Publications by authors named "J W Steffelaar"

Objective: Prompted by a British study which revealed a raised frequency of oesophageal cancer as the cause of death among the mentally handicapped, a study was made of the cancer incidence among an institutionalized population with a mental handicap, which incidence was compared with that among the population in general.

Design: Retrospective follow-up study.

Methods: An inventory was made of the cytologically or histologically confirmed cancer diagnoses among persons living in an institute for the mentally handicapped during 1 January 1974 through 1 January 1994 (n = 1020).

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The case history is presented of a 10-year-old boy with a fatal combination of acute appendicitis and infectious mononucleosis, in the literature a particularly rare combination. The boy died of a perforative peritonitis. His appendicitis appeared not to be a complication of the infectious mononucleosis.

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Three stillborn fetuses are reported in which an abdominal wall defect was associated with defects in the urogenital and anal region. Autopsy of these fetuses provided clues indicative of how and where the embryonic development went wrong. The malformation involved a disturbance of the cell deposition process, occurring in the caudal part of the embryo.

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An 18-year-old man visited the outpatient cardiology clinic because of a cardiac murmur found at a medical examination for military service. An Ebstein's anomaly was diagnosed, with tricuspid valve insufficiency. Exposure to lithium in utero was established.

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Since a population-based registry of the mentally retarded is lacking in The Netherlands, data concerning incidence and life expectancy patients with Down's syndrome (DS) are not available. Based on birth statistics, in combination with risk factors for DS in relation to maternal age on the one hand and on studies on life expectancy in DS from Denmark, British Columbia and Western Australia on the other, the numbers of these patients older than 40 years were estimated for The Netherlands for the period 1990-2025. After a considerable increase of the number of institutionalized DS patients of 40 years and older between 1958 and 1985, the present estimates indicate a further rise.

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