Wolinsky and colleagues (1979) showed  that exercised rats had remarkable
changes in the activity of catabolic enzymes in their aortas. Activities of four of six
hydrolases, including acid cholesteryl ester hydrolase, were significantly increased;
acid cholesteryl ester hydrolase was the most effected, showing an increase of 25 per-
cent to 30 percent above control levels. Such findings suggest that in humans regular
exercise may lead to an increased capacity of the arterial wall  to catabolize chol-
esteryl ester. Finally, exercise has well-known salutory effects on cardiac function
(Astrand and  Rodhal, 1970).
   The question may now be raised as to how the pathogenetic mechanisms can be
used to understand the effect of the environment on development and progression of
atherosclerotic cardiovascular disease and its  complications and to develop hypoth-
eses concerning the recent, unexplained decrease in deaths from arteriosclerotic car-
diovascular disease. We have discussed several diseases mediated by immune mech-
anisms that are associated with precocious atherosclerosis. It is reasonable to hy-
pothesize that other disease states in which similar mechanisms are operative might
permit the development of atherosclerosis at an accelerated rate. For example, viral
hepatitis, type B, can, in some humans, result in a condition in which circulating im-
mune complexes consisting of viral antigen and host antibodies deposit in the walls
of blood vessels inducing injury and inflammation and resulting in a disease called
periarteritis nodosa (Michalak,  1978). Not all cases of periarteritis are associated
with hepatitis indicating that response to other endogenous or exogenous antigens
can also result in this severe form of vascular injury. Also, only a small fraction of
people with viral hepatitis develop periarteritis. However, as many as 15 percent of
patients with hepatits develop arthralgias or joint pain early in their illness indicating
that  transient circulation and deposition of immune complexes in tissues have oc-
curred. It may be that in years hence retrospective study of such patients will reveal
that  they have a higher incidence of atherosclerosis and its complications.  Viral in-
fection of vascular tissue may produce  local injury and initiate the proliferative
change that can  lead to  the development of  atherosclerosis. Further, viruses have
been shown to lead to diabetes  in experimental animals and humans (Yoon et al.,
1979; Notkins, 1979). It is also conceivable that oncogenic viruses might contribute
to growth of atherosclerotic plaques. In this connection it is important to remember
that  Marek's disease virus which can induce atherosclerosis in some strains of chick-
ens can induce malignant tumors of lymphoid tissue in others. Of course, it is not
known whether the induction of atherosclerosis or of lymphomas in the infected
chickens proceeds  via similar mechanisms.
  Immune injury may also be triggered by ingested or inhaled antigens. We have dis-
cussed allergens  in cigarette smoke  that can provoke anaphylactic responses  and
activate the coagulation system. Can inhalation  of similar products from other
plants in large quantity in the working place, for example, in cotton mills, also result
in vascular injury? Is there sufficient absorption of dietary antigens to produce vascu-
lar injury in the sensitized host? A recent report indicates that in a form of glomeru-
lonephritis, the surface immunoglobulin form IgA2is present selectively in immune
deposits. IgA2is  present in  far less quantity than IgA in plasma. One way of inter-
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preting this observation is that antigen-IgA2 complexes were formed in the lungs or
gut, reabsorbed, and then deposited in the renal vasculature (Andre et al, 1980). If
this interpretation is valid, then the antigens concerned probably were derived from
the environment, possibly the diet.
  Chemical initiators or promoters of tumor growth may also contribute to the
growth of atherosclerotic plaques (Albert et al.,  1977). This interesting suggestion,
based on the monoclonal hypothesis set forth by Benditt, has provided an important
new direction in atherosclerosis research.
  Finally, since the formation of a thrombus within an arteriosclerotic vessel is what
initiates a heart attack or stroke in most instances, identification of environmental
substances which singularly or acting synergistically potentiate thrombus formation
is an important area that needs further study. The fact that such substances have
been described  in cigarette smoke raises the possibility that other commonly in-
gested materials may also potentiate  thrombogenesis. Genetic or acquired defects in
the regulation of clot formation might act synergistically with  these substances to
potentiate clot  formation.  The  association between taking oral contraceptives,
smoking, and the increased occurrence of heart attacks and strokes is an example of
such an interaction.

SHORT-TERM TESTS
  There are, at present, no short-term tests by which agents in the external environ-
ment  can be assayed for  a contributing role in atherogenesis. The development of
such tests might take the direction of measuring the effect of various agents on vas-
cular cells in tissue culture. However, this approach would be rather simplistic given
the complicated nature of the disease in vivo, and generalizations from results in
such models might be misleading. Alternatively, the effect of environmental agents
on the induction of atherosclerosis  can be measured in nonhuman  primates and
other animal models. Such experiments are exceedingly costly and time consuming
and their applicability to  human disease can always be questioned. Although there
are no short-term tests, there are a number of relatively new  direct and indirect
methods for clinical evaluation of the extent and severity of atherosclerosis in vivo
such as intravenous angiography, cardiac imaging and Doppler methods in ultra-
sonography and scintigraphy. These new methods may be useful in evaluating the ef-
fect of various  types of intervention on the progression or regression of human
arterial disease.
  With regard to short-term tests, measurements of personal factors that can con-
tribute to the development of atherosclerosis and  its complications are performed
regularly. These tests include the measurement of blood pressure, degree of obesity,
blood glucose and serum lipids  (cholesterols, triglycerides, and lipoproteins). To
these tests might be added monitoring of cardiac rhythm. Depending on the results,
a variety of therapeutic or preventive  approaches can be taken.  Detection  of
ventricular premature contractions  and appropriate therapy can reduce the inci-
dence of Sudden Cardiac Death.

RECENT TRENDS IN CARDIOVASCULAR MORTALITY
  Myocardial infarct (heart attacks) will result in over half a million fataltites in the
United States this year. However, despite the seriousness of the problem, there is
evidence indicating that there has been a general and continuing decrease in cardio-
vascular deaths in the United States which began in 1968. In real figures, 20 percent
or 200,000 fewer deaths occurred in  1977 than would  have been predicted in 1968.
  Unfortunately, understanding the nature of this decline may  be as elusive as un-
raveling the nature of the disease process itself; a decrease in hospital mortality from
myocardial infarction and a decline in the known risk factors may have contributed
(Wolinsky, 1981). As a result of more effective acute care of myocardial infarction
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there has been a decline in hospital mortality of about one-third during this period of
time (to place this in proper perspective it is well to remember that over one-half of
deaths occur outside of the hospital). Increase in efficiency of identification of hyper-
tensive patients and effective therapy have  increased  dramatically since 1972.
Further, in the past decade, a nutritional change has occurred in the United States,
and there has been a reduction in the consumption of fluid milk and cream of 19.2
percent, butter 31.9 percent, animal fats and oils 56.7 percent, and eggs 12.6 percent.
Overall, the average intake of saturated fat has dropped about four percent and
cholesterol 10 percent. Consumption of polyunsaturated fats has increased about 55
percent. There has been a general decrease in serum cholesterol for middle-aged men
of approximately seven percent. There has also been a decrease in cigarette smoking
by adult males although the overall cigarette consumption has not decreased.
  From known data regarding the overall consumption of each  of these substances,
it is possible to make predictions of the effect of changes of this magnitude on cardio-
vascular mortality. A decrease of mean cholesterol levels of 5 mg per 100 ml would
be expected to result in a decline of 4.3 percent in cardiovascular deaths in six years.
A 2 mm drop in blood pressure that can be attributed  to the reduction cholesterol
would be expected to triple the decline in cardiovascular mortality. The reduction in
cigarette smoking by middle-aged men (age 35 to 75) would be expected to decrease
further the predicted mortality by an overall figure of 18 percent as compared to the
actual decrease of 21 percent observed in the United States from 1968-1976 (Wolinsky,
1981). Obviously, the validity of the available data also  has implications with regard
to the diet-heart hypothesis and proposed dietary modification in a large segment of
the American population, a topic of recent controversy. Although these arguments
for the involvement of known risk  factors seem persuasive, others have interpreted
the data quite differently and are not  convinced that alterations in these factors ex-
plain the improvement in mortality (Stallones, 1980). They suggest that the improve-
ment rate is related to changes in unrecognized risk factors.
  Perhaps the recent unexplained decrease in deaths from atherosclerosis and its
complications is related to immunization against an increasingly greater number of
childhood diseases. Similarly, better socioeconomic conditions may have resulted in
greater resistance to infection and its potential sequela of immunologically mediated
vascular injury. In this connection rheumatic fever,  a sequela of Group  A strepto-
coccal infection and known to predispose to the precocious development of athero-
sclerosis, is now a relatively uncommon disease in this country. The reasons for this
are obscure, but they may well be related to improved nutrition and living condi-
tions, since the incidence of rheumatic fever had already started  to fall precipitously
before the advent of antimicrobial drugs. The decline in cardiovascular mortality
has taken place in an environment in which many of the general environmental fac-
tors have not been purposefully modified while personal ones have probably been
modified considerably. Thus, personal factors appear to be  more important as risks
in cardiovascular mortality in Western industrialized societies.
  Finally, when considering the relationship of environmental factors to  vascular
disease, it must be reiterated that known risk factors can account for only about one-
half of patients who suffer from heart attacks. Clearly other environmental risk fac-
tors must exist. An important effort in the study of cardiovascular disease must be
the identification of these factors and the characterization of mechanisms by which
they induce disease.

ACKNOWLEDGEMENTS
  We gratefully acknowledge the assistance of Carol Ibsen in the preparation of this
manuscript.
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         FRONTIERS IN SHORT-TERM TESTING

                   OF PNEUMOTOXICANTS

          D.B. Menzel, E.D. Smolko, D.E.  Gardner,

                          and J.A.  Graham


INTRODUCTION
  Pulmonary disease is a health concern for some 47  million people in the United
States, representing about 24 percent of the entire population. Interstitial fibrosis
and chronic obstructive lung disease, which includes  emphysema, bronchitis, and
asthma, are the major lung diseases. The exact contribution of environmental tox-
icants to the etiology and incidence of these diseases is  not known, but ozone, nitro-
gen oxides, and sulfur oxides are likely to be  contributing factors. Lung cancer is
mostly due to cigarette smoking, but the interaction between cigarette smoking and
air pollution may also exacerbate lung disease. Because lung diseases are difficult to
treat and are generally irreversible, increasing our understanding of the relationship
of pollutants to the causation of lung disease  is an urgent concern.
  The lung has approximately 40 cell types, all of which theoretically can be dam-
aged by air pollutants; the damage can be acute or chronic. Our focus here is on the
chronic effects of chemicals  and on potential early diagnostic techniques predictive
of human  chronic lung injury.  It will become evident that no validated predictive
short-term tests now exist. However, there are some promising methods that, with
further study, may become adequate early detection systems. Clearly, the develop-
ment of such  methods is a highly meritorious objective.
  Chronic lung diseases have been the subject  of intense study for many years. The
precise mechanisms of the pathogenesis of human lung diseases are unknown in
many instances, but the search for such mechanisms has revealed important corre-
lations between the biochemistry, function, and structure of the entire lung and cer-
tain cell types, particularly alveolar macrophages, fibroblasts, epithelial  cells, and
mucous-secreting cells. The  validity of animals as proper surrogates for man in the
study of lung disease has become increasingly sustained as the basic mechanisms of
lung disease are uncovered.  Because the precise mechanisms of human lung disease
are not known, the selection of a few critical  test parameters for study is not now
possible. Lacking such information, one is constrained to choose techniques that re-
flect some aspect(s) of the chronic disease itself and that are sufficiently sensitive to
detect early disease manifestations.
  Within these limits, what  are the special criteria for short-term tests predictive of
chronic pulmonary disease?
  1.   Specificity:  What is the extent of false positive and false negative determina-
      tions, and to what degree are such determinations acceptable? No widely ap-
The Authors: Daniel B. Menzel and Elaine D. Smolko are on the staff of the Departments of Pharmacology
and Medicine, Duke University Medical Center, Durham, North Carolina. Donald E. Gardner is on the statf
of Northrop Services, Incorporated-Environmental Sciences, Research Triangle Park, North Carolina.
Judy A. Graham is on the staff of the Inhalation Toxicology Division, Health Effects Research Laboratory,
United States Environmental Protection Agency, Research Triangle  Park, North Carolina.
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      plied short-term test is assumed to be 100 percent accurate, but such a test is
      usually used to identify chemicals for further in-depth examination. Thus,
      false positive findings are more acceptable than false negative results. Since
      the objective of the short-term test is to identify chemicals that cause chronic
      diseases of a severely adverse nature, such as emphysema and fibrosis, false
      negative results are undesirable. For ubiquitous pollutants to which large seg-
      ments of the population are exposed, the impact of false negative results could
      be catastrophic, so their acceptable level should be extremely low. A low level
      of false negative results is acceptable for the myriad of remaining, less com-
      mon pollutants to which the population at large is exposed only infrequently,
      since rapid testing will enable the toxicity of more chemicals to be identified.
  2.   Validity: The short-term test must be an accurate predictor of the chronic ef-
      fect of the pollutant in the vast majority of the human population. But first,
      knowledge is needed of the pathogenesis of the human disease process to en-
      sure an outcome analogous to the chronic disease. Careful consideration must
      also be given to unusually susceptible subpopulations. For example, observa-
      tions of young cigarette smokers showed that men responded to the smoke
      differently than women (Becklake and Permutt, 1979). Men were more sus-
      ceptible to the development of chronic obstructive lung disease (COLD), and
      they primarily exhibited an increased resistance in the small airways, whereas
      women principally exhibited an increased resistance in the large airways and a
      greater pulmonary vascular response to smoking. Therefore, a screening test
      developed in only one sex may not be predictive of the disease process in the
      other sex. Genetic differences may be similarly important, but are largely un-
      investigated.
  3.   Cost-effectiveness: With finite resources, priorities must be carefully assessed.
      If screening tests are  of poor specificity and validity, then money would be
      better spent on  chronic  studies with animals  in which the experimental
      outcome is more clearly associated with a human disease. For highly suspect
      chemicals, chronic studies must still be undertaken to validate the short-term
      results.
  With these three broad objectives  in mind, a brief review of some of the most
promising avenues  of research is presented. A short orientation to the special prop-
erties of the respiratory system and the consequent limitations of inhalation toxicol-
ogy is essential to our discussion.
MORPHOLOGY OF THE LUNG IN TOXIC RESPONSES
  The deposition and retention of inhaled gases and aerosols are influenced not only
by the physicochemical properties of the chemicals, but also by the anatomical fea-
tures of the respiratory tract, including the lung volume, the alveolar surface area,
and the structural and spatial relationships of conducting airways into the alveoli
themselves. Extensive  reviews of regional deposition of particles and gases are avail-
able (U.S. Environmental Protection Agency, 1982; Raabe, 1982; National Research
Council, 1977). The distribution of deposited material as a function of time along
with the location of the different cell types within the respiratory tract determine
particular cells or regions of the lung that are at risk. The development of short-term
tests of pulmonary diseases is complicated by regional specializations of the lung and
by the large number of different cell types that exist within these regions. The influ-
ence of  structure on function is so fundamental that most chronic lung diseases are
defined   primarily  by their morphology  and secondarily  by  their  functional
changes. A clear understanding of the morphology of the normal lung is therefore
essential to insights into both the distribution of toxicants and the responses of the
lung to  injury.
                                     176

