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The purpose of this study, through a retrospective epidemiological survey carried out over three geographical areas in France, was to characterize the aetiological factors involved in severe mental retardation (SMR) within a geographically defined population of children with disabilities aged between 7 and 16 years. The inclusion criteria for SMR (IQ

Resume Les textes reglementaires portant sur la prise en charge des enfants handicapes ont defini en France la notion de polyhandicap. Mais il existe des divergences dans les modalites d'application de cette definition ainsi que des differences de prevalence. Cette etude a pour objectif de contribuer a eclaircir ces deux points. Population et methodes L'enquete porte sur les enfants deficients pris en charge par les commissions departementales de l'education speciale (CDES) et les hopitaux de jour, dans trois departements. Ces enfants etaient nes entre 1976 et 1985. Les informations ont ete relevees dans les fiches medicales et transcrites sur un dossier informatisable par un medecin enqueteur. Resultats Les resultats montrent que les enfants polyhandicapes constituent un ensemble heterogene. La definition la plus restrictive (deficience motrice avec deficience intellectuelle profonde, etat grabataire ou passif dans un fauteuil) conduit a une prevalence de 0,73 ‰. Une definition plus large reposant sur la notion d'autonomie nulle, mais excluant les retards mentaux legers ou moyens conduit a une prevalence de 1,28 ‰. Conclusion faut insister sur la necessite de preciser les objectifs d'une definition prise a des fins operationnelles afin d'ameliorer l'estimation des besoins.

Severe and profound hearing loss (> 70 dB) were analysed in a retrospective study of 226 children, born between 1976 and 1985, and recruited from three French administrative departments. The prevalence was 0.54 per 1000 children under 9 years old, with no decrease over the study period. A hereditary origin was identified in 20.8% of cases and an infectious origin in 11.5%. Perinatal risk factors were present in 11.5%, while the aetiology was undetermined in more than half the cases. In 85.8% of the children there was no other severe impairment. Marked learning difficulties were observed: 36% of the children were two years behind their age group and 28% were more than two years behind. The age of initial care decreased over the study period but is still too advanced. Systematic neonatal screening would enable earlier care, which should limit the social and educational impact of hearing loss.

Actual data on the frequency of cerebral palsy (CP) and "infirmite motrice cerebrale" (IMC), and their relationship with perinatal asphyxia and perinatal managements, are presented. In France, the frequency of IMC at 9 years of age, approximates 1 per thousand, for the 1972, 1976, 1981 generations. Three surveys, two English and one Australian, show an association between perinatal asphyxia and CP. However computation of percent attributable risk indicates that asphyxia can explain only one case of CP out of six among term neonates. These surveys show also that 10% of CP only could be prevented by improving perinatal managements. This, in addition to other factors such as the increase in survival of very preterm babies, explains the absence of a significant reduction of CP frequency despite improvements in the perinatal care.

Resume Dans le cadre de la recherche epidemiologique sur les deficiences motrices de l'enfant en France, il devient necessaire de faire la part entre le terme francais infirmite motrice cerebrale (IMC) et le terme anglais cerebral palsy (CP). Les differences entre ces deux entites sont precisees a partir d'exemples cliniques. Les avantages et les inconvenients de l'utilisation de chacun de ces termes sont discutes. L'IMC represente un groupe plus homogene que la CP, mais il peut etre soumis a des variations difficilement controlables. Le terme CP permet de meilleures exhaustivite et reproductibilite que le terme IMC. Lors d'une enquete epidemiologique menee recemment sur les deficiences de l'enfant en France, nous avons analyse les deficiences motrices sous l'angle de chacun de ces termes. Les taux de prevalence de CP trouves dans cette etude (1,9‰) sont tres proches de ceux des etudes anglo-saxonnes. Pour que des recherches etiologiques sur les deficiences motrices de l'enfant soient possibles, il est souvent necessaire de les realiser a un niveau international, d'ou l'importance de travailler sur des entites cliniques superposables.

Les deficiences motrices d'origine cerebrale ont fait l'objet depuis une dizaine d'annees de recherches epidemiologiques relativement nombreuses qui ont permis de mieux cerner leur etiologie. Toutefois, peu d'entre elles portent sur le nouveau-ne a terme. Or, c'est pour ces enfants que se pose essentiellement le probleme actuellement tres controverse du role de l'anoxie et de sa prise en charge. Dans un premier temps, nous examinerons les problemes poses par la definition de ces deficiences et les principales etudes disponibles. Seront ensuite envisages les facteurs de risque prenatals et ceux de la periode entourant l'accouchement.

