title: Autosomal recessive juvenile cataract in Hutterites.
abstract: Autosomal recessive inheritance of juvenile cataract is described amongst several related sibships of Lehrerleut Hutterites. The main features of the cataract include onset between three and seven years of age; rapid progression to maturity within one to three months; normal intelligence; no systemic associations, and 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.
authors: Department of Ophthalmology, University of Alberta, Edmonton, Canada.
authors: W G
authors: WG
authors: Pearce
authors: J A
authors: JA
authors: Mackay
authors: T M
authors: TM
authors: Holmes
authors: K
authors: K
authors: Morgan
authors: S B
authors: SB
authors: Fowlow
authors: M H
authors: MH
authors: Shokeir
authors: R B
authors: RB
authors: Lowry
mesh_terms: D000416:Alberta; D002386:Cataract / Q000453:epidemiology / Q000235:genetics*; D002648:Child; D002675:Child, Preschool; D003241:Consanguinity; D005260:Female; D005808:Genes, Recessive; D006580:Genetic Carrier Screening / Q000379:methods; D006801:Humans; D008297:Male; D010375:Pedigree; D012067:Religion; D012525:Saskatchewan