Registries of congenital anomalies: EUROCAT.

Congenital anomalies are one of the potential adverse effects of the environment on reproductive health. Registries of congenital anomalies are useful to detect abnormal frequencies, clusters, and trends. Such registries should meet a number of conditions, including an appropriate population denominator, an efficient system for collecting information, standardized diagnostic procedures, postmortem examinations of still-births, and linkage of records. The EUROCAT (European Registration of Congenital Anomalies and Twins) program is a Concerted Action of the Commission of the European Communities initiated in 1979. One of its objectives is the surveillance of congenital anomalies as related to environmental hazards. This surveillance system covers at present 350,000 births per year in 15 countries. A number of problems encountered in the development of EUROCAT and in the course of ongoing activities are reviewed: populations coverage, classification of malformations, coding, definition and coverage of late fetal death, registration of induced abortion, validation of diagnostic information, registration of late diagnosed cases, and maintenance of motivation in data collection. The issue of confidentiality and the need for strict safeguards for the protection of individual privacy are emphasized.

Evaluation of information systems for the surveillance of congenital malformations.

Many systems for the recording of congenital malformations in infants have been developed following the thalidomide epidemic of 1958 to 1962. Systems established for monitoring increases in rates of malformations have been of three main types: (1) prospective recording of information about all pregnancies; (2) the recording of malformations observed at birth; (3) the registration of children found to be malformed at birth or at any time after birth. The latter type involves many sources of information. International collaboration in monitoring has led to the establishment of the International Clearinghouse for Birth Defects Monitoring Systems and the EEC Concerted Action Project EUROCAT. The advantages of these two projects are discussed. It is shown that results from population based registers such as are included in EUROCAT can be used to validate the results of systems which carry out monitoring at birth.

The 1980 earthquake in Southern Italy--morbidity and mortality.

The effects on health of the 1980 earthquake in southern Italy were surveyed retrospectively. The sample population includes 3619 people living in seven villages situated near the epicentre. Deaths were one hundred times and injury rates more than five times higher in trapped than in non-trapped victims. The possibility for escape was crucial for survival and depended on the type of building. Most of the rescue and relief work was carried out within a few days by unprepared local people who concentrated assistance on people sharing the same dwelling. The results suggest that the emergency phase for medical care was limited to the three to four days after impact. During the 18 months following the quake, mortality rates in injured (13.7%) and non-injured victims (15.8%) were similar. These results point to the need to establish, in each disaster prone area, a health evaluation system on which effective disaster relief and especially the preparedness of the community can be based.

Frequency of births with potentially avoidable serious chromosomal anomalies in EEC countries, 1979-1982.

Child bearing at an early age and prenatal cytogenetic diagnosis in pregnant women of advanced age, combined with selective abortion, make it possible to avoid the birth of many children with serious chromosomal anomalies. To see how many of such births were still avoidable in Europe, data from 16 regional EUROCAT registers of congenital anomalies in nine EEC countries were analysed. In the period 1979-1982 about 30% of children with unbalanced anomalies of autosomes were born (live- and still-births) to mothers over 35 years of age. This amounts to an estimated 1300 cases yearly in the entire population of the nine countries. The approach shows the possible use of registry data for monitoring effects of avoidance strategies.

Mortality in meningococcal disease in Belgium.

A survey of children admitted with meningococcal disease to 53 paediatric units in Belgium between 1975 and 1979 was made in order to assess the case mortality rate (CMR) and to identify risk factors associated with death. A total of 309 cases (226 bacteriologically confirmed and 83 unconfirmed) was recorded. The overall CMR was 6.1 per cent. It was 4.4 for bacteriologically confirmed cases and 10.8 for unconfirmed cases. The CMR was higher for septicaemia without meningitis (22.2 per cent) than for meningitis with or without signs of septicaemia (3.4 per cent). The risk of death was not related to the sex or nationality of the patients. Age was a major determinant of the CMR, independently of the clinical picture. The highest risk of death was in children under one year of age. Poor socio-economic conditions were a significant risk factor. Failure to recognise the severity of the disease by some poorly educated mothers, and the admission of the patient to a hospital lacking adequate facilities for managing severely affected children, were the two significant causes of delay of adequate treatment.

