FAMILIAL TRUE HERMAPHRODISM IN PRE- AND POSTPUBERAL GENETIC FEMALES. HORMONAL AND MORPHOLOGIC STUDIES

Abstract

The clinical findings, urinary hormonal patterns and gonadal morphology in 2 genetic female siblings with scrotal testes and ovaries are presented. As far as is known, this is the first reported instance of familial true hermaphrodism. The testes of the postpuberal sibling showed seminiferous tubule dysgenesis which was identical to that found in Klinefelter''s syndrome, whereas the testes of the pre-puberal sibling were histologically normal. These findings are p articu-larly significant in the natural history of seminiferous tubule dysgenesis.