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The Normal Anatomy of the Mammalian Lung
  Three major regions comprise the respiratory tract: the nasopharyngeal, the
tracheobronchial, and the pulmonary regions (Fig. 1). The nasopharnyx starts at the
anterior nares or buccal-nasal cavity and extends downward to the level of the
larnyx. The nasal passages are lined with a well-perfused mucous epithelium com-
posed of a ciliated columnar epithelium with many scattered mucous glands. The
nasopharnyx in humans and animals filters out large inhaled particles and increases
the relative humidity and temperature of inhaled air. The trachea, bronchi, and
bronchioles conduct air from the nasopharnyx to the alveolar region where gas ex-
change occurs. The airways of the tracheobronchial region are lined with a ciliated
epithelium, which is coated with a thin layer of mucus that is continuously secreted
from goblet cells and mucous-secreting glands. The mucous covering terminates at
the surfactant film covering the alveolar membranes. This mucous covering is con-
stantly propelled upwards to the nasopharnyx, thereby serving as a moving escalator
to transport particles out of the lung. Any particles that are deposited in the alveoli
may  be engulfed by macrophages and  removed from the respiratory tract. The
branching patterns and physical dimensions of the airways influence the deposition
of inhaled particles and the  absorption of inhaled gases.
  The physical dimensions of the airways of human and animal lungs have been de-
scribed by a number of mathematical models (Weibel, 1963; Davies, 1961). In hu-
mans, the airways tend to be equal and bifurcating, decreasing in diameter as they
divide. As bifurcation increases, the cross-sectional area increases. Since the cross-
sectional area increases as the gas diffuses downwards in the lung, a second phenom-
enon of radial diffusion also occurs. Gases tend to be diluted by this combination of
anatomical features, independent of mixing with inspired gases. While human and
animal lungs differ, the anatomies of both respiratory systems are sufficiently similar
for equivalent divisions to be made. Generally, these divisions, or so-called gener-
ations, are based upon the number of times that the major airway divides to reach a
particular  anatomical site.
  The acinus is the basic functional unit of the mammalian lung and is the primary
location of gas exchange between the environment and the blood. The acinus con-
sists of structures, or acini, starting at the respiratory bronchiole. About  200,000
acini exist in the human lung, including three or four generations of respiratory
bronchioles, several generations of alveolar ducts and alveolar sacs, and hundreds of
alveoli and associated blood vessels, lymphatic tissues, supportive tissues, and nerve
endings. Quantitative anatomical information on the lung is available, including
estimates of airway tube numbers, diameters and lengths; alveolar numbers and di-
ameters; and surface areas and mean thicknesses for the air-to-blood barrier (Weibel,
1963; Kliment, 1973). This information is important to the quantitative estimation
of dose in humans on the basis of dose received by animals.  The dose-response rela-
tionship for various regions within the lung is highly important to the eventual devel-
opment of short-term tests.
  Because structure is so intimately associated with function in the lung and, in turn,
with the deposition and the dose of toxicants reaching specific lung segments, short-
term tests need to be specialized to regions of the lung. Chronic diseases in humans
are often localized  in specific segments  rather than existing throughout the lung.
Bronchitis, for example, is confined to those airways containing ciliated and mucous
secretory glands. Emphysema, on the other hand, is confined to the alveolar or gas
exchange regions. The anatomical separation of diseases can be analyzed in more de-
tail by using the pulmonary function tests and morphological examinations that are
discussed below.
  Tests that are designed to sample cells from  the various regions of the lung have
been  developed.  Fiberoptic bronchoscopes allow biopsy procedures to sample
major conducting airways in humans, although such devices are too large to extend
                                    177

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  Upper respiratory tract
 Anterior nares
                                                       rachea
      Lower   vxlxjxixlxxivxix;:;:
    respiratory •|:jX:X;:;Xx:;::Xjx;:;X;:
      tract     :*::x:x::::::::::::*:*
Pulmonary
                                                                  Bronchus
Aveoli
 Figure "\.  Three major regions of the respiratory tract. The upper respiratory tract is
           known as the nasopharyngeal region. The lower respiratory tract is
           divided into the conducting airways (tracheobronchial) and the gas
           exchange (pulmonary) regions.
beyond the major bronchi of animals used routinely in toxicology. An alternative
means of sampling is lung lavage (Section VIII). This procedure samples all of the
cells and secretions that can be removed by mild agitation with solutions in the lung.
One cell type readily recovered by lavage is the pulmonary macrophage, which nor-
mally resides within the alveoli. The macrophage is motile and arises from the bone
marrow; in the lung, macrophages serve an important function by collecting soluble
and insoluble material from the alveoli and transporting the material up to the junc-
tion with the mucociliary escalator. There, the dead or dying macrophages are trans-
ported slowly out of the lung on the blanket of mucus covering the conducting air-
ways. Another important function is to engulf and kill bacteria that otherwise might
cause lung infections. The death of macrophages is not without consequences in the
lung because they contain powerful degrading enzymes  which,  when released, act
upon other cells to produce a number of pathophysiological conditions. When pur-
ified, concentrated, and instilled into the lung, these degrading enzymes can produce
experimental emphysema and bronchitis.
  Many of the common chronic pulmonary diseases of humans result in changes in
the numbers or anatomical relationships of cells within the lung. Interstitial fibro-
sis results from a thickening of the distance between the gas exchange region and the
pulmonary capillary bed. Since the lung is designed to function with an exquisite
balance between gas exchange and blood flow, changes in either condition result in
inappropriate gas exchange and in major compromises in the functional capacity of
the lung.

The Anatomical Pathology
  Although  humans suffer from a large number of pulmonary diseases, chemicals
have been im plicated in the etiology of only a few of the more common diseases. One
                                    178

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large class is chronic obstructive lung disease (COLD), which includes primarily
bronchitis and emphysema. Bronchogenic cancer is also associated with cigarette
smoking and, in animals, with experimental exposure to chemical carcinogens.
Bronchitis and asthma may be exacerbated by exposure to chemicals, and interstitial
fibrosis may be initiated or aggravated by chemical exposure. Pulmonary fibrosis is
associated with fibrous mineral exposure, and mesothelioma, a tumor of the pleura,
is particularly associated with asbestos exposure in both humans and animals. Pneu-
moconiosis  is  associated  with exposure  to  mineral  dusts  in humans  and
with formaldehyde in animals. Some examples of these diseases, as well as normal
tissue, are shown in Fig. 2.
  COLD is particularly important in terms of long-term exposure to air pollutants
and can be induced in rodents by both ozone and nitrogen dioxide at concentrations
greater than those occurring in urban air (Freeman et al., 1972, 1974). The charac-
teristics of obstructive and restrictive lung diseases have been reviewed by West
(1977) and Thurlbeck (1979). COLD is of two major types—emphysema and chronic
bronchitis—but the two types are generally classed together because small airway ob-
struction (<2 mm), leading to gas trapping which may make expiration difficult in
advanced cases, is  a common hallmark. In  some patients, both emphysema and
chronic bronchitis are present.
  The classical definition of emphysema is the destruction of the alveoli distal to the
terminal bronchiole, with distension of their walls. These anatomical changes may
be associated with  measurable changes in pulmonary function.  Pulmonary func-
tion tests are widely used in humans  as a  diagnostic aid. Although anatomical
changes have been used more extensively than pulmonary function tests to estimate
the progression of pathophysiology, there is an insufficient number of detailed quan-
titative analyses in  animals that relate anatomical changes to pulmonary function
measurements. The issue, then, for a chemically induced chronic disease is which is
the more sensitive and practical technique of potential application to a short-term
test. Recent advances in measuring pulmonary physiology in small animals (O'Neil
and Raub, 1982) suggest that this issue could be evaluated shortly. Minor to extreme
levels of chronic diseases, such as emphysema and fibrosis, can be chemically in-
duced with elastase,  bleomycin, or silica. At various times during pathogenesis,
physiologic,  morphologic, and morphometric measurements could be made and
compared. A few such studies have already been performed (Raub et al., 1982a;
Snider and Sherter, 1977), but the doses of elastase used have caused both functional
and structural alterations. A further complication is that human emphysema pres-
ents as several different types (centrilobular, panlobular, panseptal, and bullous),
depending on the  site and distribution of the lesion. However, emphysema  in
animals has some differences from emphysema in man (Port etal., 1977). In another
recent study, neonatal rats were exposed intermittently for 6 weeks to 490 /j.g of
ozone perm3 (0.25 ppm) (Raub etal., 1982b;Raub, 1982; Barry etal., 1982a,b).  Vital
capacity and total  lung capacity were increased, while other pulmonary function
parameters were not altered. Qualitative  morphology detected no damage to the
gaseous exchange region of the lung, although sophisticated morphometric analyses
showed a number of alterations in the alveolar epithelium. At present, data are insuf-
ficient to state confidently whether structural or functional changes occur first, thus
making np single technique preferred for short-term testing. When such information
is available,  the level of difficulty of the test procedure will likely be a significant
determinant of the choice of short-term test (i.e., functional or structural).
  Chronic bronchitis is clinically defined as excessive bronchial mucus production,
causing  excessive expectoration of sputum. Histological examination of human
bronchitic airways  shows hypertrophy of mucous glands in the large bronchi and
chronic  inflammatory changes, including cellular infiltration, edema, and some
increase in smooth  muscles in the small airways. In COLD, the increased resistance
to airflow can be caused by conditions that affect the diameter of the functional air-
                                    179

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Figure 2.  Some common lung diseases in man The authors are grateful to Phillip C.
          Pratt, M D., Department of Pathology, Duke University Medical Center, for
          providing the photomicrographs.
          (a)  Interstitial fibrosis. Note the  honeycombed appearance of the lung.
                                     180

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(b)  Primary carcinoma. The white mass in the upper left portion of the lung has grown
    around the major airways and vessels and has even reached the lower margin
    of the aorta.
                                   181

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                                                                       «^*;"'«   »-*:.-• "*  7-is^'-~'#*5l£.-:
                                                                       •«^L:-:,I*.StJ•:-".'  •  S&»R>4ifc^S?
  
-------
(d)  The wall of the bronchus of a chronic bronchitic patient. The mucous glands are greatly enlarged, and the fine boundary of cilia and mucous
    cells has been lost.  (4Ox original).                                                      '                      .  >               u  ,.

-------
way lumen. In bronchitis, excessive mucus secretions or thickening of the airway
wall by edema or muscular hypertrophy effectively narrow the lumen. In emphyse-
ma, the loss of alveolar walls and parenchyma, which normally exert a radial trac-
tion on the airways, allows the airway to narrow.
  In restrictive pulmonary diseases, the ability to expand the lung is decreased,
making inflation of the lung more difficult; this can be caused by alterations in lung
parenchyma, pleura, chest wall, or neuromuscular function. Pulmonary fibrosis is
the major disease group in this category. In fibrosis, the interstitium, i.e., the tissue
between the alveolar epithelium and the capillary endothelium, becomes thickened.
The process begins with an inflammatory infiltration of lymphocytes and plasma
cells, followed by an increase in the number of fibroblasts that produce collagen. In
its most severe form, the alveolar structure is destroyed, and excessive deposition of
collagen or scarring occurs. In fibrosis, the airway diameter is enlarged due to exces-
sive radial traction, as opposed to a narrowing in COLD.

PULMONARY EXPOSURE METHODS
  Since the respiratory tract is the main portal of entry and one of the first surfaces
to come in contact with airborne chemicals, toxicological studies must be designed
and conducted to mimic as closely as possible this natural route of exposure. The test
substance, therefore, must be generated in clean air in a physical and chemical state
that most nearly duplicates that found in the environment. The choice of technique
for conducting  inhalation studies with experimental animals must take into con-
sideration the duration  of the exposure, the chemical and physical state of the test
substance, the cost and availability of the exposure facility, and the need for the pre-
vention of exposure through nonrespiratory pathways, such as skin, eyes, or food. A
number of excellent reviews describe different exposure systems (i.e., whole-body,
head-only, nose- or mouth-only, intratracheal instillation, and isolated ventilated
perfused  lung),  operational procedures, generation of test atmospheres, and sam-
pling techniques (Willeke,  1980; Phalen, 1976; Drew, 1978; Rampy, 1981; Jacoby
and Barthold, 1981). Depending on the objective of the experiment, animals are ex-
posed completely or partially to the test atmosphere. Whole-body exposures are the
commonest and are reviewed in detail by Hinners et al. (1966), Leach (1958), and
Drew and Laskin (1973). Exposures restricted to the head (Thomas and Lie,  1963;
Mauderly et al., 1971; Lippman,  1980) or to the nose or mouth (Blair et al.,  1969;
Raabe, 1973; Braretal., 1975) have the advantage of reducing the simultaneous in-
gestion of material deposited on the body. Test material can also be instilled via the
trachea (Hatch etal., 1981; Phalenand Morrow,  1973; Auerbachetal., 1970; Bianco
et al.,  1974; Gardner etal., 1972). Effects of toxicants on the lung can be measured ex
vivo in perfused lung preparations (Niemeier,  1976; Anderson and Eling,  1976;
Charles and Menzel, 1979; O'Neil and Tierney, 1974; Young 1976) from exposed or
normal animals. The technology of each method is complex.

FUNCTION  TESTS FOR CHRONIC PULMONARY DISEASE
  A number of pulmonary function tests, which can be used on a routine basis with
small  laboratory animals for general toxicological testing,  have been developed.
Several excellent books, reviews, and monographs describe the principles of respira-
tory physiology and the specific techniques for making such measurements (O'Neil
and Raub, 1982; Horvath, 1977; West, 1977; Slonim and Hamilton, 1976; Becklake
and Permutt, 1979). This section will briefly discuss some of the measurements that
were specifically developed to be similar to tests that are being used in humans to
diagnose lung disease.
  The maximum volume of gas that the lung can contain is referred to as the total
lung capacity (TLC). The TLC can be conveniently subdivided into four indepen-
dent volumes and four lung compartments or capacities that can be measured by
pulmonary function tests. Fig. 3  shows these subdivisions.


                                     184

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£
u
13
z
                                         *   I    J     J
                                    INSPIRATORY RESERVE VOLUME
    RESTING TIDAL
    VOLUME
                FUNCTIONAL
               ,  RESIDUAL .
                 CAPACITY
                                             t-A-A-
                                                      TIDAL VOLUME
                                                      (ANY LEVEL OF
                                                        ACTIVITY)
                                      EXPIRATORY RESERVE VOLUME
                                           v    t    r     t
t
                                                 RESIDUAL
                                                  VOLUME
                                                    1
           SPECIAL DIVISIONS FOR
        PULMONARY FUNCTION TESTS
                                          PRIMARY SUBDIVISIONS
                                             OF LUNG VOLUME
                                                                  -O
                                                                                       	J
   Figure 3.  Spirometric measurements of lung volumes. The resting tidal volume is the volume of air
            inspired and expired during normal breathing. By inspiring and expiring maximal amounts of air,
            the vital capacity is measured. Other divisions are apparent and are described in pulmonary
            function tests (from West, 1977; Slonim, 1976).

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  Another category of static mechanical measurements made in laboratory animals
is the volume-pressure relationship of the respiratory system. Compliance is a mea-
sure of the distensibility of the chest wall and lungs, i.e., the ease with which lung vol-
ume is changed. Static compliance is the slope of a static volume pressure curve at a
given point or the slope of a linear approximation to a nearly straight portion of such
a curve in the tidal volume range. Compliance of the respiratory system (Crs) in-
cludes that of the chest wall (Cw) and the lung (Cl); assessment of both Cland Cwis
important when examining the elastic recoil of the system. Cw values vary with the
size and species of the animal, but Cl values are determined largely by the elastic
recoil of the lung. The elastic recoil is the sum of the recoil forces of the lung tissues in
the alveolar walls and smallest airways, and the surface tension forces of the liquid-
air interface lining in the gas exchange area of the lung.  Diseases that change the
elastic  properties of the lung or the surface tension within the lung can affect lung
compliance.  Certain diseases decrease lung compliance, and the lungs become stiff;
other diseases increase compliance, with a loss of normal elasticity.
  In contrast to the static measurements of lung function, dynamic measurements
describe and quantify the mechanics of the respiratory system under conditions of
respiratory gas flow. These include measurements of pulmonary and airway resist-
ance, flow-volume relationships, and frequency dependence of compliance and re-
sistance. Measuring dynamic pulmonary mechanics in small mammals involves a
number of difficulties: (1) the high frequency at which the animal breathes; (2) the
small dimensions of the airways; (3) the relatively large dead space of conventional
measuring equipment; and (4) the relatively poor frequency-response characteristic
of equipment measuring flow, volume, and pressure in the respiratory system (Koo
et al., 1976;  Sinnett, et al.,  1981).
  Minute  ventilation is calculated as the product of tidal volume (TV) and respir-
atory frequency (f).  Dead space ventilation (VD) includes  both the anatomical and
the physiological dead space.  In terms of gas exchange, this dead space is wasted
ventilation and increases with certain lung diseases. Anatomical dead space is in-
creased with exercise, age, or emphysema, but is decreased in asthmatics as a result
of airway narrowing. In patients with lung disease, the physiological dead space may
increase because of an inequality in blood flow and ventilation within the lung. Pul-
monary resistance (Ri_), a measurement of the frictional resistance in the airway
(RAW) and the tissue viscocity resistance (Ry), is relatively low in the healthy animal.
In cases where alterations are found in the cross-sectional area of the large airways
(bronchoconstriction),  RT can change  substantially.  In  laboratory  animals,
RAW can be  substantially altered following exposure to chemicals.
  A very useful test of pulmonary function is the measurement  of the maximal
volume of air that can be exhaled forcefully (FVC) after a maximal inspiration. The
FEVi is the volume  of air that can  be forcibly exhaled during the first second of ex-
piration; this measurement has been regarded as one of the most useful overall spiro-
metric  measurements in humans. The measurement of maximum respiratory flow at
different volumes can also be used to examine the relative contribution of small and
large airways to pulmonary resistance in injured lungs. When airway disease or in-
jury is present, or if lung recoil is reduced, flow at any volume can be reduced, com-
pared to that of the normal lung. Mauderly et al. (1979) found that rats with emphy-
sema have lower flows at all volumes and lower initial peak flows than control ani-
mals.
  A number of techniques are used to detect maldistribution of ventilation following
mild injury to the small airways by inhaled toxicants (O'Neil and Raub, 1982; Drill
and Thomas, 1980). Measurement of the impedance to gas flow in and out of the
lung may be the most sensitive  functional test for mild, small-airway lesions. The
single-breath nitrogen washout test gives an index of the gas distribution in the lung
and has been shown to be sensitive for detecting airway disease (O'Neil and Raub,
1982; Drill and Thomas, 1980).  This test has not been widely used in small animal
                                    186

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toxicology. Multiple breath nitrogen washout has also been used. Raub et al. (1982a)
observed that hamsters that have experimental emphysema show marked changes in
the slope of the nitrogen washout and in the breath index.
  The lung's primary function is the exchange of oxygen and carbon dioxide, which
move between air and blood by simple diffusion. The transfer of respiratory gases
between the alveolar spaces and the pulmonary capillary blood depends on the level
of alveolar ventilation, the evenness of ventilation, the volume and distribution of
pulmonary blood flow, and the distance needed for diffusion between the alveolar
wall and the pulmonary capillary blood. Abnormal gas exchange can be estimated
by measuring the rate at which a test gas, such as carbon monoxide, is absorbed into
the blood from the airspaces. Such measurements  (diffusion capacity for carbon
monoxide, DLCO) can be accomplished  in small animals  and appear sensitive to
subtle changes  in pulmonary structures  (Takezawa et al., 1980).
  Measurement of arterial blood-gas partial pressures can give a general overview of
the respiratory gas exchange and total pulmonary function. Pulmonary damage re-
sults in an abnormally low partial pressure or concentration of oxygen and, in some
cases, an abnormally high concentration and partial pressure of carbon dioxide.
Low arterial oxygen partial pressure (hypoxemia) can result from: (1) hypoventila-
tion, (2) low-inspiratory oxygen, (3) pulmonary alveolocapillary diffusion impair-
ment, (4) mismatching of ventilation and perfusion, and (5) right-to-left pulmonary
vascular shunt. The  measurement of blood gases can be useful in evaluating both
acute and chronic conditions associated with pulmonary dysfunction. Acid-base im-
balances, increases or decreases in the partial pressure of carbon dioxide or oxygen,
and differences in the oxygen partial pressure between the alveolus and the arteri-
alized blood have all been used to compare the blood gases in normal and emphy-
semic laboratory animals  (Lucey et al.,  1978).

