5 alpha-reductase deficiency without hypospadias.

Ng, W K; Taylor, N F; Hughes, I A; Taylor, J; Ransley, P G; Grant, D B · Arch Dis Child · 1990

case_report · Level V

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Abstract

A boy aged 4 with penoscrotal hypospadias and his brother aged 12 with micropenis had typical changes of homozygous 5 alpha-reductase deficiency. After three injections of chorionic gonadotrophin there was a trivial rise in plasma dihydrotestosterone with a normal increase in plasma testosterone. Urine steroid chromatography showed abnormally high 5 beta: 5 alpha ratios and 5 alpha-reductase activity was appreciably reduced in genital skin fibroblasts. The results indicate that 5 alpha-reductase deficiency is not invariably associated with genital ambiguity.

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