A systematic registration was carried out in 1985-1986 and 1989 in 14 French 'departments' in order to assess whether the prevalence rates of different components of motor disability (MD) in three different birth cohorts (1972, 1976 and 1981) had changed at a time when the preterm birth rate and neonatal mortality were decreasing and there was evidence of changing perinatal practice. A total of 1355 MD were registered amongst resident children born in 1972, 1976 and 1981 with a prevalence of 3.34 per 1000. The prevalence of the MD types due to different causes did not differ significantly amongst the three birth cohorts with the exception of an excess of hereditary and degenerative disease of the central nervous system (CNS) among children born in 1981. The prevalence of cerebral palsy (CP) remained stable in the three birth cohorts: it was 1.30, 1.06 and 1.08 per 1000 respectively, for children born in 1972, 1976 and 1981. The prevalence of pre- or perinatal-origin of other motor disabilities (OMD) and of CNS malformations did not differ amongst the three birth cohorts. The method of registration is discussed and the results are related to those of the French perinatal surveys performed in 1972, 1976 and 1981, which showed a decrease in preterm birthrate, an increase in perinatal care and a decrease in the mortality rate of high-risk infants.

The relationship between the risk of breast cancer and oral contraceptive use was investigated in a case-control study conducted in France between 1983 and 1987 in five public hospitals. Some 464 cases aged 25 to 56 years and 542 matched controls were interviewed about their history of the use of oral contraceptives (OC). Results are given for the entire population and for the subgroup of 358 and 379 premenopausal cases and controls. The multivariate relative risk estimate, for ever user, was 1.5 (p less than 0.01) in the whole group as well as in the premenopausal subgroup (p less than 0.02). However, there was no evidence that the effect varied appreciably according to duration of use, age at first use, use before first full-term pregnancy (FFTP) and time since first or last use. The risk was not altered for any particular brand of OC. We conclude that, because of the widespread attention given to the relationship between OC use and breast cancer, information bias might be responsible for part of the excess in risk observed among OC ever users.The relationship between the risk of breast cancer and oral contraceptive (OC) use was investigated in a case-control study conducted in France between 1983-87 in 5 public hospitals. Some 464 cases ages 25-56 years and 542 matched controls were interviewed about their history of OC use. Results are provided for the entire population and for the subgroups of 358 and 379 premenopausal cases and controls. The multivariate relative risk estimate, for ever-use, was 1.5 (p0.01) in the entire group as well as in the premenopausal subgroup (p0.02). However, there was no evidence that the effect varied appreciably according to the duration of use, age at 1st use, use prior to 1st fullterm pregnancy, and time since 1st or last use. The risk was not altered for any particular OC brand. The authors conclude that because of the widespread attention given to the relationship between OC use and breast cancer, information bias might be responsible for a part of the excess of risk observed among OC ever-users.

To evaluate the effects of gestational hypertension on fetal growth, we studied the standardized records of 2996 single live-birth pregnancies. Mothers all had documented diastolic blood pressure of less than 85 mmHg before the 16th wk of amenorrhea and no history of pre-pregnancy hypertension or kidney disease. Diastolic blood pressure readings exceeding 84 mmHg were found later in pregnancy in 38.4% of the mothers, and were associated with an increased number of small-for gestational-age infants: 3.2% in mothers whose diastolic blood pressure had never reached 85 mmHg, 6.3% when peak diastolic blood pressure had been in the 85-94 mmHg range, and 8.5% when it had exceeded 94 mmHg (p less than 0.01). In mothers who had had one or more diastolic readings of more than 84 mmHg, and for all peak diastolic pressures, the rate of small-for-gestational-age infants was higher when hypertension had begun early in third trimester (between the 27th and 36th wk), than in the second trimester or later than the 35th wk (10.2% compared to 5.6 and 6.1% respectively, p = 0.02). This temporal reinforcement of the adverse fetal effects of hypertension when it began in the early third trimester was not explained by differences in the incidence of proteinuria or in maternal age, parity, obstetric history or smoking habits.

The performance of 2 maternity units is compared. In comparable groups of average risk the prematurity rate was significantly lower in the unit practising intensive supervision during pregnancy.