PULMONARY FUNCTION TESTS FOR  CHRONIC OBSTRUCTIVE
AND RESTRICTIVE  LUNG DISEASES
  The most  common and severe obstructive (emphysema and chronic bronchitis)
and restrictive (fibrosis) lung diseases are associated  with functional as well as struc-
tural alterations (West, 1977; Thurlbeck, 1979). Rapid tests predictive of chronic
lung disease in humans have been sought (Becklake and Permutt, 1979), and the
ones most described have  been pulmonary function measurements. A patient with
COLD will not be able to exhale air forcefully after maximal inhalation as quickly as
a normal person; i.e., FEVi is reduced, and the total volume (FVC) expelled is less
(Fig. 4). However, considerable morphological changes occur before FEVi is altered
(Thurlbeck,  1979). In patients with restrictive lung disease, FVC is also reduced, but
a larger percentage of air is exhaled  in the first second compared to patients with
COLD. Maximum midexpiratory flow rate (MMFR) is also altered in these disease
states. In COLD, MMFR is below normal;  in restrictive disease, MMFR can be
above normal. Flow-volume curves (Fig.  5) and lung volumes (Fig. 6) are also altered
characteristically in restrictive and obstructive diseases. The distribution of ventil-
ation within the lung is altered as measured by nitrogen washout techniques. In pa-
tients with COLD, small airways are enlarged, causing a lower concentration of in-
spired nitrogen in more distal airways; alveolar units can receive inspired gas from
adjacent units,  which can then be detected. Closing volumes can be a sensitive mea-
surement of alveolar dysfunction in patients with  COLD. In apparently healthy
smokers with normal vital capacity (VC), closing volumes can be increased. Changes
in closing volume may be useful in the early identification of people likely to develop
COLD, but its use in animals is highly speculative at present. Inflammatory changes,
hyperplasia of  mucous glands, and excess mucus secretions of chronic bronchitis
narrow airways and  can cause a premature airway closure. Destruction of alveolar
walls and the  resulting loss of radial  traction on small airways  can also cause
increased closing volumes.
                                    187

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        A. NORMAL
                                B. OBSTRUCTIVE
                                                           C. RESTRICTIVE
          FEV = 4.0
          FVC = 5.0
            % = 80
        MMFR = 3.5 I/sec
          FEV = 1.3
          FVC = 3.1
            % = 42
       MMFR = 1.4 I/sec
  FEV = 2.8
  FVC = 3.1
    % = 90
MMFR = 3.7 I/sec
 Figure 4.  Examples of forced expiratory volume (FEV), forced vital capacity (FVC),
           and maximal midexpiratory flow rates (MMFR) in normal individuals
           and patients with chronic obstructive and restrictive lung diseases
           during forced expiration into a spirometer. The percent is the ratio of
           FEV  to FVC (from West, 1977).
                          8 -i
                  FLOW
                  RATE
                  (I/sec)
                          6 -
4 -
                          2 -
                                             Normal
                                8642

                                   LUNG VOLUME (I)
                             i
                             0
  Figure 5.  Flow-volume curves illustrating the different patterns existing in
            patients with obstructive and restrictive diseases (from West, 1977).
  Other techniques like the single-breath nitrogen test, DUO, frequency depend-
ence of compliance, closing volume, and difference in flow rates (MMFR 50 and 25
while breathing air and a helium-oxygen mixture) have promise, but experience is
still sparse due to the recent introduction of these tests (Becklake and Permutt, 1979;
Tattersall et al., 1978; Ranga and Kleinerman, 1978).
  Pulmonary function testing in animals is based on the same principles as in hu-
mans and has been reviewed by O'Neil and Raub (1982) and by Leith( 1976).  Most of
these tests assume a commonality of physiological principles, and this is generally
true. For example, functional changes observed in elastase-induced emphysema in
hamsters and rats (Raub et al., 1982a; Mauderly et al.,  1979; Radinsky and Snider,
1978) are similar to those occurring in humans. Nevertheless, there are differences,
and one should exercise caution in automatically assuming such similarities (O'Neil
and  Raub, 1982).
                                     188

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                                                     LUNG VOLUMES
                                                                                         OBSTRUCTIVE DISEASE
                   NORMAL
                                                                                    1C
                                                                                        IRV
                                                                                              TV
                                                                                           ERV
                                                                                        RV
                                                                                                             vc
                                                                                                             RV
                                                                                                                   o
Figure 6.  Comparison of normal lung volumes to those observed in restrictive and obstructive disease states. Abbreviations are as follows:
          TLC, total lung capacity; 1C, inspiratory capacity; FRC, functional residual capacity; VC, vital capacity; TV, tidal volume; IRV,
          inspiratory reserve volume; ERV, expiratory reserve volume; RV, residual volume.

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  The chronic effects of air pollutants are illustrative of the difficulties likely to
occur when attempting to develop and use short-term tests. These difficulties arise
out of the complexity of environmentally induced pulmonary disease. An extensive
chronic study of air pollutant toxicology has been summarized by Stara et al. (1980).
Beagle dogs were exposed for 14 hours per day for 68 months to air or to one of seven
treatments with raw or photochemically reacted auto  exhaust, oxides of sulfur or
nitrogen, or their combinations. After exposure ceased, the dogs were allowed to re-
main in clean air for 32 to 36 months. Pulmonary function was studied after 18, 36,
and 61 months of exposure and at 2 years postexposure; pulmonary structure was
examined 32 to 36 months postexposure. Lung morphology was studied using light
microscopy, scanning electron microscopy (SEM), and transmission electron micro-
scopy (TEM) (Hyde et al.,  1978). The observed structural changes were interpreted
as being analogous to an incipient stage of human proximal  centrilobular
emphysema.  Some  pulmonary function changes, which were  correlated to  the
structural changes in the postexposure period, also occurred.
  Could the morphological changes have been observed earlier by pulmonary func-
tion tests? Compared to controls, no significant effects on pulmonary function were
observed afer 18 or 36 months of exposure (Vaughanetal., 1969; Lewis etal., 1974).
However, in an analysis of the incidence of dogs having abnormal values based on
clinical criteria,  the oxides  of nitrogen  group had a lower Duo/TLC after 36
months of exposure. After 61 months of exposure, an analysis of incidence of dogs
with normal values showed that the only major change was in the oxides of nitrogen
group, which  had a decrease in peak expiratory flow rate (Lewis et al., 1974). A wide
battery of pulmonary function tests was applied, but sensitive tests of small airway
function in animals  were not available at that time. About 2 years after exposure
ceased, pulmonary function was examined again by others and included some newer
techniques, such  as frequency dependence of compliance, that should be helpful in
detecting changes in small airways (Gillespie, 1980). Additional changes were ob-
served in the  oxides of sulfur or nitrogen groups, including decreased Duo/TLC,
decreased chest wall compliance,  increased TLC, and  greater changes in dynamic
compliance with  increasing frequency. When the results for the immediate and 2-
year postexposure studies  were compared, residual volume, vital capacity,  func-
tional residual capacity, inspiratory capacity, and total lung capacity of the experi-
mental dogs increased compared  to the controls. Thus, pulmonary function con-
tinued to deteriorate after exposure ceased. Since the functional changes in all  ex-
posure groups correlated well with structural changes, it can be hypothesized that
structural changes also progressed during the postexposure period.
  Transient changes in the breathing mechanics of experimental animals have also
been used as a short-term test of the acute effects of pulmonary irritants. Two sys-
tems have been developed for general use. Amdurand Mead (1955,1958) developed
a method to measure the pulmonary mechanics of the unanesthetized guinea pig.
The method has been evolved into a highly sophisticated procedure which monitors
pulmonary function. Most irritant gases, such as sulfur dioxide, produce an increased
resistance to air flow. Dose-response curves can be developed and related quantita-
tively to exposures. Generally, the Amdur method using unanesthetized guinea pigs
is more  sensitive to pulmonary irritants than other methods using anesthetized
animals  or animals other than guinea pigs. The general correlation between guinea
pig and human responses is good, and the method has been used extensively in air
pollution research.
  Alarie and  coworkers (1973) developed a method using mice confined in a modi-
fied whole-body plethysmograph. They found good correlations between a number
of industrial chemicals that are irritants for man and changes in respiratory rate in
mice. Alarie (1973) presented an extensive review of the physiological basis for the
assay and compared the relative potency of these irritants in the mouse assay with
reports from  the workplace.
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  At present, pulmonary function measurements are not more predictive of injury
than morphological changes. More research including state-of-the-art techniques
for pulmonary physiology and morphometry is needed before this issue can be re-
solved. To date, such comparisons have indicated that structure is measurably altered
before function (Raub et al., 1982b,c; Barry et al., 1982a,b). However, the morpho-
metric techniques used in these studies are too sophisticated for use in routine test-
ing, further  complicating the issue. At issue, also,  is the relevancy of transient
changes in pulmonary function measured by the Mead and Amdur, and Alarie tech-
niques. No correlation has been attempted between these techniques and the chronic
effects in animals or occupational disease in man. Alarie (1973) addresses this issue
in a more detailed discussion of his technique.
EARLY DETECTION OF CHRONIC BRONCHITIS
  A research group (Lamb and Reid, 1968; Reid, 1970; Reid and Jones, 1980) devel-
oped an animal model of human chronic bronchitis in rats. The rat was chosen be-
cause, unlike the hamster, mouse, rabbit, and guinea pig, it has mucous glands that
in chronic bronchitis increase in size, number, and activity,  and spread  to deeper
parts of the lung. Characteristics of chronic bronchitis were produced by using sulfur
dioxide or cigarette smoke as the inducing agent, and the size and number of mucous
glands in different regions of the lung were measured microscopically. The magni-
tude of these changes was dependent on the dose of sulfur  dioxide and cigarette
smoke. Four types of acid glycoproteins are produced by mucous glands and can be
measured biochemically. The proportion of cells producing a given type of  glycopro-
tein changes in chronic bronchitis. This rat model has been used to study the toxicity
of sulfur dioxide and cigarette smoke, but not with a view toward its potential value
as an early indicator of bronchitis-producing agents. A 6-week exposure to sulfur
dioxide was indicative of progressive changes in chronic bronchitis. Given the time-
and dose-dependency of the alterations in mucous glands, more investigations
would  be useful, particularly ones  that correlate such biochemical changes with
pulmonary function alterations.
DETECTION OF LUNG FIBROSIS

  Many industrial processes produce dusts that could be harmful if inhaled. The
pneumoconioses, parenchymal lung diseases  caused by inorganic dust inhalation,
are a class of diseases characterized by massive fibrosis in their advanced states. Per-
haps the most studied form is silicosis, particularly with respect to potential early in-
dicators of chronic disease (Reiser and Last, 1979; Ziskind et al., 1976). Silicosis is a
general  class  of diseases produced  from the inhalation of dusts predominantly
associated with mining, mineral refining and processing, but which are also found in
a wide variety of urban situations. With the recognition that pulmonary disease can
arise from the inhalation of such dusts, man-made substitutes have been sought, and
alterations have been made in industrial processes to reduce the exposure to, and the
toxicity of,  dusts.  Because many different types  of dusts can be generated, a rapid
screen of these materials for their biological activity would provide producer, con-
sumer, and regulator with an important tool.
  Silicosis results from chronic exposure to free crystalline silica (Ziskind et al.,
1976). The occupational form  most studied arises from the inhalation of particles
less than 10 jum in diameter, with the assumption that smaller particles of less than 3
/um are responsible for the  lesions. Environmental silicosis, if it exists,  may result


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from the inhalation of free silica particles of 2.5 to 15 jum mass median aerodynamic
diameter.  Three forms of silicosis have been characterized in humans: Chronic,
caused by moderate exposure levels for 20 to 40 years; accelerated, caused by higher
exposure levels for 5 to 15 years; and diffuse, from a still higher level of exposure for
less than 5 years (Ziskind et al., 1976). The years given are the time to appearance of
silicotic nodules on chest roentgenograms. The  classical silicotic nodules can be
produced  in  rats after a 1-year exposure. In the  chronic  form in humans, lung
nodules have concentric rings of connective tissue around a zone of connective tissue
with interspersed free silica particles 1 to 2 /^m in  diameter. Massive fibrotic lesions
composed of nodules of fibrosis with destroyed blood vessels and bronchi occur in
the  upper  lobes  of  the   lung.  Complications  with   rheumatoid  arthritis,
emphysematous  changes, and tuberculosis can also occur. Accelerated silicosis has
similar pathological changes, but progression is  more rapid, and massive fibrosis
occurs more frequently. Acute silicosis is infrequently observed, and nodules are not
grossly observable. Lung consolidation does occur, as do edema and inflammatory
reactions.
  Diagnosis  of human silicosis  is based upon exposure history and roentgeno-
graphic evidence. Although adequate for clinical diagnosis, such procedures are far
too insensitive to consider for short-term tests,  especially in rodents. Pulmonary
function has been measured in human silicosis, but because of the complexity of the
disease, which is  often accompanied by  infection, bronchitis,  emphysema, and
smoking, changes unique to silicosis have not been observed (Ziskind et al., 1976). In
controlled animal studies, these confounding variables are absent,  but adequate pul-
monary function tests have not yet been reported. In a review of animal models for
silicosis, Reiser and Last (1979) point out that factors such  as the presence of lung
pathogens and the route of administration (intratracheal versus inhalation) can have
a great  influence on whether the animal model  mimics the human disease (Holt,
1974; Zaidi, 1969).
  Until there is an understanding of the mechanism(s) of the pathogenesis of sili-
cosis, the development of short-term tests will be impeded. Seven major events in the
pathogenesis of silicosis have been described (Ziskind et al.,  1976): (1) phagocytosis
of the particles by alveolar macrophages; (2) death of the macrophages that have in-
gested the silica  particles; (3) release of the intracellular contents of the injured and
dying macrophages, including silica particles and enzymes; (4) phagocytosis of re-
leased silica by other macrophages, followed by the death of these macrophages; (5)
accumulation of silica in other lung cells; (6) increased production of collagen fibers
in affected areas; and (7) hyalinization. The cytotoxicity of silica is an essential com-
ponent of these processes, and, centering on this, in vitro models have been proposed
and are described in the following section. Collagen production,  being the integral
part of fibrosis, has also been studied and is discussed in the  section on metabolism.
  Autoimmune phenomena have been observed in the development of silicosis and
have been reviewed by Ziskind et al. (1976) and by Reiser and Last (1979). Individ-
uals with silicosis have an increased amount of serum autoantibodies, i.e., rheuma-
toid factor, antinuclear antibody and gamma globulins, as well as an increased pre-
valence of autoimmune disease.  Silicotic lesions contain antibody-producing cells
and immunoglobulins. Animals injected  with silica and antigens have far higher
specific antibody liters than animals injected with antigen alone. This adjutant effect
does not occur with relatively nontoxic dusts like  kaolin and coal.  Although specific
lung autoantibodies have not been observed in humans with silicosis, autoantibodies
that reacted  primarily with collagen have been found in patients with various
chronic lung diseases. In vitro studies have shown that lung connective tissue anti-
bodies can stimulate alveolar macrophages to secrete a factor that stimulates col-
lagen production in fibroblasts. When properly activated, T-lymphocytesand other
cell types also produce these factors. The immunological involvement is interesting
because simple blood tests may have potential as screening methods.
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 THE USE OF PULMONARY LAVAGE TO DETECT LUNG
 DAMAGE

