Male pseudohermaphroditism

male pseudohermaphroditism

Endocrinology A type of intersex in which the testes are present and cryptorchid–ie, 46,XY–male genotype and female phenotype; '…a heterogeneous condition in which the gonads
are exclusively testes, but the genital ducts and/or external genitalia are incompletely masculinized' Clinical The phallus is either very small or there is severe hypospadias; the testes may not have descended in the scrotum, resulting in ambiguity on examination; MP may be caused by defects during sexual differentiation; testosterone production is inadequate–due to ↓ LH or hCG receptors on Leydig cells; Pts are raised as ♀–Morris syndrome and may have CNS defects–eg, defective gonadotropin response, primary gonadal defects–eg, idiopathic, defective pregnanediol–3-β 17-α, 17,20-des-17 β synthesis, regression of müllerian tubes, Leydig cell agenesis, androgen insensitivity, ↑ risk of breast CA, Sertoli adenoma, germinoma in situ, seminoma, Leydig cell tumor. See Intersex disorder. Cf Male hermaphroditism.