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Severity of retardation and nature of medical history significantly affected recurrence risk, while sex of propositus and sibs and presence or absence of epilepsy, cerebral palsy, microcephaly, and short stature did not. Segregation rations were 0.095 in cases of severe retardation with normal medical history, 0.216 for mild retardation with normal medical history, 0.230 in cases of all retardation with maternal reproductive inefficiency, and 0.110 for all retardation with other reported prenatal, perinatal, or infantile complications. Estimated recurrence risks in most simplex families dropped sharply with each additional normal child. The recurrence risks are higher than some previously published estimates. Different ascertainment criteria may be responsible for this variation. The criteria used here were compatible with a 3.2% population prevalence of mental retardation with a 0.223 segregation ratio in multiplex sibships. It is recommended that future studies of recurrence risks include similar data permitting evaluation of sensitivity of ascertainment criteria.<\/jats:p>","DOI":"10.1002\/ajmg.1320270403","type":"journal-article","created":{"date-parts":[[2005,6,12]],"date-time":"2005-06-12T02:58:55Z","timestamp":1118545135000},"page":"753-766","source":"Crossref","is-referenced-by-count":9,"title":["The risk of having a second retarded child"],"prefix":"10.1002","volume":"27","author":[{"given":"Hanan","family":"Costeff","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Leonard","family":"Weller","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"John M.","family":"Opitz","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"James F.","family":"Reynolds","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2005,6,9]]},"reference":[{"key":"e_1_2_1_2_1","first-page":"44","volume-title":"Epidemiology and Genetics of Mental Deficiency in a Southern Swedish Popula\u2010tion","author":"Akesson HO","year":"1961"},{"key":"e_1_2_1_3_1","first-page":"173","article-title":"Genetic prognosis in severe mental defect","volume":"19","author":"Angeli E","year":"1975","journal-title":"J Ment Defic Res"},{"key":"e_1_2_1_4_1","first-page":"323","article-title":"Mental retardation in over half\u2010a\u2010million successive livebirths: An epidemiological study","volume":"89","author":"Baird PA","year":"1985","journal-title":"Am J Ment Defic"},{"key":"e_1_2_1_5_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1469-8749.1986.tb03861.x"},{"key":"e_1_2_1_6_1","first-page":"115","article-title":"Recurrence risks in severe undiagnosed mental deficiency","volume":"18","author":"Bundey S","year":"1974","journal-title":"J Ment Defic Res"},{"key":"e_1_2_1_7_1","first-page":"339","article-title":"Consanguinity analysis in Israeli mental retardates","volume":"29","author":"Costeff H","year":"1977","journal-title":"Am J Hum Genet"},{"key":"e_1_2_1_8_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1469-1809.1983.tb00973.x"},{"key":"e_1_2_1_9_1","first-page":"237","article-title":"Recessive genes in severe mental defect","volume":"17","author":"Dewey WJ","year":"1965","journal-title":"Am J Hum Genet"},{"key":"e_1_2_1_10_1","doi-asserted-by":"publisher","DOI":"10.1007\/BF00442637"},{"key":"e_1_2_1_11_1","doi-asserted-by":"publisher","DOI":"10.1002\/ajmg.1320070407"},{"key":"e_1_2_1_12_1","doi-asserted-by":"publisher","DOI":"10.1002\/ajmg.1320130210"},{"key":"e_1_2_1_13_1","first-page":"1","article-title":"Genetic tests under incomplete ascertainment","volume":"11","author":"Morton NE","year":"1959","journal-title":"Am J Hum Genet"},{"key":"e_1_2_1_14_1","doi-asserted-by":"publisher","DOI":"10.1542\/peds.41.1.106"},{"key":"e_1_2_1_15_1","first-page":"42","volume-title":"Textbook of Pediatrics","author":"Nelson WE","year":"1969"},{"key":"e_1_2_1_16_1","series-title":"Birth Defects Original Article Series, Volume XIV, Number 6B","first-page":"1","volume-title":"Diagnostic genetic studies in severe mental retardation","author":"Opitz JM","year":"1978"},{"key":"e_1_2_1_17_1","first-page":"120","volume-title":"The Biology of Mental Defect","author":"Penrose LS","year":"1972"},{"key":"e_1_2_1_18_1","unstructured":"PenroseLS(1938):\u201cA Clinical and Genetic Study of 1280 Cases of Mental Defect\u201d. 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