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One hundred and forty\u2010six children (87 boys and 59 girls) were ascertained, out of a total population of 46,960, with a prevalence of 1 in 320 or 3.1 per 1,000. Approximately 1\/3 (47) had the Down syndrome, 1 per 1,000 population.<\/jats:p><jats:p>It was possible to establish a diagnosis in a further 45 cases, which included 1 additional case of autosomal chromosome abnormality and 7 each of autosomal dominant, recessive and X\u2010linked conditions; 17 were associated with presumed multifactorial etiological factors; in 6 the condition was thought to have been caused by an environmental agent. It was not possible to establish a cause in the remaining 54 cases.<\/jats:p><jats:p>Recurrence risks of severe mental retardation in cases where it is impossible to establish a definite diagnosis are discussed and the potential value, for genetic counseling purposes, of categorizing such patients into broad symptomatological groups, is suggested.<\/jats:p>","DOI":"10.1002\/ajmg.1320010109","type":"journal-article","created":{"date-parts":[[2005,6,11]],"date-time":"2005-06-11T13:20:08Z","timestamp":1118496008000},"page":"75-86","source":"Crossref","is-referenced-by-count":58,"title":["An etiological survey of the severely retarded Hertfordshire children who were born between January 1, 1965 and December 31, 1967"],"prefix":"10.1002","volume":"1","author":[{"given":"Renata","family":"Laxova","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"M. A. 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