Artigo Revisado por pares

Inheritance of Fuchs' Combined Dystrophy

1979; Elsevier BV; Volume: 86; Issue: 10 Linguagem: Inglês

10.1016/s0161-6420(79)35340-4

ISSN

1549-4713

Autores

Malcolm Magovern, George R. Beauchamp, John W. McTigue, Ben S. Fine, Robert C. Baumiller,

Tópico(s)

Corneal Surgery and Treatments

Resumo

The inheritance pattern of Fuchs' combined corneal dystrophy is not confirmed. Published pedigrees fail to demonstrate a 50% segregation and sex ratio. They include no more than two generations of affected individuals and indicate a strong, female predilection. The pedigree we will present shows 16 affected persons in four generations. The ratio of affected to unaffected and men to women is 1:1. Penetrance is apparently 100%. Nine of the affected are under 50 years of age; four are subteen age. Light and electron micrographs of corneal tissue from three patients in three different generations are consistent with the diagnosis of Fuchs' dystrophy. Fuchs' dystrophy can therefore be established as a classic autosomal dominant pattern.

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