  The lungs of humans and animals can be washed out or lavaged with various solu-
tions and the resultant fluid analyzed for alterations in fluid and cellular compo-
nents. Presently, this technique is not sufficiently validated as a short-term test for
chronic disease in animals, but several research groups (Hunninghake et al., 1979;
Henderson, 1982), especially Henderson and coworkers, are conducting investiga-
tions in this promising area.
  Several protein constituents of normal human lavage fluid are listed in Table 1
(Hunninghake et al., 1979). The proteases and antiproteases may prevent the devel-
opment of emphysema and other destructive lung diseases. Table 2 depicts several
parameters in lavage fluid that may be indicators of lung damage (Henderson, 1982).
Measured changes in these parameters at any arbitrary point during exposure do not
imply that chronic disease will be the outcome. For example, elevated protein in lung
lavage, which is commonly assumed to reflect pulmonary edema, is a classical acute
response to nitrogen dioxide and ozone exposure (Selgrade et al., 1981; Hu et al.,
1982). This lavage technique has not been applied after chronic exposures to nitro-
gen dioxide and ozone, and the magnitude of protein exudation has not yet been cor-
related with the extent of chronic disease in either case.
  Lavage analyses of patients with chronic lung diseases, such as alveolar protein-
osis or cystic fibrosis, have been correlated with their respective disease states. Al-
though no environmental component has been implicated in these human diseases, a
promising diagnostic role for pulmonary lavage fluid is developing.  For example,
Kubich et al. (1980) are pursuing immunological methods to detect developing em-
physema. Studies by Henderson et al. (1978) and Pickrell et al. (1981)  of the balance
between proteolytic and antiproteolytic activity in lavage fluid may also lead to early
tests for emphysema. Analogous experiments in animals could provide a screen for
early lung injury likely to produce COLD.
  Analysis of the cellular content of lavage fluid is also important. In both humans
(Hunninghake et al., 1979) and animals (Gardner,  1982a), the predominant (more
than 90 percent) cell type is the alveolar macrophage. The remaining cell types are
lymphocytes,  polymorphonuclear leukocytes, eosinophils, and basophils. Changes
in the numbers and functions of these cells can reflect lung damage and be part of the
pathogenesis  of  disease. Table  3  shows most  of  the functions of alveolar
macrophages  in humans. The alveolar macrophages  of animals perform similar
functions (Brain et  al., 1977).  Probably their most important activity is to phago-
cytize and kill microbes, thereby maintaining the sterility of the lung. Microbiocidal
activity in macrophages is inhibited by acute pollutant exposure  (Gardner and
Graham, 1977). Other major roles for macrophages are as  effector and accessory
cells in inflammatory and immune responses. Their involvement in emphysema is
proposed by virtue  of their ai-antiprotease content (Hunninghake et al.,  1979).
  Human lavage studies of smokers show an increased number of inflammatory and
immune effector cells, and morphological studies have located these cell accumu-
lations at sites of lung destruction (Hunninghake etal., 1979). Smokers have an un-
usually high  proportion  of polymorphonuclear leukocytes in the  lavage  fluid.
Smokers' alveolar macrophages (also present in larger numbers) are activated, and,
in contrast to macrophages from nonsmokers, they secrete elastase that stimulates
the influx of polymorphonuclear leukocytes and the secretion of proteases. In ad-
dition, antiproteases, although found in normal quantity, do not function as well in
smokers as  in nonsmokers.

EXPOSURE ASSAYS OF LUNG CELLS IN VITRO
  Exposure of cells in vitro provides the most easily performed short-term test. Cell
lines of Type II alveolar cells and lung fibroblasts are readily available; primary cul-


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Table 1.   Inflammatory and Immune Related Protein Constituents of Nor-
           mal Human Alveolar Epithelial Fluid*
        Components
    Status in Normal Lung
 Immunoglobulins
  IgG
  IgM
  IgA
    Monomeric
    Dimeric
    Free secretory piece
  igE
  igD
 Complement
  Classical pathway
  Alternate pathway
  Common pathway
 Antiproteases
  or, -antiproteinase
  cr2-macroglobulin
  Anti-chymotrypsin
  Low-molecular-weight
   bronchial  inhibitor
 Enzymes thought to be
  capable of deranging
  alveolar structures
  Collagenase
  Elastase
  Neutral protease
 Other enzymes
  Lysozyme
  /3-glucuronidase
  Esterase
  Acid protease
  Phospholipase A
  Glycosidase
          Present
          Absent
          Present
         9% of total
        91% of total
          Present
          Present
         Functional
         Functional
         Functional

          Present
     Very low if present
? (present in bronchial mucus)
? (present in bronchial mucus)
          Absent
          Absent
          Present

          Present
          Present
          Present
          Present
          Present
          Present
 *From Hunninghake et al. (1979)
tures of alveolar macrophages are simple to obtain by pulmonary lavage; tracheal
rings, lung tissue slices, and tracheal explants can be maintained in vitro for several
days. Although these cells are altered in chronic disease states, more investigation is
required  before in vitro exposures  can be applied widely as short-term tests for
chronic lung disease.

Macrophages

  Investigations of the mechanisms of silicosis led to the development of model sys-
tems in which alveolar or peritoneal macrophages are exposed in vitro to silica, and
the resulting cytotoxicity is measured morphologically, histochemically, or bio-
chemically. Other particles have been tested, and macrophage cytotoxicity has been
reasonably well correlated with fibrogenicity of fibers (Reiser  and Last,  1979;
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Table 2.   Indicators of Injury in Lung Lavage Fluids*
Endpoint
        Site
                             Possible Indication
                                 if Altered
Lactate dehydrogenase
Glucose-6-phosphate
Acid phosphatase
 and
/J-glucuronidase

Alkaline phosphatase
Glutathione peroxidase
 and
Glutathione reductase
Total protein

Sialic acid
Phagocytic cells
  PMNs
  Macrophages
Cytosol (glycolysis)
Cytosol (hexose mono-
 phosphate shunt)
Lysosomes


Plasma membranes
Type II cell lamellar
 bodies
Serum

Cytosol

Extracellular

Mucous glycoproteins
Cell damage
1.  Cell damage
2.  Leakage from cells
    undergoing repair
1.  Release during phagocytosis

2.  PMN and/or macrophage
    damage
1.  Type II cell damage or
    increased secretions
2.  Transudation of serum-
    proteins
Protection mechanism activated
 against lipoperoxidation

Transudation of proteins a'cross
 alveolar-capillary barrier
1.  Increased mucus secretion
2.  Transudation of serum glyco-
    proteins

Acute inflammation
Persistent inflammation
 *From Henderson (1982)

 Chamberlain et al., 1979). However, this correlation does not hold for various forms
 of asbestos (Allison, 1971).
  One research group (Allison, 1971, 1975; Nash et al., 1966; Allison and Davies,
 1974) studied the interaction of silica and macrophages extensively. The silica par-
 ticles were found to have two major  types of effects: (1) at high doses, rapid cyto-
 toxicity occurs, characterized by release of lysosomal and cytoplasmic enzymes into
 the medium; and (2) at lower doses,  toxicity is delayed for several hours. Silica is
 taken into phagosomes, and lysosomes cluster around and empty their contents into
 the phagosomes. A few hours later, signs of cell damage are seen, probably due to the
 interaction of silica with the secondary lysosomal membrane, which makes it perme-
 able to lysosomal hydrolytic enzymes. The immediate high dose effects are thought
 to be characteristic of particle interactions with cell plasma membranes. The delayed
 cytotoxicity appears to be  a  function of the interaction  of  hydrogen bonding
 between the surface of silica and the membranes of the secondary lysosomes. Silico-
 sis is only generally associated with exposure to these active forms of silica.

 Red Blood Cell Hemolysis
  Research on the mechanisms of interactions between toxic particles and cell mem-
 branes led to the development of hemolysis assays. Mixing the fibers with a suspen-
 sion of erythrocytes results in hemolysis. The hemolysis itself is unrelated to dust dis-
 eases but it is a relatively easy way to study the interaction of chemicals with plasma
 membranes. Most investigations in this area have focused on silica, asbestos, and re-
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Table 3.  Properties and  Functions of Normal Human Alveolar Macro-
           phages*
  Origin
    Derived from bone marrow
    Capable of self-replication
  Surface receptors
    Fc-IgG
    C3B
    C3d
    Proteases-antiproteases
  Interactions with microorganisms
    Bacteria
    Fungi
    Viruses
  Interactions with noninfectious particulates
    Phagocytosis of particulates
    Pinocytosis
    Metabolism of ingested compounds
  Effector and accessory cell  in inflammatory and immune reactions
    Responds to lymphokines
    Responds to chemotactic stimuli
    Regulates  lymphocyte response to  mitogens and antigens
    Secretes
      Colony-stimulating factor
      Chemotactic factor for  neutrophils
      Superoxide  anion
      Platelet-activating factor
      Elastase
      Collagenase
      Neutral protease
    Mediates antibody-dependent cellular cytotoxicy
  Protection of alveolar structures from protease attack
    Contains a,—antiproteinase
    Cytosol inhibits human neutrophil elastase
    Ingests neutrophil elastase
*From Hunninghake et al. (1979)
 lated substances. For silica and asbestos, the correlation between macrophage cyto-
 toxicity and  hemolytic activity is good (Harington et al., 1971).
   The hemolytic activity of forms of silica correlates well with fibrogenic activity
 (Allison,  1971; Harington et al.,1971). However, for forms of asbestos, the corre-
 lation is less  clear. For example, chrysotile, crocidolite, and amosite are very fibro-
 genic when inhaled, but the latter two do not significantly affect hemolysis. Ben-
 tonite and kaolin,  nonfibrogenic compounds, are also more hemolytic than cristo-
 balite and quartz, figrogenic compounds (O'Hery and Gornley, 1978). Various tech-
 nical factors, such as pH, species of animal donating the erythrocytes, time of incu-
 bation, and the precise physicochemical properties of the fiber, can affect results and
 make correlations between different laboratories difficult (Harington et al., 1971;
 Schnitzer and  Pundsack,  1970;  Palekar  et  al.,  1979; Light and  Wei,  1977;
 Summerton  et al.,  1977).
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  Although these hemolysis studies may, therefore, lead to a better understanding of
plasma membrane-fiber interactions, good correlations between silicosis and hemo-
lytic potency have not been reported.

Fibroblasts
  Macrophages can stimulate collagen biosynthesis by fibroblasts, a major step in
the development of fibrosis. Many of the studies of macrophage activity were con-
ducted with silica (Reiser and Last, 1979).  Briefly, when alveolar and peritoneal
macrophages are incubated with quartz, the medium supernate or macrophage
lysate  will stimulate the synthesis or release of collagen from fibroblasts. The direct
addition of quartz to the fibroblasts does not cause the same response, but activated
macrophages without quartz can also stimulate fibroblasts. Complete interpretation
of these studies is again confounded by differences in technique.
  Collagen and prolyl hydroxylase (the rate-limiting enzyme in collagen biosyn-
thesis) were examined in dogs 2-1/2 to 3 years postexposure to irradiated auto ex-
haust  as an indicator of fibrosis (Orthoefer et al., 1976). The collagen content (hy-
droxyproline)  was  unchanged.  Prolyl hydroxylase  was increased  in  one group
having emphysema, but was unchanged in another group similarly diseased. Thus,
collagen biochemistry as studied here was insensitive. However, given  the impor-
tance  of collagen turnover in chronic lung disease and the complexity of collagen
biochemistry, it should  not be dismissed without further research.

Tracheal Epithelium  and Mucus
  Several investigators have successfully correlated the in vitro and in vivo acute ex-
posure effects  of cadmium, nickel, fly ash,  sulfuric  acid, and carbon on ciliary
beating frequency of tracheal rings (Adalis et al., 1977,  1978; Schiff et al., 1978,
1981). However, no attempts have been made to correlate the in vitro acute studies
with chronic in vivo experiments.
  Perhaps a more promising area for short-term tests of chronic effects is in vitro
models of mucus biosynthesis, since mucus production is excessive in chronic bron-
chitis. Tracheas are incubated with labeled precursors of mucous glycoproteins, and
the incorporation of precursors and production of glycoproteins are measured (Last
and Kaizu, 1980; Sturgess and Reid, 1972; Reid and Jones, 1980). Humans, rats, and
monkeys are suitable donors. Mucus-secreting cells have been isolated from ham-
sters and cultured;  these  cells were capable of mucous glycoprotein synthesis
(Baseman et al.,  1980). Most of these studies have been concerned with in vivo pol-
lutant exposure, but investigations of secretory mechanisms have been made in vitro
with pharmacological agents, and in vitro pollutant exposures may be  of interest.

Tissue Slices
  The use of lung tissue slices in toxicology has been revei wed by O'Neil and Young
(1982) and Freeman and O'Neil (1982). The sample preparation is relatively simple
and has been applied to studies of lung metabolism, particularly of phospholipids
and carbohydrates. A significant feature of this method is that because several cell
types are present, cell-cell interactions proceed. The major limitation is that normal
metabolism continues for only about 5 hours  with current techniques, and the
medium is poorly mixed in the airways of the slices,  so kinetic studies are difficult.
No studies have been undertaken to examine the correlation between the effects ob-
served with lung slices and chronic exposure.