Meningococcal disease in Belgium. Secondary attack rate among household, day-care nursery and pre-elementary school contacts.

During a recrudescence of meningococcal disease mainly due to serogroup B, 1913 notified cases were investigated in Belgium from 1971 to 1976. From 1971 to 1973, 76 secondary cases were reported out of a total of 1455 cases (5.2 per cent); and from 1974 to 1976, nine secondary cases were reported of a total of 458 cases (2.0 per cent). Seventy per cent of the secondary cases occurred within seven days after the Index case. The 4.7 per cent fatality rate among secondary cases was lower than the 9.8 per cent fatality rate among primary cases. The estimated secondary attack rate was 685 per 100 000 among household contacts, 404 per 100 000 among day-care nursery contacts and 77 per 100 000 among pre-elementary school contacts. These attack rates were significantly higher (P less than 0.001) than the incidence rates in the corresponding age-groups in the general community, indicating the need for prophylaxis in these contacts.

Longitudinal study of asymptomatic meningococcal carriage in two Belgian populations of schoolchildren.

In Brussels, a 15-month longitudinal survey was conducted in two primary schools, from March 1975 to May 1976, in order to analyse the dynamic of asymptomatic meningococcal carriage, during an epidemic mainly caused by serogroup B, serotype 2 Neisseria meningitidis. In the first school, which is situated in a suburban area with upper-middle socio-economic status of residents, a mean prevalence of carriers of 10 per cent, an acquisition rate of eight per 1000 months, and a mean duration of carriage of 12.4 months were observed among 158 schoolchildren aged six to 11 years old. In the second school, which is situated in a densely populated area with low socio-economic status of residents, a mean prevalence of carriers of 33 per cent, an acquisition rate of 28 per 1000 months, and a mean duration of carriage of 11.7 months were observed among 203 schoolchildren aged three to 14 years old. For both schools, the median duration of carriage was estimated at 9.4 months. The differences of prevalence and incidence of acquisition between the two schools cannot be explained by age, sex or ethnic factors and are probably related to socio familial variables. The theoretical relationship between prevalence, incidence and duration of meningococcal carriage was for the first time demonstrated in this study. The results also suggest that populations of low socio-economic status and living in densely populated areas constitute a target population for meningococcal disease prevention.

Validity of the recording of meningococcal disease according to various sources of information.

A study was made in Belgium in order to assess the completeness and specificity of the recording of meningococcal disease by routine sources of information. Ninety-three cases identified in a hospital survey were linked with those recorded in mortality statistics, in the notification of communicable diseases, and by the National Reference Laboratory for meningococci. Statistics based on mortality data appeared to be of low validity. The overall completeness of recording was 44% for the notification of communicable disease, and 40% for the reference laboratory. When these two sources were used for surveillance, the completeness of case-finding increased to 56%. When the analysis was restricted to bacteriologically-confirmed cases, the completeness of recording was 62% for the notification system, 70% for the laboratory, and 84% for both sources. The surveillance of communicable diseases should rely on various sources of information. Laboratory data should be systematically used in order to improve both the completeness of recording and the specificity of case-ascertainment.

Leprosy in Portugal 1946-80: epidemiologic patterns observed during declining incidence rates.

Compulsory notification of leprosy in Portugal formed the basis for the establishment of a national patient registry used in an epidemiological study. Highest incidence rates were observed in the coastal counties in the middle of Portugal and particularly in the municipalities with a high annual rainfall. Peak incidence rates in males was observed at the age of 25-29 years against 50-59 in females. A continuous and increasing decline in incidence rates was observed throughout the observation period, 1946-80. Towards the end of the period the slopes of the incidence curves seemed to be identical with those observed in other countries where leprosy has previously been eradicated. This is consistent with the notion that towards the end of an endemic situation no new transmission of the disease occurs, and the incidence curve takes the shape of the right part of the distribution of incubation periods which apparently is uniform in leprosy, irrespective of time and place. The pattern observed in other areas during declining incidence rates, of an increase in age at onset by year of onset together with a lack of increase in age at onset by year of birth was confirmed by the Portuguese data, also consistent with a break in the transmission of the disease a long time before the final termination of the endemic situation.

Smoking patterns during and before pregnancy: weight, length and head circumference of progeny.

