Autosomal recessive juvenile cataract in Hutterites
- 1 January 1987
- journal article
- research article
- Published by Taylor & Francis in Ophthalmic Paediatrics and Genetics
- Vol. 8 (2) , 119-124
- https://doi.org/10.3109/13816818709028527
Abstract
Autosomal recessive inheritance of juvenile cataract is described amongst several related sibships of Lehrerleut Hutterites. The main features of the cataract include (1) onset between three and seven years of age; (2) rapid progression to maturity within one to three months; (3) normal intelligence; (4) no systemfc associations, and (5) no urinary reducing substances and normal erythrocyte galactokinase activity. Genetic analysis demonstrates the close relationship between parents of affected sibships with a coefficient of inbreeding of affected sibships of 0.0512. Estimates of heterozygote frequency within Lehrerleut Hutterites at 0.128 indicate that if current inbreeding practice continues additional cases can be expected.Keywords
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