CHEMICAL CARCINOGENESIS BY TRACHEAL TRANSPLANTS

  Our discussion of short-term chemical carcinogenesis assays will be limited to the
tracheal transplant and the strain A mouse pulmonary tumor bioassay. In vitro car-
cinogenesis assays are presented by Upton (Chapter 3).
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  Carcinogenesis in  the respiratory tract is known to be  induced by exogenous
factors such as cigarette smoke (Hammond,  1966; Wynder et al., 1970) and occupa-
tional or environmental exposure (Frank, 1978; Kraybill, 1977). The complex inter-
actions of the  precise components of these exposures render the determination of
direct causative  relationships a  difficult task.  In  addition, various endogenous
factors are possibly involved in the induction of cancer. Some individuals may be
predisposed to the development of neoplasms due to physiological, nutritional, or
disease states (Templeton, 1975)  or to genetic abnormalities (Mulvihill, 1975) that
could affect functions such as drug metabolism (Nebert et al., 1978; Paigen et al.,
1978).
  One of the principal methods used in studying the etiology of bronchogenic cancer
is the tracheal transplant. Briefly, the trachea is removed from a donor animal and
transplanted in its entirety into the deep subcutaneous connective tissue of an iso-
genic recipient. After several weeks, essentially no histological difference is discern-
ible between the transplanted tissue and the host tissue. Pellets containing a carcin-
ogen are inserted into the grafted trachea to induce tumor production.  Tracheal
transplants have been successful in rats, mice, and hamsters (Kendrick et al., 1974).
Initial studies with the tracheal transplant system used gelatin pellets containing the
carcinogen (Kendrick et al., 1974); however,  several disadvantages were discovered.
The gelatin pellet inserted into the tracheal lumen dissolved within 10 minutes. Thus,
the carcinogen was released rapidly and moved to the proximal end of the transplant
where most tumors develop. The problem has been eliminated by using beeswax
pellets, which do not dissolve in vj'voand which allow a more even distribution of the
carcinogen throughout the tracheal epithelium. This method also permits better
control over the  release of the carcinogen and the length of exposure (Griesemer et
al., 1977).
  The tracheal transplant model is a valuable tool in determining the relationship
between age and susceptibility to cancer. For example, by using older animals as
donors and young adults as recipients, age-specific characteristics can be studied and
confounding factors associated with old age can be eliminated. This  model is also
useful in studying the immune system's effect on the development of squamous car-
cinomas in that  the host can  be immunosuppressed without exposing the target
tissue (Nettesheim et al., 1981). One criticism of the procedure has been its use of
tracheal tissue rather than bronchial tissue. The trachea in rodents, however, is
similar to the bronchus in humans in that it  contains cartilage, compound mucous
glands, and goblet cells. No other segment of the respiratory tract in rodents has such
a resemblance to the human bronchus (Kendrick et al., 1974).
  The tracheal transplant system has several other advantages, the primary one
being that known quantities of a carcinogen can be administered to  a specific tissue
for a designated  period of time. This kind of control is not possible  with inhalation
exposure or with intratracheal instillation (Nettesheim and Griesemer, 1978). In ad-
dition, the transplanted tissue can be easily inspected for  tumor formation; vital
functions  of the host continue with no interference from the carcinogen or from
developing tumors; and the target tissue and the host can be controlled independent-
ly, allowing the study of endogenous and exogenous factors affecting tumor devel-
opment (Kendrick et al., 1974).
  Using the tracheal transplant method, Topping et al. (1978) studied a range of
polycyclic hydrocarbons (PCHs) both in vitro and in vivo and compared the release
rates of the compounds from beeswax pellets. The results indicated that in vitro re-
lease does not generally predict in vivo release. In vitro release was faster, implying a
slow exchange from the tracheal lumen to the circulation in the transplant. Also, in
vivo release was more variable, probably because changes occur in the tracheal wall
that  affect rates of exchange and release. Later studies by the same group decreased
the variability by altering the pellet matrix (Pal et al., 1978). The results of Topping
et al. (1978) showed that a range of PCHs, including noncarcinogens, initiators, and
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carcinogens, cause significant changes in the epithelial and mesenchymal pathology
of respiratory tract mucosa. The toxic response caused by noncarcinogens was short-
lived. Carcinogens caused long-lasting responses characterized by altered epithelial
differentiation (metaplastic changes). The severity of response to a carcinogen corre-
sponded directly to its potency.
  Nettesheim et al. (1977) used the tracheal transplant system to study the induction
of preneoplastic and neoplastic lesions produced by benzo(a)pyrene, B(a)P, deliv-
ered from beeswax pellets. Exposure time ranged from 1 to 6 months, and cumula-
tive doses ranged from 10 to 2,490 ug. The five highest B(a)P doses (900 to 2,490 /ug
per pellet) had a release rate constant of about 2.8±0.6 percent per day for the first 2
months. Since the amount released is determined by the amount in the pellet, more
was delivered early in the exposure. The duration of the exposure was determined by
the B(a)P dose, and exposures ranged from 0.5 to 6 months. At the three lowest
doses (10 to 300 /ug per pellet),  most of the  B(a)P was released in 1  to 2 weeks
(Nettesheim et al., 1977). Tracheal grafts receiving 3000 /ug B(a)P in gelatin pellets re-
tained about 30 percent of the dose 3 weeks after instillation of the  carcinogen
(Kendrick et al., 1974).  With  B(a)P  doses ranging from 480 to 2,490 ug per pellet,
massive hyperplasia developed in the first few weeks. At  1  month, atrophic-pleo-
morphic lesions were seen, with the reappearance of normal mucociliary epithelium.
This change might indicate the appearance of cells that are resistant to the toxic ef-
fects of B(a)P. A significant number of squamous metaplasias did not develop until 4
to 6 months for any exposure level, and the lesions were only focal; such lesions may
be early preneoplasia,  since some of them appeared months after carcinogen release
had stopped (Nettesheim et al., 1977).
  The first 2 weeks of exposure to tumorigenic doses of B(a)P produced a consistent
hyperplastic reaction throughout the tracheal graft, thus indicating a uniform ex-
posure. Later in the experiment, the response within the same trachea varied greatly.
The implication is that a heterogeneous cell population like the tracheal epithelium
has a variety of responses to a carcinogen, in spite of a uniform exposure (Nettesheim
et al., 1977). Neoplasms  were induced in 10 months or less by B(a)P doses of 900 /ug.
In many cases,  noninvasive and  invasive carcinomas were seen in the  same graft.
Dysplasias were most often found in tracheas with early neoplastic lesions. The pre-
neoplastic and preinvasive neoplastic lesions seen in the tracheal transplant are sug-
gestive of lesions in the  human larnyx and bronchi (Nettesheim et al., 1977).
  Grafted rat tracheas have also been used to determine the effects of 7,12-dimethyl-
benz(a)anthrancene (DMBA). DMBA doses ranged from 0 to 3,200 ug per pellet
(beeswax), and about  1.7 percent of the carcinogen remaining in the pellet was re-
leased per day (Griesemer et al., 1977). At levels of 325 to 3,200 /ug DMBA per pellet,
all grafts showed the same degree of hyperplasia, metaplasia, keratinization, and cel-
lular atypia. After 1  month, the first microinvasive lesions were seen, and at  2
months, tumor nodules were present. All cases were identified as invasive squamous
cell carcinomas, but in some cases of high dose exposure, sarcomas developed with
the carcinomas. At levels of 10 to 210  ug DMBA per pellet, lesions varied both be-
tween and within dose groups. Preneoplastic and neoplastic focal lesions developed.
A22-month tumorigenesis study at these lower doses showed that as the  level of car-
cinogen decreased, tumor incidence decreased and tumor latency increased. The low-
est dose of DMBA to produce tumors with the tracheal transplant system was be-
tween 40 and 10 /ug (Griesemer et al., 1977).
  Cocarcinogenic activity has been studied primarily using skin as the experimental
tissue (Van Duuren, 1969; Van Duuren et al., 1971). Inhalation studies of gaseous air
pollutants have shown no definite cocarcinogenic effect, with the possible exception
of sulfur dioxide (Nettesheim et al., 1981). Rats exposed to B(a)P aerosols and 10
ppm sulfur dioxide developed a 20-percent lung tumor incidence, but only a 3-per-
cent incidence was observed in animals exposed to B(a)P alone (Laskinetal., 1970).
Topping et al. (1981)  studied  the effects of benzo(e)pyrene, B(e)P, in combination
                                     199

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with B(a)P on tracheal mucosa of rats; both B(e)P and B(a)P are found in tobacco
smoke and exhaust from gasoline engines. Previously, B(a)P was found to be cocar-
cinogenic for mouse skin (Van Duuren and Goldschmidt, 1976). The tracheal trans-
plant system was selected by Topping et al. (1981), because this model allows control
of exposure to the carcinogen and the suspected carcinogen  and  of targeting to a
specific segment of respiratory tract mucosa. Data showed no carcinogenicity in the
tracheal epithelium associated with B(e)P, but significant cocarcinogenicity in the
tracheal-peritracheal connective tissue. These results, in combination with the pre-
vious findings using mouse skin (Van Duuren and Goldschmidt, 1976), indicate that
the target tissue type is a major determinant of the cocarcinogenicity of B(e)P.
  The strain A mouse pulmonary tumor bioassay has also been studied as a short-
term test for predicting potential chemical carcinogenicity (Shimkin and Stoner,
1975). Maronpot et al. (1982) used this bioassay in experiments with 60 chemicals.
These results were then compared with those of a 2-year rodent bioassay. Available
data using the 2-year test were adequate for only 54 of the chemicals, so 6 of those
used with the mouse bioassay were excluded from comparative analysis. Overall, the
results lacked agreement, including a high percent of false negative results with the
strain A  mouse test. Possible reasons for the dissimilarities include differences in:
pharmacokinetics and metabolism by species or strains, routes of chemical adminis-
tration, adminstered dose, or duration of exposure to the chemicals. Another factor
might be the precise determination of what constitutes a positive result; judgments
are frequently difficult with the criteria presently used. Although the results seem to
question seriously the validity of the strain A mouse pulmonary tumor bioassay,
several points require attention. First, the 2-year bioassay has not been conclusively
validated, and the strain A bioassay may actually be more predictive in some cases.
In addition, validation must take into account considerations such as the chemical
metabolism in each bioassay model, the specific organ responding to the carcinogen,
the extent to which the response is positive,  the sensitivity  of the bioassay, and
genetics. Any conclusions regarding the use of the strain A mouse system in predic-
ting carcinogenicity cannot be reached until more data are available.

BIOCHEMICAL STUDIES USING_EX VIVO TECHNIQUES

   In the last 5 years, considerable strides have been made in the use of isolated, ven-
tilated, and perfused lung (IVPL) preparations in toxicological studies (Smith and
Bend, 1981). The precise methodology varies between laboratories, but all apply the
same principle of ventilation occurring simultaneously with perfusion. Ventilating
the lung is essential to ensure adequate perfusion. The mechanical  movement of the
lung seems also to stimulate prostaglandin production and may influence a number
of other metabolic steps as well.  Usually, hemoglobin-free solutions, such as Krebs-
Henseleit, supplemented with serum albumin  are used for the perfusion, and re-
quired rates entail the use of large volumes of solution even for small lungs, such as
those from rats. Perfusion rates of less than 2 ml per minute are not generally satis-
factory, and rates of 10 ml per minute or greaterare often used with rat lungs. Blood
has also been used as a general medium, but this procedure suffers from the difficul-
ties of requiring several donor rats, being highly complex and variable, being con-
taminated easily by prior treatment of the donor rats, and being subject to clotting
and hemolysis. Direct measurement of product formation in blood is also difficult,
although it is easily accomplished in hemoglobin-free media (Williams and Menzel,
1982). Because of overlapping absorption  spectra, direct measurement of the oxida-
tion-reduction state of hemoproteins in the lung is not possible in blood-perfused
preparations.
   The major driving force for the use of IVPL preparations is the very complexity of
the lung itself. Only fibroblasts and type II pneumocytes are now cultured easily,
with promise that Clara cells may soon become available. Primary cultures of the re-
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maining cells are difficult to produce with any purity, although strides have been
made in the sorting of separated cells from the lung. Still, cell separation by differ-
ential centrifugation,  elutriation, or cell sorting remains cumbersome and unpro-
ductive. The likelihood that for the next 5 to 10 years these methods will yield suf-
ficient numbers of cells for routine short-term tests is low. Given proper perfusion
medium and careful control of the physiological parameters of the preparation, the
interrelationships between the  multiple cell types of the lung can be preserved. The
IVPL, then, becomes a potential tool for the short-term evaluation of pneumotoxi-
cants.
   Although there is no widely  accepted method for the evaluation of pneumotoxi-
cants using the IVPL, this preparation is easily adapted to studies of the metabolism
of xenobiotic compounds (Williams et al., 1982). Covalent reactions between B(a)P
and lung macromolecules can be observed by perfusion with 3H-B(a)P, homogeniza-
tion of the lung, separation of lung macromolecules by precipitation and exhaustive
washing, and measurement of labeled covalently reacted B(a)P metabolites (Williams
et al., 1982). If covalent reaction with cellular macromolecules is presumed to lead to
either mutagenesis, carcinogenesis, or toxicity, these methods could be used to rank
chemicals quickly.

TESTS OF PARTICLE REMOVAL FROM THE LUNG

   Although the primary function of  the lung is to ensure an  efficient exchange of
gases, this organ also provides the body with a primary protective barrier against air-
borne assaults from both viable and nonviable agents. Any breach in this defense sys-
tem would be expected to increase the host's  risk of disease (Green et al., 1977;
Gardner, 1979; Proctor,  1979; Brain et al., 1977; Sanders et al.,  1977; Brain and
Valberg, 1979). It is readily evident that any dysfunction can  lead to disease, espe-
cially pulmonary infections, bronchial cancer, chronic bronchitis, bronchial asthma,
pneumoconioses, and possibly pulmonary emphysema (Proctor, 1979). It remains
to be determined whether or not the tests that are now available for evaluating the
adequacy of respiratory defenses are useful to detect or screen for disease suscepti-
bility, to note its inception, to study it pathogenesis, and to improve our under-
standing of the relationship between  morphology and function.
  The tests of host defenses that have been most widely used for examining problems
associated with infectious lung disease include measurements of mucociliary clear-
ance, functioning of alveolar macrophages, and susceptibility of experimentally in-
duced infections. The effectiveness of the mucociliary escalator can be determined
by measuring the rate of transport, the frequency of ciliary beating, and the size and
distribution of  mucus-secreting cells (Bang and  Bang, 1977). Any adverse effect
would be likely to reduce the amount of inhaled and deposited substances that could
be removed from the respiratory tract.  Numerous gases and particles have been
shown to impair host defenses  through the depression of the mucociliary escalator
(Adalis et al., 1978; Grose et al., 1980; Wolff et al., 1975; Schiff et al., 1979).
  Another group of investigators (Lippmann et al., 1982; Schlesinger et al., 1978,
1979) investigated the effects of inhaled pollutants on bronchial airway function in
donkeys and humans. After various  periods of exposure, the subjects inhaled an
inert test aerosol ("TC-FezOs), and tracheobronchial mucociliary clearance of the
particles was measured. Mucociliary clearance is an important defense mechanism
of the lung and is usually altered in chronic bronchitis. Generally, the same sequence
of events on mucociliary bronchial clearance was produced  by sulfuric acid and
cigarette smoke after both short-term  and chronic exposures in donkeys. The short-
term exposure studies of donkeys and humans were correlated for both pollutants.
Given the chronic bronchitis produced by cigarette smoke and the results of their
studies, these investigators believe that the observations in donkeys may be related
to chronic bronchitis. From this interpretation, a potential short-term animal model
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might exist. However, the technique itself is more amenable to a research situation,
as opposed to being applied to a screening test.
  Within the. lung, the resident population of alveolar macrophages is a crucial de-
fense because these cells are responsible for a variety of important activities, includ-
ing the detoxification and removal of deposited airborne particles; maintaining pul-
monary sterility against  invading microorganisms; interacting with lymphocytes for
immunological function; as well as acting as scavengers in removing various cellular
debris. In order to fulfill their purpose adequately, these cells must maintain an inte-
grated membrane, mobility, and phagocytic activity, and have a well-developed and
functioning enzyme system (Gardner, 1982a). A number of tests have been developed
to evaluate the "health"  of these cells after either in vivo or in vitro exposure (Waters
et al., 1975; Agostra et al.,  1975; Aranyi et al.,  1981; Brain,  1977; Coffin and
Gardner, 1972; Goldstein, 1977; Graham etal., 1975). Parameters of number, stabil-
ity,  viability,  morphology, function,  biochemistry,  and  metabolism  have been
studied;  the most useful measurements are those  of functional capacity, such as
phagocytosis and bacteriocidal activity.
  Primary cultures of alveolar macrophages have  been recommended recently for
use in an early  stage cytotoxicity assay to obtain preliminary environmental assess-
rnent information, to identify problem areas, and to prioritize  testing procedures
(Duke et al., 1977). Because the macrophage represents the first line of pulmonary
defense,  these cells are appropriately used to define and to "rank" the acute cellular
toxicity  of airborne  chemicals (Aranyi et al., 1981; Waters et al., 1975). The para-
meters most generally tested include viability,  ATP, and  phagocytic activity.  Al-
though these test systems have been utilized primarily to evaluate acute toxicity, the
cells may also provide a key to understanding some chronic pulmonary effects that
could be attributed to the action  of toxic chemicals on these defense cells. For  ex-
ample, as a result of cell injury, cell death, or selective exocytosis, macrophages re-
lease into the lung a number of potent and biologically active proteolytic enzymes,
such as elastase and  collagenase, that are thought to be contributing factors in  the
pathogenesis of chronic lung  disease (Gardner, 1982a; Janoff et al., 1977;  Brain,
1980).
  Because any suppression in the host's body defenses by environmental chemicals is
expected to result in prolonged microbial viability and enhanced multiplication of
the infectious organism, a number of model systems have been developed to deter-
mine the degree of efficiency of the total pulmonary defense system  in a  compro-
mised host. The most  successful model is to introduce  a viable, opportunistic
pathogen within the respiratory tract of the test subject. If the  host's defenses  are
functioning normally, the lungs will return to a sterile condition within a few hours.
However, if the host's defenses have been altered to a degree that permits the infec-
tious agent to establish itself and to multiply, a dysfunction of the defense  system is
then easily recognized (Coffin and Gardner,  1972; Gardner, 1982b). This infectious
model system has been utilized to identify the effects of pollutants on acute pulmo-
nary infections due to Streptococcus, Diplococcus, KlebsieUa, and influenza virus
(Ehrlich, 1980; Gardner, 1981; Ehrlich and Fenters, 1973; Maigetter et al., 1976).