The efficacy of caproate of hydroxyprogesterone and acetate of chlormadinone in preventing premature labore was compared in a controlled trial. The survey was based on 211 pregnant women with a high risk of premature delivery discovered during clinical examination. There are no significant differences between the two groups in either length of gestation, delay between the beginning of treatment and delivery or other parameters related to prematurity. The absence of evidence suggesting any significant difference between the two treatments can have three possible causes (which are discussed): the methodology, the inefficacy of the two products or the equivalent efficacy of the two products.

In France, between 1976 and 1981, the use of ultrasound examination during pregnancy increased from 11.3 to 81.8%. In spite of this dramatic increase, many inequalities observed in 1976 still exist in 1981. Despite a decrease in 1981 of the differences in practice in public maternity units and private care units, large differences remained between general practitioners and specialists. In a logistic regression, the type of practitioner responsible for care appeared to be the most important factor determining access to ultrasound examination. Independent of other inequalities in the type of antenatal care, three groups of women remained disadvantaged in 1981: women under 20 years old, grand multiparas and women of low educational level. The latter group was particularly disadvantaged. Obstetric pathology has little influence on the overall distribution of ultrasound examinations.

L'hypotrophie fœtale, temoin d'une souffrance in utero, semble liee a certains retards du developpement ponderal, statural ou psychomoteur de l'individu, et regroupe, a ce titre, des enfants a risque eleve.

A prospective survey was carried out by the French National Institute of Health and Medical Research (INSERM) in 12 University hospitals in Paris and included 12,764 women; 189 gave birth to babies with unequivocal, non-chromosomally based, malformations. There was a significant excess of malformed infants when women had taken phenothiazines during the first three months after the last menstrual period. This association did not seem due to chance or to bias and could not be explained by the influence of the outcome of previous pregnancies. These data are to be added to results of recent surveys showing a significant increase of malformations in children exposed in utero to anticonvulsants, carbamates, benzodiazepines, tricyclic antidepressants, and amphetamines.

I.N.S.E.R.M. has carried out two prospective inquiries centred on the evaluation of the teratogenic action of drugs on human beings. The first survey was between 1963 and 1969 and the second between 1975 and 1977 and they were carried out in maternity hospitals. The first test, which was carried out on 12,764 women, had shown no overall association between the use of hormonal tests for the diagnosis of pregnancy (oestrogens-progestogens) and congenital malformation in the infant. The second study, which was carried out on 3,451 women who were questioned in the same way, shows that there is an excessive number of newborn babies with malformations when the mothers took certain products (oestrogens-progestogens when the progestogen was a derivative of testosterone). The problem of a methodological bias is discussed and the results are interpreted in the light of epidemiological studies that have recently been carried out abroad.

This study concerns women followed from the first trimester of pregnancy, in a university clinic in Paris in 1977, 1979 and 1981. The cesarean section rate was separately analysed for primiparas, and multiparas with and without previous cesarean section. The overall rate of cesarean section was 11.4% in 1977, 17.2% in 1979 and 21.1% in 1981. The 9.6% increase observed between 1977 and 1981 is mainly attributable to an increase in primary cesarean section, particularly among primiparas, between 1977 and 1979. This does not seem to have arisen from sample variations. There is a change in obstetrical attitude and more cesarean sections are performed in cases of hypertension, breech presentation or intrauterine growth retardation. The greater number of previously sectioned women explains the increase in the rate between 1979 and 1981. The main fact among primiparas is the 18.8% increase in diagnosis of dynamic dystocia between 1979 and 1981. Three key areas allow us to envisage a reduction in cesarean section rate: obstetrical attitude towards previous cesarean section, breech presentation, and management of labor, whose perturbations lead to diagnosis of dynamic dystocia.

The study of a national sample of births showed that, in 1976, more than 50% of French women worked during pregnancy. Working during pregnancy was, on the whole, associated with better antenatal care, and better outcome of pregnancy. This relationship remained after taking into account the more favourable social characteristics of working women. Nonetheless, physically hard working conditions were associated with a high risk of preterm delivery.

\ The detection of high-risk pregnancies has become one of the major concerns of physicians and midwives in charge of pregnant women. Detection must be made very early, and it would be highly desirable for certain factors of risk to be discovered before the beginning of pregnancy, and even before any pregnancy in any given woman. In the work reported here, our objective was to predict perinatal mortality during the first three months of pregnancy in order to discover the groups at risk requiring particular care. Predictions will be based not only on pathological findings, but also on characteristics, the importance of which is sometimes less well known, such as age, social and economic Status, and circumstances of life during pregnancy. On the basis of an analytical study of these factors, an attempt at predicting stillbirth and neonatal death is made.