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Related to male pseudohermaphroditism: Female pseudohermaphroditism
male pseu·do·her·maph·ro·dit·ism[MIM*261550, MIM*264300, MIM*312100]
pseudohermaphroditism in which the gonads are male and the karyotype is XY but with genital anomalies.
male pseudohermaphroditismA type of intersex in which the gonads are exclusively testes, which are present but cryptorchid (i.e., not present in the scrotum), and the genital ducts and/or external genitalia are incompletely masculinised—i.e., a 46,XY male genotype with a feminine phenotype.
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 decreased LH or hCG receptors on Leydig cells); patients are raised as females (Morris syndrome) and may have CNS defects (e.g., defective gonadotropin response), primary gonadal defects (e.g., idiopathic, defective pregnanediol (3-beta 17-alpha, 17,20-des-17 beta) synthesis, regression of Müllerian tubes, Leydig cell agenesis, androgen insensitivity); increased risk of breast cancer, Sertoli adenoma, germinoma in situ, seminoma and Leydig cell tumours.
male pseudohermaphroditismEndocrinology 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.
A condition in a male marked by a small penis, perineal hypospadias, and scrotum without testes, thereby resembling the vulva. This condition can be due to disease of the adrenal gland or a feminizing tumor of the undescended testis.
See also: pseudohermaphroditism