SUMMARY

  At present, no short-term tests for chronic pulmonary disease have an acceptable
false negative error rate. In attempting the development of adequate  tests, several
factors must be considered. A more rapid means to  detect chronic lung disease is
desirable, but it must detect all potential major disease classes. A rapid test for only
fibrosis would not be satisfactory, because chronic studies would still be needed to
determine if the chemical caused other diseases like emphysema. In the chronic study
in search of emphysema, the presence of other diseases, like fibrosis, would  be de-
tected. Thus, models for all major lung diseases must be pursued concurrently.
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  The most promising short-term tests for chronic diseases would measure early
events in pathogenesis, like alterations of DN A or cellular transformation in tests for
mutagenesis and carcinogenesis. The isolated, ventilated, and perfused lung prepar-
ation is a new rapid assay system of particular promise. The ease with which cova-
lently reacted  DNA, RNA, and proteins can be isolated from perfused lungs after
treatment with toxicants and the preservation of the physiological state of the lung
during measurement make this system particularly useful (Williams et al.,  1982).
Toxicants can be administered rapidly by intratracheal  instillation, ventilatory
gases, or aerosol. Particles are easily applied in liquid suspension. Radiolabeled, as
well as nonradiolabeled, compounds can be evaluated. Validation should be under-
taken by correlating short-term binding and metabolism with chronic toxicity.
  Several early events for chronic lung disease are known, but when this knowledge
is applied to the development of model systems, correlations with chronic effects are
very weak. Perhaps part of the problem is that the available models, like collagen
synthesis and macrophage cytotoxicity, measure only a few of the total early events.
The more successful rapid screens for mutagenesis and carcinogenesis employ a bat-
tery of tests, each making a contribution in terms of detecting a single early event in
the disease process; together they cover the range of potential events reasonably ef-
fectively. To overcome this problem for pulmonary diseases will require mechanistic
studies on the pathogenesis of the major pulmonary diseases, so that a battery of
tests could be developed. To select just one test for each disease would probably lead
to excessive false positive error rates if the pathogenic event were too early in the dis-
ease process or to excessive false negative rates if the mechanism being screened were
an intermediate event.
  Attempting  to  develop, refine, and  validate a battery  of  short-term tests for
chronic lung disease is a vast undertaking. A typical chronic study takes 1 to 2 years
and is relatively expensive, because a large number of animals are used and extensive
examinations are  performed. However, if the typical chronic study could be refined
to reduce both the time and cost of testing, then it might meet more of the criteria for
a good short-term test than would a series of in vitro studies. Refining and validating
a more rapid chronic study will require intense investigation. Shortening the length
of exposure, from even 12 to 6 months, will require the identification of early  stages
of pathogenesis. Thus,  more studies of mechanisms in animals are needed, both to
identify early stages and to correlate such changes with those in humans, to ensure
that the data will have value in human risk assessment. To reduce costs, the number
of endpoints examined must be reduced; mechanistic studies are also required to en-
sure that the needed endpoints remain in the protocol. In addition, improvements in
measurement techniques  will reduce variability and, hence, lower the number of
animals required. Dose-rate considerations in toxicology can be complex, and the
potential recovery or progression of the effects when exposure ceases is important.
Usually, a high dose shortens the time required to observe an effect in inhalation
toxicology, but the level can be so high that additional mechanisms of toxicity  which
are unrealistic  will come into  play. Thus, definition of a high dose is quite difficult
and might have to be arbitrarily assigned as some function of the LC5o. For some
compounds, even determining the LCso can be difficult because of engineering con-
siderations in generating the proper test atmosphere. Choosing the proper exposure
time is also critical. As currently defined, chronic diseases are just that—chronic; they
take some time to develop. If the exposure period is too short relative to the endpoint
for a particular stage in the pathogenesis of the disease, a false negative error  will
occur. This question might be resolved by encouraging researchers on current or
projected long-term studies to make examinations at several times, especially when
newer techniques of pulmonary pathology, function, and  biochemistry are  being
used. The accumulated data can be used in determining validation procedures for
the short-term test. The validation would include chemicals of high, medium, and
low potency for inducing all  major types of chronic lung disease.
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  Despite the difficulties, short-term tests need to be developed for specific pulmo-
nary diseases. Inhalation is a major, efficient route of exposure to a wide variety of
chemicals. The effects of these chemicals on man are not now known precisely, and
thus the risk to the exposed population is also poorly quantified. The driving force
for short-term tests is real and urgent enough to encourage further development. Re-
search into basic mechanisms is paramount, for in this way more relevant tests can
be developed. Knowing the mechanisms of human disease, short-term in vitro tests
are more likely with cell, tissue, or organ culture. The rapid advances in lung cell and
organ culture are encouraging. A major parallel effort is underway to define the in-
haled dose to the target region of the lung. By comparing human and animal lungs,
the magnitude of the regional dose can  be  estimated and a  better scale-up from
animals to humans can be determined. Although no quantum jumps in short-term
tests for pulmonary toxicity are on the horizon, steady progress is being made to as-
sure the applicability of animal test results to human disease processes, to reduce the
time needed to attain  a clear indication  of pulmonary disease in animals, and to
compare doses producing disease in animals with those required in man.


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                                     INDEX
 Abortion (see also Human abortuses)
   chromosomal defects, 30,70
   surveillance methods, 70
   spontaneous, 30,70,78,79
   therapeutic, 78
 Acetaminophen
   toxic effect of, 97
 Achondroplasia, 73
 Acinus, gas exchange, 177
 Acrylamide
   neurotoxic effects  of, 114
   weight loss from,  114,121,132
 Activity, measures of
   and hazard identification, 118,120,133
 Acute versus chronic exposure, 4
 Adenocarcinomas, vaginal
   and diethylstilbestrol, 2
 Adenosine triphosphate
   transport functions, 90
 ADH (see Antidiuretic hormone)
 Aflatoxin B,
   activation-requiring, 17
   liver carcinogen, 17
 Aging
   induced by toxic substances, 11,81
   of brain, 131
 Air pollutants
   and COLD, 179
   behavioral toxicity,  109,124
   carcinogenicity, 199
 Air samples (particulates)
   complex mixtures, 31
 Alcohol syndrome, fetal, 82,83
 Alkylating agents, 44,45,59,63-66,75
   chronic exposures, 4
   reaction with DNA, 4,6,8
 Alkylguanine, 4,5
 Ames test (see also "mutagenicity test"
 under Salmonella)
   for mutagenicity, 18,19,49,71
p-Aminohippuric acid (see PAH)
 Amniocentesis
   and fetal monitoring, 78
AMP, 101
Amphetamine
  behavior modified  by, 129
Analgesics
  nephrotoxic effect, 97
Aneuploid cells
  focus assay, 41
  spontaneous transformation, 43
Aneuploidy
  assays for, 21,26,28,29
  development of tests for, 23
  Down's syndrome, 30
  evaluation, 21,28,29
  fungal assays, 23,28
  mental and physical defects, 15
  spontaneous abortion, 30
Angina pectoris
  and exercise performance, 139
Animal pharmacokinetics
  dose-response extrapolation, 7,8
Anticoagulants
  dicoumarin, 83
Antidiuretic hormone (ADH), 94,95,101
Antigens, and
  glomerular lesions, 96
  vascular  injury, 144,145,161
Aroclor-1254
  induction of enzymes,  17
Aromatic amines, 44,54,60,62
Arsenic
  and cardiovascular mortality, 141
Arterial endothelium
  contractile protein in, 146
Arterial injury
  and tobacco antigen,  158
  hypothesis, 143-144,153
  lipid accumulation,  156-157
Arterial permeability
  and angiotensin II, 158
Arteriosclerosis (see also Atherosclerosis),
 and
  inflammation, 144
  thrombus formation,  162
Arteriosclerotic heart disease
  personal  risk factors,  137-138
Arteriosclerotic plaques (see also
 Atherosclerotic plaques; Plaque formation)
  development  of, 144
  isoenzymes, 153
  transformation of smooth muscle cells,
   154-155
Arylamines, 45
Asbestos
  and COLD, 179
  hemolytic activity, 196
  lung mesothelium, 2
  macrophage cytotoxicity, 194
  transformation effects, 49
Aspergillus flavus
                                         211

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  aflatoxin synthesis, 17
Aspirin
  nephrotoxic effects, 97
  teratogenicity, 71
Assay systems (see also  individual test
 systems; In vitro tests)
  ideal, properties of, viii,39
  comparative results, 44,45,49-50,63-66
Asthma, and
  anatomic dead space, 186
  chemical exposure, 179
Atherosclerosis (see also Arteriosclerosis)
  and diabetes, 137,159
  and graft rejection, 149-150
  and immune reactions, 151
  and platelet function, 143
  and trace elements, 141
  and vasectomy, 148
  and virus infections, 146,151
  deaths from,  136,137
  development of, 143
  distribution in arterial tree, 148
  precocious development of, 145,161
Atherosclerotic plaques  (see also
 Arteriosclerotic plaques;
 Plaque formation)
  avidity for lipid, 148
  cholesterol, 137
  tumor growth, 162
Atomic bombs
  human mortality data, 2,3
ATP (see Adenosine triphosphate)
ATPase (see also Na, K-ATPase)
  activity affected by uranium, 99
  transport-related,  105
Autoimmune phenomena
  and silicosis,  192
Automobile exhaust
  benzo (a)pyrene in, 18,200
Aversion and attraction
  toxicity assessment by, 122,124
Avoidance conditioning
  toxicity assessment by, 120

Background level
  as risk assessment  parameter, 1,9
  of DNA adducts, 6,7,8
  of ionizing radiation,  2
Bacterial DNA repair tests
  as short-term tests, 18,19,23
Bacterial mutation test,  50,63-66
 (see Ames test; "mutagenicity test"
 under Salmonella)
Base-pair substitutions
  Salmonella assay,  19
Behavior, and
  heavy metals, 109
  effect of microwaves on,  121-122
  forced-choice inventories,  129
  rating scales,  109,110
Behavioral teratology, 122,125,129
Benzo(a)pyrene
  and neoplasia,  199
  sources, 18
Bioactivation, 41,43,46,71,75,76
 (see also Hepatic microsomal
 monooxygenase; Hepatocyte feeder
 layer; Liver microsomal homogenates;
 Metabolic activation)
Biotransformation (see Bioactivation)
Blood, in
  micronucleus assay, 25
  mutagenicity tests, 27
  population monitoring, 32
Blood lipid
  and ischemic heart disease, 137
Blood pressure
  physical factors in,  142
Blood ultrafiltrate
  Bowman's capsule, 90,91
Blood urea nitrogen
  as renal function test, 101
Bone cancer
  from atomic bomb, 2,3
Bone marrow  cells
  chromosome aberrations assayed in, 26
Boron
  nephrotoxic, 98
Brain, and
  aging effects, 122-123,125
  electrical "noise", 112,114,131
5-Bromodeoxyuridine
  for detection of SCEs, 26
Bronchial airway function
  and inhaled pollutants, 201
Bronchitis, and
  chemical exposures, 178-179
  cigarette  smoking, 179-191
Bronchoscopes, 177

Cadmium
  brain serotonin decreased by,  132
  excretion, 99
  inhalation, 99
  nephrotoxic, 98
  protein binding of, 96,99
  pulmonary assays, 197
  testicular atrophy from, 118
Capillary bed, renal, 90
Carbon
  in vitro assays  for, 197
Carbon disulfide
  and cardiovascular disease, 142
  and weight loss, 132
  behavioral toxicity from,  108,130
Carbon monoxide
  and ischemic heart disease, 139-140, 158
Carbon tetrachloride
  tissue damage from, 100
Carboxyhemoglobin
  and atherosclerosis, 139
Carcinogenesis
                                         212

-------
  multicausal, multistage nature, 47,81
Carcinogenic analogues, 55
Carcinogenicity
  and mutagenicity, 31
Carcinogens
  correct identification, 51,52
  tracheal transplants, 198-199
Carcinoma of lung, 181
Cardiac rhythm
  and magnesium, 140
Cardiovascular diseases
  and occupational inhalants, 141-142
  and water hardness and trace metals,
    140-141
  deaths from, 136
Cardiovascular mortality, and
  arsenic, 141
  inactivity, 160
  recent trends,  162
Cell culture (see  also Colony assay;
 Embryonic cells; Fibroblasts; Focus assay;
 In vitro tests; Mammalian cells in
 culture; Mass cultures; Transformed cells)
  and chemical Carcinogenicity assays, 48
  epithelial, 46,48,103
  in teratology, 75
Cellular membranes, renal
  transport mechanism, 90
Center for Disease Control, 78,83,87
Chemical Abstracts Service Registry
 Numbers, 82,86
Chemicals (see also specific classes)
  Carcinogenicity assays of, 39
  complex mixtures, 31,32,35
  miscellaneous compounds,  44,45,59-66
  mutagenic effects,  13
Chinese hamster ovary cells
 (see also Hamster cells)
  in vitro tests, 27
Chlorinated hydrocarbons
  nephrotoxic, 100
CHO  cells (see Chinese hamster ovary cehX
Cholesterol, and
  ischemic heart disease, 137,157
  low-density lipoprotein, 156
Chromium
  and cardiovascular disease, 141
  nephrotoxic, 98
Chromosomal aberrations
  aneuploidy, euploidy, 15
  in fungi, 23,28
  in vitro and in vivo tests for, 22,23,27,28,
   29,30,32
  micronucleus test for, 25,26
  teratogenic, 69, 70, 74
  translocations  and inversions, 15
Chromosome complement
  of humans, 15
Chronic bronchitis, and
  description,  179,180
  glycoprotein changes, 191
   pulmonary function tests, 187-191
   sulfur dioxide and cigarette smoke, 191
Chronic obstructive lung disease (see also
  Bronchitis; Chronic bronchitis;
  Chronic pulmonary disease; Emphysema)
   flow volume curve, 188
   maximum midexpiratory flow rate,
    187,188
   pulmonary function tests, 187-191
   susceptible subpopulations,  175
   types of, 179
Chronic pulmonary disease
   function tests, 184
   short-term tests,  175,203
Chronic restrictive lung disease
   description,  184,188
   flow volume curve, 188
   lung volumes, 189
   maximum midexpiratory flow rate,
    187,188
   pulmonary function tests, 187-191
Cigarette smoke
   allergenic glycoprotein antigen in,
    146,158
   benzo(a)pyrene in, 18,200
  carbon monoxide in,  139
   mucociliary  clearance and, 201
   mutagenic, 31
   respiratory tract carcinogenesis, 198
Cigarette smokers (see also Smoking)
  age effect, 158
  ischemic heart disease, 137-138, 158
   lung cancer, 16,17
   mutagenicity of urine, 32
   pulmonary lavage, 193
  sex differences in COLD, 176
Circulatory system
   aging of, 160
Clara cells
   in lung preparations, 200
Clastogenic agents, 15
Cleft lip and palate, 73
CO (See Carbon monoxide)
Cobalt
   and atherosclerosis,  141
Cocarcinogens
   assays for, 47,48,56
COHb (see Carboxyhemoglobin)
COLD (see Chronic obstructive
  lung disease)
Collagen biosynthesis
   macrophage-stimulated,  197
   short-term assays, 203
Colony assay, 39-40,42,48
   advantages, 40
   comparisons, 43
   soft agar assay, 43,45,48,63-66
Common garden pea (see Pisum sativum)
Complement cascade
   development of atherosclerosis, 146-147
Complementarity
                                         213

-------
   of replication,  14
 Complex behavior
   assessment of,  118-121,128-129
 Complex chemical mixtures
   and environmental monitoring, 31,32,35
 Computer technology
   in behavioral toxicology, 128
 Congenital defects, 70,73,78,80,82
 Consumer Product Safety  Commission,
  66,67
 Coronary heart disease
   psychological stress, 142
   zinc/copper ratios, 141
 Cortisone, 71,73
 Critical organ concentration
   definition of, 94-95
 Croton oil
   absence of transformation, 49
 Cytochalasins
   and cell division, 75
 Cytochrome P^tSO
   in induced S-9  fractions, 17
 Cytosol, renal,  104

 Data collection, vi,79
   extrapolation of, v,vi
   false positive findings, 79-80
   memory bias, 80
 DDT
   effect on nervous system, 109,132
 Dead space ventilation, 186
 Deactivation pathways, 4
 Degranulation, 50,63-66
   liver reticulum, 49
 Delaney Clause, 53,87
 Density-dependent inhibition
   of cell division, 38
 Deoxyguanbsine
   ethylation products of, 6
 Deoxyribonucleic acid (see DNA)
 Diabetes, and
   abnormal microvasculature, 159
   atherosclerosis, 159-160
   autoimmune disease, 160
   viruses, 160-161
 Dichloromethane
   behavioral toxin, 108
 Dicoumarin
   teratogenic, 83
 Diethylstilbestrol
  transplacental carcinogen, 78,81
   vaginal adenocarcinomas, 2,9
Dimers (see Pyrimidine dimers)
Dimethylbenz (a)  anthracene
   in tracheal transplants, 199
Dimethylnitrosamine
  activation of, 17,18
  dose-response curve, 3,4
Diploid cells
  in colony assay, 42
Distal tubule (see  also Tubular [adj],
 Tubules)
  anatomy, 90,91,93
  and mercurials, 97
Diuretic drugs
  mercurials in, 97
  urine concentration, 94
DNA
  structure of,  14
DNA adducts (see also Alkylguanine;
 Ethylguanine)
  background levels, 6,7,8
  concentration vs dose, 5,6
DNA damage
  assayed by SCE, 26,27
  gene conversion in fungi, 23
  short-term tests for,  20-22,23,24,26-27,
   28,29
DNA repair
  of sunlight damage,  5
  quantitative role in carcinogenesis, 5,7
  tests for, 20-22,23,29,49
Dominant lethal test
  whole-mammal in vivo  test, 25
Dosage regime, vi
  maximum, fractional, minimum, 73-74
Dose-response curves, v,vi,71
  and teratogenicity, 71,73,75
  extrapolation of,viii,l,2,3,9
  linear, linear quadratic, quadratic, 2,3,4
  survival versus mutation, 13
Doubling dose
  in cancer mortality, 2
Down's syndrome, 80
  aneuploidy, 30
  trisomy of X chromosome, 15
Drosophila melanogaster
  activating enzymes in, 18,24
  aneuploidy detected  in, 28,29
  In vivo short-term assays, 12,30
  sex-linked recessive lethal test, 12,24,30
  spontaneous  mutations,  12
  x-ray-induced mutations, 12

