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H Dolk

Publications and source records attributed to H Dolk.

At least 55 records · Page 3Linked to original sources

Distribution of single organ malformations in European populations. EUROCAT Working Group.

The aim of this study was to evaluate the prevalence of congenital anomalies in 19 areas from 13 European countries. A total of 291,126 births were surveyed. On average most of the anomalies were recorded in livebirths. The total prevalence of congenital anomalies was 21.8 per 10,000 births. Four groups of congenital anomalies were of particular note in the consideration of geographic variations: neural tube defects, cleft lip, limb reduction defect and Down syndrome. For neural tube defects, 24 cases per 10,000 were recorded in the British Isles whereas only 9.6 cases per 10,000 were registered in Continental Europe. Cleft lip had a much higher prevalence in Groningen (The Netherlands) and in Odense (Denmark) than in the other European countries under study. The prevalence of limb reduction defect was high in Bilbao (Spain), in Odense, in Strasbourg (France) and in Groningen. In the study population the prevalence of Down syndrome ranged from 5.6 to 21.3 per 10,000 livebirths.

Cleft Lip↗

High reported prevalence of congenital anomalies in a Scottish city.

The Glasgow Register of Congenital Anomalies reports the highest birth prevalence of congenital anomalies of all 18 participating centers in the EUROCAT project for the years 1980 to 1988. A high prevalence is found in most defect categories, with Glasgow in the top three places in the EUROCAT "league" in 7 of the 10 categories studied. While the high rate could reflect a high level of ascertainment, we have concluded that there is enough reason to be seriously concerned that the high Glasgow rate is a reflection of risk rather than methodology.

Abnormalities, Multiple↗

The epidemiology of tracheo-oesophageal fistula and oesophageal atresia in Europe. EUROCAT Working Group.

The total prevalence rate of tracheo-oesophageal fistula and oesophageal atresia in 15 EUROCAT registries covering 1,546,889 births during 1980-8 was 2.86 per 10,000. There was a decreasing prevalence rate over time (3.5 per 10,000 in 1980-2, 2.7 in 1983-5, 2.5 in 1986-8). Ten per cent of cases were associated with chromosomal anomalies and of the remaining cases, half were multiply malformed. Sixty two per cent of cases were males. There was a significantly increased risk for mothers of less than 20 years of age (odds ratio compared with mothers of 25-29 = 1.82, 95% confidence interval 1.23 to 2.67). There were no apparent epidemiological differences between isolated and multiply malformed cases in secular trend, sex ratio, or maternal age. Both isolated and multiply malformed cases tended to be premature and small for gestational age. There was variation between centres in survival of affected liveborn children up to 1 year of age.

Diseases in Twins↗

Neural tube defects: a survey of lesion descriptions made by different European pathologists.

Recent epidemiological interest has focused on separation of neural tube defects (NTD) into subgroups which may differ pathogenetically and aetiologically, for example, 'upper' and 'lower' spina bifida. In order to validate the use of pathologists' lesion descriptions by epidemiologists and others, a postal survey of 18 European perinatal pathologists, identified by EUROCAT registries, was conducted. Pathologists were asked, anonymously, to describe and identify the lesions in 15 photographs of midtrimester termination fetuses. There was a 50% response rate. Even taking into consideration the limitations of dealing with photographs rather than the fetuses themselves, there was often marked variation in the descriptions. Standardisation of terminology and international consensus about the type of detail recorded for NTD are urgently needed.

Abortion, Therapeutic↗

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.

Abortion, Induced↗

Heterogeneity of neural tube defects in Europe: the significance of site of defect and presence of other major anomalies in relation to geographic differences in prevalence.

In the period 1980-1987, neural tube defects were two to three times more prevalent in populations covered by EUROCAT registries in the United Kingdom and Ireland (UKI) than in Continental Europe and Malta (CEM). 1864 NTD cases in a total population of 580,000 births in UKI and 455 cases in a population of 380,000 births in CEM were analysed to find if there were differences in the ratio of prevalence rates between UKI and CEM according to site of the defect and association with non-central nervous system (CNS) anomalies. The prevalence rate ratio was high for anencephaly with accompanying spina bifida, iniencephaly, and upper spina bifida, and low for encephalocele, lower spina bifida, and anencephaly without other neural tube defects. There was a greater female excess for anencephaly with accompanying spina bifida, iniencephaly, and upper spina bifida than for other defects in both geographic areas. There was a female excess for encephalocele in UKI but a male excess in CEM. Certain sites (anencephaly with accompanying spina bifida, iniencephaly, and encephalocele) were more likely to have accompanying non-CNS anomalies. The prevalence rate ratio of multiply malformed NTD was in general lower than for isolated NTD but showed the same pattern by site. The prevalence rate ratio was high for multiply malformed anencephaly with accompanying spina bifida, iniencephaly, and upper spina bifida. The sex ratio was similar between isolated and multiply malformed cases when site of the defect is taken into account. It is concluded that the geographic prevalence pattern and sex ratio differ according to site of NTD but do not differ substantially according to whether NTD is isolated or associated with non-CNS anomalies.

Abnormalities, Multiple↗

The predictive value of microcephaly during the first year of life for mental retardation at seven years.

Of 41 term infants surviving to seven years of age with head circumferences (HC) - 3 SD, 21 were mentally retarded (IQ less than or equal to 70) at seven years. HC - 2 SD was associated with mental retardation in 11 per cent of children. Socio-economic status did not affect mental outcome of children with HC - 3 SD, but higher socio-economic level was associated with better outcome for those with HC - 2 SD or who were normocephalic. Mental retardation in children with HC - 3 SD was more common in children with additional pathology. Of 25 children with no other pathology, one-quarter were mentally retarded at seven years; this was more common in children of normal birthweight. Disproportionate head-to-height ratio did not significantly affect outcome, although short stature was associated with less favourable outcome for those with HC - 2 SD or who were normocephalic.

Brain Damage, Chronic↗

[Epidemiological survey of central nervous system anomalies and the implications of teratogens].

One of the aims of epidemiologic surveillance is to assess the role of environmental factors acting during pregnancy (teratogens) in abnormal development of the nervous system. The data of Eurocat, a european system of congenital anomaly surveillance, was analysed and evaluated. Great geographical variation is observed in the prevalence of neural tube defects. Geographical variation in the prevalence of microcephaly, hydrocephaly and holoprosencephaly, may be related to problems of definition, validation and precision of diagnosis. A better definition of the unit of surveillance is required for anomalies where phenotypic heterogeneity is linked to aetiologic heterogeneity (such as neural tube defects) and for anomalies which range from minor to major forms (such as microcephaly). The strengths and deficiencies of the surveillance system for central nervous system anomalies are revealed by its performance in the evaluation of the Chernobyl accident.

Abnormalities, Drug-Induced↗

Incidence of congenital rubella syndrome in 19 regions of Europe in 1980-1986.

Twenty-five cases of congenital rubella syndrome were recorded in 1,458,126 live births in 19 EUROCAT birth defects registries from 1980 to 1986. During the study period, the incidence declined steadily from 3.50 to 0.41 per 100,000 births. Rubella infection occurred in 12 multiparous women indicating failure in immunization programme.

Cross-Cultural Comparison↗

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.

Chromosome Aberrations↗