This study indicates that tobacco smoking by the mother is associated with a significant reduction of the newborn's size. This effect cannot be explained by either an association of the smoking habit with malformation, premature birth, exaggerated consumption of coffee matrimonial status or paternal smoking, or by a combination of malformation, prematurity and any one of the other factors. The effect is also unrelated to the continuation or discontinuation of smoking during pregnancy. Paternal smoking seems to be associated with a reduced weight in children born to nonsmoking mothers.

PIP: Between 1972 and 1974 202 cases of congenital malformation were reported among 17,970 live births in different hospitals. 175 control were compared with the group of malformed children for such factors as smoking habits of parents prior and during gestation, coffee consumption, social characteristics, and birth weight, height and head circumference of children. Aim of the study was to investigate the possible association among these factors. Tobacco smoking was not associated with the presence of malformations, but with a significant reduction in newborn size. The average birth weight of chidren born to nonsmokers was 3250 g., as compared to 3028 g. for children born to smokers. The height difference was 1.30 cm., and the head circumference difference 0.93 cm., all significant differences. This effect cannot be explained by an association of the smoking habit with malformation, premature birth, consumption of coffee, marital status or paternal smoking, or by a combination of malformation, prematurity, or any of the other factors. none of these modifications are significant. The effect of coffee on size deserves further investigation, as it does the possible association between paternal smoking and a reduction in weight of children born to nonsmoking mothers.

[Priorities in health activities in refugee camps (author's transl)]. / Les activités de santé prioritaries dans les camps de réfugiés.

In times of natural or man-made disasters, settlements of refugees in temporary camps face the health authorities with new public health problems. The allocation of priorities and resources among Medical Care, Prevention and Sanitation, and Nutrition are usually not based on objective assessment of the situation. The potential role of epidemiological techniques and particularly of the surveillance is illustrated by the case studies of the Bengladesh civil war and the Niger drought. A rational priority order will usually rank nutrition at the top and individual medical care at the bottom.

[Attitude toward the risk of epidemics during sudden disasters (author's transl)]. / Attitude face au risque d'épidémie lors de désastres soudains.

Following sudden disasters, major outbreaks are rare unless malnutrition and starvation are common. No dramatic increase of water borne diseases has been reported and adequately documented in the aftermath of the earthquakes, cyclones or floods of this decade. However, the attitude of the public, the mass media and of the health services is sometimes irrational leading to impressive but ineffective mass immunizations. An epidemiologic system and accurate information on the actual situation are essential in case of major disasters.

[Epidemiologic surveillance of congenital abnormalities using the EUROCAT Register]. / La surveillance épidémiologique des anomalies congénitales par le registre EUROCAT.

The EUROCAT programme is a concerted action of the European Economic Community for the epidemiologic surveillance of congenital anomalies. Surveillance is based on a network of regional registries coordinated by a central registry. The programme started in 1979 and by 1987, 23 centres were participating, covering together more than 300,000 births per year. The surveillance process implies the selection of the anomalies possibly associated with environmental teratogens or mutagens, the definition of abnormal variations in the rate of these anomalies (the alarms), the establishment of base-line rate and the continuous monitoring of rate. The programme is evaluated by reference to four criteria: the sensitivity of the system in detecting teratogens or mutagens, the specificity of alarms, the rapidity in warning of alarm and in investigating their causes, and the programme efficiency expressed as its cost-utility ratio.

[Definition and classification of perinatal mortality]. / Problèmes de définition et de classification des décès périnatals.

Analysis of problems related to the classification of perinatal mortality was made possible through the evaluation of data collected from the medical records of nine maternity hospitals in South-Hainaut. Medical records of 135 fetal and early neonatal deaths were investigated. Perinatal mortality statistics were compiled on the basis of five different definitions of perinatal mortality. Depending on which definition was used, perinatal mortality varied between 10.2% and 15.1%. This study shows that reporting of perinatal mortality in hospital registries according to the legal requirement is incomplete. Standard data should be collected for each pregnancy product, on the basis of clearly defined, national and international accepted definitions. It is suggested that the 1975 recommendations of the World Health Organization (International Classification of Diseases, 9th edition), be used for definition and classification of perinatal mortality.