E. co/i (see Escherichia coli)
Electrophysiological tests
  toxicity assessment by,  112-114,130,131
Embryo-fetal lethality, 71,73,80
Embryonic cells
  colony assay, 41
  in vitro tests, 75,76,81
  mass  culture  assay, 44,47
Emphysema
  in alveoli, 177
  immunological tests,  193-194
  pulmonary function  tests, 187
  types of, 179
Endothelial cell surface
  and immune complexes, 146,151
  Fc and C3 receptors, 146
  nicotine effects, 158
  renin effects, 158
                                         214

-------
Endothelium
  and diabetics, 159
  barrier function, 157
  permeability and renin, 158
Environmental monitoring
  by higher plants, 32,33
  of complex chemical mixtures, 31,32
Environmental Protection Agency,
 v,19,34,38,67,87
Environmental risk factors
  and ischemic heart disease,  138-142
Environmental Teratogen Information
 Center, 86
Enzyme assays, vii, 104-105
Enzymic radioimmunoassays
  ultrasensitive, 5
Enzymuria
  measurement of, 101
Epidemiologic studies
  limitations of, 16
  risk assessment, v,vi, 1,2
Epidermal  cells (see  Cell culture)
Epithelial cell culture (see Cell culture)
Escherichia coli
  repair capacity,  23,24
  supermutagenic  effects, 13
Ethology
  and behavioral toxicology, 128
O '-Ethylguanine
  carcinogenic, 5,6
Euploidy
  and lethality, 15
Excision-repair pathway (see also Nucleo-
 tide excision-repair pathway)
  error-prone, 14
Exercise
  cardiovascular fitness, 160
  respiratory dead space, 186
Exposure limits
  legal processes, viii
  setting of, vii
Exposure regime (see Dosage  regime)
Extrapolation, vi,vii
  linear, 7
  mathematical models,  2-4
Ex vivo lung techniques, 200-201

False negative findings, viii,40
  complementary tests, 45,49,50
  false-alarm probability, viii
  in behavioral toxicology,  133
False positive findings, viii,40
  complementary tests, 45,49,50
  epidemiological  studies, 79,80
  in behavioral toxicology,  133
Fetal alcohol syndrome,  82
Fetus
  monitoring of, 78
  sensitivity to methylmercury, 125
  vunerability,  122
Fibroblasts (see also Cell culture)
  cell transformation studies, 38,40,43
  collagen biosynthesis by, 197,201
  colony assay, 39
  focus assay, 41-42
  require metabolic activation, 54-55
  unscheduled DNA synthesis, 27
Fibrosis
  collagen deposition, 184,194
  contrasted to COLD, 184
  fibrous mineral exposure, 179,191-192
  interstitial, 178-180
  pulmonary function tests, 187
Fish
  flounder kidney preparations, 104
  methylmercury contamination, 112
Fixed interval performance, 121,133
Fluoro-alkanes
  and myocardial irritability, 142
Fly ash
  in vitro assays for,  197
Focus assay, 48,55,62-63
  comparison with  colony assay, 42
  method, 41-42
Food additives
  behavioral toxicity  of, 109
Food and Drug Administration, 53,66,67,
 87,88
Forward mutation
  in fungi, 23
  in mammalian cell culture, 23
  in Neurospora, 28
  in vivo test in somatic cells, 22,24,25,29
Frameshift mutagens
  and Salmonella assays, 18-19
Fruit flies (see Drosophila)
Fungi
  metabolic activation, 18
  short-term tests, 19,23,28-30
Fungicides, mercurial
  nephrotoxic, 98

Gene conversion
  in S. cerevisiae, 23,28
Gene mutations, 15
  and congenital defects, 70,73
Genetic heterogeneity
  mutation-prone genotypes, 32
  variation in susceptibility, 73,80
Genetic toxicology
  defined,  11
  future emphases,  35
Gene-Tox Program, 19,20-22,34
Germ cells
  dominant lethal mutation assay, 25
  in vivo mammalian tests, 24,25,26,30
  of higher plants, 23
  sex-linked recessive lethal assay, 25
Glomerular filtration, 92, 100
  dynamics, 90,91
  immunologic-type reactions, 96
  in vitro tests, 102,103
                                         215

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Glomerular tubule function
  in vitro tests, 102
Glomerulus, 90,91,92,93,96
Golden Syrian hamster cells
 (see under Hamster cells)
Guinea pig cells
  spontaneous transformation, 39
  embryo focus assay, 41,48

Hamster  cells (see also Chinese
 hamster ovary cells)
  baby hamster kidney (BHK-21)
   colony formation in soft agar, 43
   focus  assay, 42
  Golden Syrian, for colony assay, 40,42,
   48,59-61
  spontaneous transformation, 39
Hazard identification and evaluation, 27-30
Hearing
  impaired by methylmercury, 111
Heart attacks
  age effects, 160
  familial predisposition, 138
  induced by sex hormones, 160
  toxic substances, 11
Heavy metals (see also Cadmium; Lead;
 Mercury; Platinum; Uranium)
  subcellular partitioning,  95
  toxicity of, 89,90,94,98,108
Henle's Loop (see Loop  of Henle)
Hepatic coma
  neurological effect, 132
Hepatic microsomal monooxygenase
 (see also Liver microsomal homogenates)
  for bioactivation, 41,71,75
Hepatitis
  and cardiovascular disease,  161
Hepatocyte feeder layer
 (see also Hepatic microsomal
 monooxygenase; Liver microsomal
 homogenates), 55
Herpes virus
  and atherosclerosis, 151
Higher plant assays, 23
  in silu environmental monitors, 32
  metabolic activation systems, 23
Histopathology
  in toxicity assessment, 131
Homeostatic functions,  121-122
  role of kidney, 94
Host-mediated assays
  for genotoxic activity,  16,27
Human abortuses (see also Abortion)
  thyroid  tissue,  75
  spontaneous, 78
Human body fluids
  population monitoring, 32
Human cells
  in focus assay, 41,48
  population monitoring, 32
  spontaneous transformation, 39
 Human population monitoring
   short-term tests, 32,34,35
   use of body fluids and cells, 32
 Humans
   cancer mortality from atomic bombs, 2,3
   chromosome complement of,  15
 Hydralazine
   and glomerulonephritis,  96
 jV-Hydroxyurethane
   genotoxic, 23,24
 Hyperactivity
   lead-induced,  133
 Hypercholesterolemia
   endothelial injury, 157
 Hyperlipemia, 156
 Hypertension
   and ischemic heart disease, 141,158-159

 Identical twins
   and drug clearance, 78
 IHD (see Ischemic heart disease)
 Immune complex deposition
   antigen effects, 145,161
   caloric intake, 148
   from drugs and microbes, 145
   in arterial endothelium, 146-147
 Industry
   evaluation of short-term  assays by, 30-31
   teratologic testing by, 87
 Inhalants
   and ischemic heart disease, 141-142
 Initiating agents (see also Carcinogens)
   properties of, 49
 Insecticides
   nephrotoxic,  100
 Instrumented tasks
   and reflex  testing, 114
 Insulin
   and heart disease, 159
 Interagency Regulatory Liaison  Group,
 53,66
 International Agency for Research in
 Cancer, 87
 Interstitial  nephrotoxins, 96-100
 Intimal thickening
   and atherosclerosis, 154,157
 Inulin
   renal clearance, 101
 In vitro tests (see also Assay systems;
 Cell culture; Colony assay; Embryo
 culture; Focus assay; Mass culture;
 Organ culture)
   for carcinogens, 42,55
   for renal function, 102-105
   for teratogens, 74-76,81
   mammalian cell culture
   chromosome aberrations, 26-27
   forward mutations, 23,24
   sister chromatid  exchange, 26
In vivo tests
   for renal function, 101
                                        216

-------
   mammalian
    chromosome aberrations, 26
    mouse spot test, 24,25
    sister chromatid exchange, 26,27
    specific locus test, 25
 Ionizing radiation
   carcinogenic,  2,81
 IQ (see also Rating scales)
   and toxicity assessment, 125,126
 Ischemic heart disease, and
   birth control pills, 138,160
   carbon monoxide, 139-140
   cholesterol, 137
   cigarette smoking, 137-138
   environmental risk factors, 138-142
   high blood pressure, 137
   zinc/copper ratio, 141

 Kepone
   neurological disorders, 115
 Kernicterus, 70
 Kidney (see also Glomerular [adj.];
 Nephron; Renal [adj.]; Tubular [adj.];
 Tubules)
   anatomy of 89,90,91
   as target organ, 99-100
   functional tests,  101,105
   in vitro cell assays,  102,103,105
   in vitro perfusion, 102
   functions, 89,90
   reserve capacity of, 94,100
   urine formation in, 90

 Lead
   and hypertension, 141
   behavioral toxicity from,  108,121,133
   blood levels of, 121,125
   depressed nerve conduction velocity from.
    116
   depressed school  performance from,  108,
    125,126,133
   in deciduous teeth, 125
   nephrotoxic, 98,99
   sources of, 99,105
 Learned behavior
   avoidance conditioning, 120,121
   foraging behavior, 129
   Schedules of Reinforcement of, 121,129
   toxicity assessment by, 121,129
 Leucocytes
   and chromosome aberrations, 27,32
 Leukemia
  incidence, from atomic bomb, 2,3
 Life span
  and caloric intake, 148
Limbs of Henle (see also Loop of Henle)
  and mercurial  toxicity, 97
Lipid accumulation
  in cellular membranes, 90
  potentiated by carbon monoxide, 158
  preferential sites for, 148
Lipid filtration hypothesis
  in atherosclerosis,  143-144
Lipoprotein
  in injured arteries, 157
  mitogenicity, 157
Liver
  activation enzymes in, 14
  damage from carbon tetrachloride, 100
Liver cells (see Cell culture)
Liver microsomal homogenates
 (S-9 homogenates) (see also  Bioactivation;
 Hepatic microsomal monooxygenase;
 Hepatocyte feeder layer; Metabolic
 activation; Rodent liver homogenates)
  and colony formation, 43
  for transformation, 41,49,54
Longitudinal studies
  in behavior teratology, 125,129
Loop of Henle (see also Limbs of Henle)
  anatomy of, 90,91
  as countercurrent multiplier, 94
  carbon tetrachloride effects on, 100
  uranium effects on, 98
Lung architecture
  elastic compliance  of, 186
  mathematical models of, 177
Lung cancer
  and cigarette smoking, 16,17
Lung disease (see Asthma; Bronchitis;
  Chronic bronchitis; Chronic obstructive
  lung disease; Chronic pulmonary disease;
  Chronic restrictive lung disease;
  Emphysema)
Lung fibrosis (see Fibrosis)
Lung functions
  measurements,  186
Lung metabolism
  in tissue slices,  197
Lung preparations
  as promising assay systems, 203
  fibroblasts, pneumocytes, Clara cells,
   200-201
  in vitro assays,  194-197
  metabolism  of xenobiotics,  201
  methodology for, 184,199,200
Lung ventilation
  measurements of, 184-187
Lung volume
  compliance of,  186
  in chronic obstructive and restrictive lung
  disease,  187
  measurement of, 184-186
Lymphocytes
  chemically induced SCEs in, 27

Macromolecular adducts
 (see also DNA adducts)
 removal of, 5
Macrophages,  alveolar, 177,178,196,202
  and silicosis, 192,195
  elastase and  collagenase release by, 202
                                         217

-------
  primary cultures of, 193,194,195,202
  properties and functions, 196
  short-term cytotoxicity assays, 202
Magnesium,
  and cardiovascular diseases, 140
Maize
  activating enzymes in, 18
  as environmental monitor, 31-32
Mammalian cells in culture
 (see also Cell culture)
  activation ability, 18
  chromosome aberrations, 27
  forward mutations in, 23,24
  sister-chromatid  exchanges  in, 25,26
Mammalian embryo culture
 (see Embryonic cells)
Mammalian in vivo tests, 24-27
Manganese, and
  motor dysfunction,  114
  secretonin concentration, 132
Mass culture, 46,47,48,55
Maze
  activity assessment in, 118
Mega mouse studies, 87
Membranes (see Cellular membranes, renal;
 Plasma membranes, renal)
Mental retardation, 70
  and fetal alcohol syndrome, 82
Mercury (see also Methylmercury)
  behavioral toxicity from, 108,115
  nephrotoxic, 97-98
Mesenchymal derivatives (see Fibroblasts)
Mesotheliomas, lung
  and asbestos, 2,179
Metabolic activation (see also Bioactivation)
  formation of mutagenic  intermediates, 13
  in vitro,  17
  kinetics of, 4
  of cellular macromolecules, 4
  of higher plant systems,  23
Metallic compounds
  nephrotoxic, 98
  transformation and  mutagenicity of, 44
Metallothionein
  cadmium binding by, 99
Methylbutyl ketone
  and nerve conduction, 116
Methylmercury (see also Mercury), and
  brain  and sensory development, 108,111
  electrophysiological assessment of, 114
  fetal sensitivity to, 123
  neuromuscular lesions, 122
  peripheral vision, 113
  poisoning epidemics, 114,122
  psychophysical assessment,  112
  weight loss,  121,132
Methyl methanesulfonate
  dose-response curve of, 4
Mice (see also  Mouse cells), and
  behavioral research with, 116,124
  host-mediated assay, 27
   MSG-induced brain damage in, 131
   teratogenicity assays, 71,73
   whole mammal in vivo tests, 24-27
Micronucleus test
   for somatic cell risk estimation, 25,26,30
Microsomes (see also Liver microsomal
 homogenates)
   activating enzymes in, 17
   renal,  104
Microwave exposure, behavioral effects,
 121,123
Minamata disease (see "poisoning epidemics"
 under Methylmercury)
Mitochondria, renal,  104
Mitogens, platelet-derived
   in injured arterial wall, 144,148,157
Mitomycin-C
   induction of SCEs by, 26,27
Monkeys, in
   behavioral toxicology, 112,113,121
   teratologic toxicology, 71
Monoclonal hypothesis
   of atherosclerosis,  143,153-158, 161
Monocytes, and
   immune complex deposition, 148
   mitogens, 148
Monooxygenase system
   bioactivation of, 71,75,76
  species variation in, 75
Monosodium glutamate
  and brain damage,  131
Monosomy
  and spontaneous abortions,  15
Motor control,  117
Mouse cells
   BALB/3T3 and C3H/10T1/2 embryo
   fibroblasts, 41
  spontaneous transformation of, 39,40
Mouse spot test
  forward mutation, 24,25
  somatic cell risk estimation,  28,30
Mouse, strain A
  tracheal transplant bioassay,  197-199
Mucociliary clearance
  and chronic bronchitis, 201
Mucous biosynthesis
  in vitro lung studies, 197
Multilocus deletions, 15
  for risk assessment, 28,30
  in heterozygotes, 28
  in Neurospora, 28
  strains and tests for, 19-23,28,29
Multivariate analyses, 129,130
Mustard  gas
  mutagenic, 13
Mutagenicity
  and carcinogenicity, 31
Mutant
  defined, 12
  in short-term assays, 13
Mutants, biochemical
                                        218

-------
  in Neurospora, 13
Mutation-prone individuals
  identified, 32
Myocardial infarction, and
  CO pollution, 139
  inhalants, 141-142

