Paternity by Intrauterine Insemination with Sperm from a Man with 5α-Reductase-2 Deficiency

Abstract

Male pseudohermaphroditism results from the abnormal differentiation of male external genitalia in a genotypic male. One cause of male pseudohermaphroditism is a deficiency of 5α-reductase-2, the enzyme that converts testosterone to 5α-dihydrotestosterone in specific androgen-dependent target tissues.1–3 Males who are homozygous for the disorder usually present with pseudovaginal perineoscrotal hypospadias at birth. Most have distinct urethral and vaginal openings within a urogenital sinus and a clitoris-like phallus. Virilization occurs at puberty with phallic growth, rugation and pigmentation of the scrotum, and testicular descent.1–3 At this time, the voice deepens and muscle mass increases, with the development of a . . .