Na, K-ATPase
  inhibition by platinum, 100
  transport-related  enzyme,  94,96,105
Nares, tumors of, 179
National Cancer Institute, 30,87
National Center for Toxicologic Research,
 87
National Institute of Child Health and
 Human Development, 87
National Institute of
 Environmental Health Service, 86,87
  Toxline, 86
National Institute of Occupational Safety
 and Health, 87
National Library of Medicine, 86
National Toxicology Program, 67,83,87
Naturalistic behavior
  toxicity assessment  by  118,120,128
Neonate
  behaviorial teratology  in,  122,125
Neoplastic transformation, 40
  spontaneous, 39
Nephron (see also Glomerular [adj];
 Glomerulus; Kidney; Renal [adj];
 Tubular [adj]; Tubules)
  anatomy of, 89,90-94
  in flounder, 104
  in vitro tests, 102-105
  micropuncture of, 102
  salt and water movement  in, 93
Nephrotoxins
  tubular and interstitial, 94-100
Nerve conduction velocity, 116-119
Nerve gases
  as anticholinesterase agents, 120
Neural tumors
  from  O '-ethylguanine, 5
Neuromuscular lesions
  methylmercury-induced, 123
Neurospora
  biochemical mutants of, 13
  forward mutations in,  28
  metabolic activation of, 18
Newborn monitoring, 78
Nickel
  and serotonin concentration, 132
  pulmonary, in vitro assays for,  197
Night blindness
  and methylmercury poisoning, 112
Nitrogen oxides
  in pulmonary function  tests, 175,190
Nitrogen washout tests, 187
Nitrosamines and amides, 44,54,59,62
Nonhuman test systems, vi,74 (see also
 individual animals)
Nonmelanoma skin cancer, 7,8 (see also
 Xeroderma pigmentosum)
Nucleotide excision-repair pathway (see also
 Excision-repair pathway)
  in Salmonella, 18,32
  in Xeroderma pigmentosum, 32

Occupational Safety and Health
 Adminstration, 66,67
Oocytes
  chromosome aberrations in, 27
Open-field device
  in behavioral tests,  123
Organ culture, 46,75
Organic compounds
  nephrotoxic, 98,100
Organomercurial fungicides (see Fungicides,
 mercurial)
Organophosphate pesticides
 (see "organophosphate" under
 Pesticides)
Organ transplantation
  and  atherosclerosis, 143,149
Osmunda regalis
  as environmental monitor, 32
Ovigenesis
  as in vitro test, 74
Ozone
  and  lung disease, 175,179
  behavioral effects of,  109,120

PAF (see Platelet activating factor)
PAH (p-aminohippuric acid)
  renal clearance of, 101
  secretion of, 103
  uranium-inhibited transport  of, 98
Parenchyma! lung diseases
  and  inorganic dust inhalation, 191
PBBs (see Polybrominated biphenyls)
PCBs (see Polychlorinated biphenyls)
Pea (see Pisum sativum)
Peanut mold
  as source of aflatoxin, 17
Perfused kidney
  in vitro test, 102
Periarteritis
  and  hepatitis, 161
Perinate
  in behavioral teratology, 122,125
Peripheral artery disease
  and  diabetes, 137
Personal risk factors
  in atherosclerosis, 137-138
Pesticides
  activation of, by  higher plants, 18,23
  and neurological disorders from, 109,115
  infertility associated with, 74
  organophosphate
   as anticholinesterase  agents, 120
   effect of, on electroencephalogram, 114
                                        219

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Phenacetin
  toxic effects of, 97
Phenylbutazone
  and glomerulonephritis, 96
Phenylketonuria, 73
Photoproduct
  pyrimidine dimers, 5
Pink bread mold (see  Neurospora)
Pisum sativum
  and Mendelian genetics, 12
Placenta, 72,74
  chorioallantoic, 81
  passage of carcinogens and teratogens, 81
  species variation in, 74
Plant poisons
  and glomerular lesions, 96
Plants
  in short-term tests, 21,28,29,31-32,33
Plaque formation (see also
 Arteriosclerotic plaques; Atherosclerotic
 plaques)
  and monoclonal  theory, 150-156
Plasma membranes, renal
  subcellular fractionation of, 104
  transport proteins in, 92
Platelet activating factor, 145
Platelet function, and
  atherosclerosis, 143
  immune complex, 146,147
  release of mitogens, 144,147-148,157
Platinum
  nephrotoxic, 98,99
Pneumoconiosis
  and mineral dusts, 179,191
Polybrominated biphenyls
  and behavioral toxicology,  130
Polychlorinated biphenyls, and
  enzyme induction by,  17
  motor  activity, 118
Polycyclic aromatic hydrocarbons
  activation requiring, 17-18
  UV-mjmetic, 5
Polycyclic hydrocarbons, 44,45,59,62,63-66
  effect  on respiratory tract niucosa, 198
Polyethylene glycol
  renal clearance of, 101
Polymorphonuclear leucocytes
  in human pulmonary lavage, 193
Population monitoring (see Human
 population monitoring)
Precarcinogen (see  Procarcinogeh)
Primates, in
  psychophysical testing, 128
  terotogenicity testing,  74
Procarcinogen
  activation of, 41
  chemical exposures, 4
  defined, 13
Promoters
  of carcinogens, 7
  of tumors, 4
Promoting factors
  assays for, 47-48, 49,55
  properties of, 49
Promutagen
  defined,  13
  host-activated,  16
Proximal tubular function
  in vitro tests, 102
  in vivo tests, 101
Proximal tubule (see also Tubular
 [adj]; Tubules)
  anatomy of, 89,91
  and heavy metal exposures, 97-100
  and organic compound exposures, 100
  isolated membranes of, 104
  reabsorption and secretion by,
   91,93,95,100,103
  vulnerability to xenobiotics, 97
Psychophysics, 111-112,128
Public laws and environmental carcinogens,
 66
Pulmonary defense mechanisms
  alveolar macrophages, 196,202
  mucociliary clearance, 201-202
Pulmonary exposure methods,  184
Pulmonary function tests
  forceful exhalation, 186
  for chronic lung disease, 187
  ozone effects on, 179
  predictive,  190
  with guinea pigs, 190
Pulmonary irritants, short-term test
 for, 190
Pulmonary lavage fluids, and
  alveolar macrophages, 193
  detection of edema, 193
  indicators of injury, 195
  protein constituents of, 194
Pulmonary lavage studies
  and cigarette smoking, 193
Pulmonary tumor bioassay,  with
  strain A  mouse, 200
  tracheal transplants,  197-200
Pyrimidine dimers
  formation rate,  5
  UV-induced, 5,14
Quartz
  and  collagen biosynthesis, 197
Quinine
  and  peripheral  vision, 112

Rabbits, 71,73
Radiation exposure
  and  chromosome imbalances, 74
Radiofrequency radiation
  and  blood pressure,  142
Rat cells
  embryonic, for focus  assay, 41,48
  spontaneous transformation of, 39
  virus-infected transformation of, 47
Rat hepatocytes
                                        220

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  and unscheduled DNA synthesis, 27
Rating scales (see also IQ)
  of behavior, 110,126
Rats
  and integrative functions tests, 129
  brain damage in, 131
  for dominant lethal mutation test, 25
  in teratogenic assessment, 71,73
Recessive lethals (see Sex-linked
 recessive mutation test)
Red blood cell hemolysis assays, 195-196
Reflex testing
  reflex modulation, 127,128
  toxicity assessments  by, 111,114-116, 122
Regulatory processes, v-viii
Renal accumulation
  of heavy metals, 94
Renal cellular membrane (see Cellular
 membranes; Plasma membranes, renal)
Renal clearance
  of test substances, 101
Renal function
  assessment of, 100-105
Renal microperfusion
  limitations of, 100,102
  localization of PAH secretion by, 103
Renal micropuncture
  transport function tests, 102
Renal transport
  isolated membrane studies of
    mechanism, 104
  methods of measurement,  100
Renal tubule (see Distal tubule; Proximal
  tubule; Tubular [adj]; Tubules)
Repair-deficient strains, in
  E. coli, 23,24
  Salmonella, 18
Repair levels
  saturation of, 5
Repair pathways (see Excision-repair
  pathway; Nucleotide excision-repair
  pathway)
Repair-proficient strains
  E. coli, 23,24
  Salmonella, 18-19
Repetitive stimulus
  tumorigenesis from, 7
Reproductive behavior,  118,120
Reproductive loss
  incidence of, 69,70
Respiratory rate
  and  industrial chemicals, 190
Respiratory tract carcinogenesis
  and cigarette smoke, 198
Respiratory tract epithelium
 (see Cell culture)
Restrictive lung disease
 (see Chronic restrictive lung disease)
Reverse  mutation  tests with
  fungi and higher plants, 28,29
  Salmonella his mutants, 18,23
Rh factor
  maternal immunization to, 70
Righting reflexes, 114
Risk assessment, vi-viii,3,87,88
  parameters of, 9
  quantitative, 3
  versus benefits, 7
Risk estimation, 27,28-30
Risk factors in atherosclerosis
  cigarette smoking, 158
  diabetes mellitus, 159-160
  diet and hyperlipemia, 136,138,156-157
  hypertension, 158-159
  sex, age and physical activity, 137,160
Rodent liver homogenates (see also Liver
 microsomal homogenates)
  in  vitro activation by,  17
Rodents (see also Hamster cells; Mice;
 Rats),  71
R plasmid
  in  Salmonella, 19
Rubella syndrome
  congenital, 70,73,82

S-9 fraction
  induced, 18
  in vitro metabolic activation by,  17
S-9 homogenates (see Liver microsomal
 homogenates; S-9 fraction)
Saccharomyces cerevisiae
  gene  conversion in, 23,28
  metabolic activation by, 18
Salivary gland cells (see  Cell culture)
Salmonella
  coat-deficient mutants, 18
  excision-repair deficient mutants,  18
  his mutants, 18
  microsome  test, 31,32
  mutagenicity test, 40,44,45,49,50,
   53,59-61,71
  plasmid-bearing mutants, 19
  reverse-mutation test,  18,23
SCE (see Sister chromatid exchange)
Schedules of behavioral  reinforcement,
 121,129
School  performance
  and lead, 125,126
Scintigraphy,  162
Sense organs
  and toxic effects, 111-114
Serotonin
  and liver function, 132
  antigen excess, 145
  effect of DDT and heavy metals  on,  132
Serum cholesterol
  in ischemic  heart disease, 137,141
Serum sickness
  and arterial injury,  145,146
Sex
  as atherosclerotic risk factor, 160
Sex-linked recessive lethal mutation test
                                          221

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  in Drosophila,  12,24,30
Sickle cell anemia, 15
Silica, and
  collagen biosynthesis stimulation, 197
  cytotoxicity,  192,195,196
  interaction with macrophages, 195
Silicosis
  animal models for,  192
  autoimmune  phenomena in, 190
  from dust inhalation,  191
  pathogenesis  of, 192
Silver
  nephrotoxic,  98
Sister chromatid exchange
  as DNA-damage test, 26,27,32
Skin cancer (see also Nonmelanoma skin
 cancer; Xeroderma pigmentosum)
  and UV in  sunlight, 7
Skin sensitivity
  as toxicity assessment test,  111
Smoking (see also Cigarette smokers)
  and early menopause, 81
Smooth muscle cell proliferation
  and mitogens, 148
Sodium chloride
  as teratogen,  71
Soft water
  and Sudden Death, 140
Solvents
  behavioral toxicity of, 108,111,116
  carcinogenicity  of, 44
Somatic cells
  forward mutations in, 24
  short-term tests with, 20,22,29
Species variation, in
  development  of kidney, 106
  drug clearance,  72,78
  maternal P450,  75
  teratogenic response, 71,72
Specific locus test, and
  germ-cell risk estimation, 28-30
  in vivo forward mutations,  24
Spermatogenesis, in
  in vitro tests, 74
  in vivo tests,  25,26-27,30
Spermatoxic agents
  assayed in mammals, 26
Spermatozoa
  in epidemiological studies, vii,74
Sperm YFF test
  for aneuploidy,  26
Spontaneous abortion (see Abortion)
Spontaneous transformation
 (see  Transformation)
Standard setting, vii.viii
Startle response
  toxicity assessment method, 111
Strains, microbial
  development  of, 16-19
Stress
  and coronary heart  disease, 142
  Strokes
    induction by toxins, 11
  Subcellular fractions
    and heavy metals, 95
    in vitro renal  tests, 104
  Subhuman primates (see individual
  animals; Nonhuman test systems;
  Primates)
  Sucrose
    as teratogen, 71
  Sudden Death, and
    cigarette smoking, 137,158
.   diabetes, 138
    water quality, 140
  Sulfhydral groups
    in kidney, 96
  sulfonamides
    and glomerulonephritis, 96
  Sulfuric acid
    in vitro, lung studies for,  197
    mucociliary clearance of,  201
  Sulfur dioxide
    cocarcinogenic in trachea!
     transplants, 199
  Sulfur oxides
    and lung disease, 175
    pulmonary function tests  for, 187,190
  Sunlight
    carcinogenic wavelengths  of, 5,7
  Supermutagens, 13
  Susceptible populations, 32
    sex differences and lung disease, 176
  Swordfish
    methylmercury body burden, 112
  Syrian hamster (see under Hamster cells)

  Tar
    from cigarettes, mutagenic, 31
  Target organ concept, 94-96
  Target tissue
    DNA adduct concentrations  in, 5,6
  Teratogenic agent (see Teratogens)
  Teratogenic outbreak
    epidemic, 82-83
  Teratogenic susceptibility, 71,72,78
    and stage of development, 71
  Teratogens, 77
    defenses against, 78,79
    maternal blood  concentrations of, 71
    selection for testing, 87
    species variation in response, 71
  Test-agent administration
    time periods for, 74
  Testosterone secretion
    cadmium effects on, 118
  Thalidomide, 71,78,83
  Thallium
    nephrotoxic, 98
  Three-tier approach
    for assessing genotoxicity, 30
  Threshold dose, 1,9
                                        222

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Thrombogenesis
  and heart attack,  162
Thyroid tissue
  in organ culture, 75
Toluene
  and behavioral toxicity, 108
Tooth lead
  and teachers' rating scales,  125-126
Toxic Substances Control Act,
 34,87,110
Toxline, 86
Trace metals
  and cardiovascular mortality, 140-141
Tracheal instillation
  pulmonary exposure method, 184
Tracheal rings
  for in vitro lung studies, 197
Tracheal transplants
  in carcinogenesis, 197-200
Tradescantia
  as environmental  monitor, 32,33
Transformation
  of hamster embryo cells, 44,59-61
  spontaneous, 39,42
  virus-induced, enhancement of, 42
Transformed cells
  colony assay, 39,43
  properties of, 39,40,41
Translocation, chromosomal, 15
Translocation, heritable
  in mouse germ cells, 26
  in risk assessment, 30
Transmembrane ion gradients, 100
Tremor
  mercury vapor-induced, 114,115
Triangulation
  in teratology, 81-82
Trichloroethylene
  behavioral toxicity from, 108
Triethyl tin
  behavioral toxicity from, 111
Trisomy
  and  Down's syndrome, 15
Tubular cells
  anatomy of, 96
  ischemic injury to, 97
Tubular filtrate
  antidiuretic hormone effect on, 94,95
  heavy metal impairment by, 98-100
Tubular segments, isolated
  in vitro renal function  test,
  102-103
Tubules (see also Distal tubule;
  Proximal tubule, Tubular [adj])
  reabsorption and secretion by, 92-94
Tumor promoters (see Promoters)
Tuna
  methylmercury body burden, 112
Turner's syndrome
  monosomic condition,  15
Ultimate carcinogen
  defined, 13
Ultimate mutagen
  defined, 13
  formation by host's enzymes, 16
Ultrasonography, 162
Ultraviolet light
  biochemical mutation induction,  13
  skin cancer, 5
Unscheduled  DNA synthesis assay, 27
Uranium
  hemodynamic changes from, 98
  tubular lesions from, 98
Urinary bladder  epithelium
 (see Cell culture)
Urine
  analysis, 100,101
  cigarette smokers, 32
  final composition of, 93,94
  in human population monitoring, 32
  renal function test, 101
Uterine leiomyoma
  and monoclonal hypothesis, 153
UV (see Ultraviolet light)
UV-mimetic chemicals
  polycyclic aromatic hydrocarbons as, 5

Vaccines
  and glomerular lesions, 96
Vaginal carcinoma
  and diethylstilbestrol, 78
Vasectomy
  and atherosclerosis, 148
Vasoactive amines
  and immune complexes, 146
Vibration white finger, 142
Vigilance performance
  carbon monoxide effect on, 109
Virus infections
  and atherosclerosis, 146,151
Vision
  perimetry testing of,  112,113
  psychophysical studies of, 111
Vitamin deficiency
  teratologic  effects of, 71

Water hardness
  and cardiovascular mortality, 140-141
Weight loss, from
  acrylamide and methylmercury
   poisoning, 121
  carbon disulfide, 132
  heavy metals,  132

Xenobiotics, excretion of, 89
Xeroderma pigmentosum
 (see also Nonmelanoma skin cancer)
  autosomal  recessive disease, 32
  repair-deficient condition, 5,7,32
Xeroderma pigmentosum cells
  effect of UV-mimetic chemicals in, 5
                                        223

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X rays
  induction of mutations by,  12,13

Y chromosome
  fluorescence test for aneuploidy, 26

Zinc/copper ratios
  and coronary heart disease, 141
                                      224

                               •ft-U.S.  Government Printing Office:1983--659-095/0596

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