male 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 pseudohermaphroditism

A 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.
Clinical findings
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.
Pathogenesis
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 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.

male pseudohermaphroditism

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

单词	male pseudohermaphroditism
释义	DictionarySeepseudohermaphroditism male pseudohermaphroditism male pseudohermaphroditism [māl ‚süd·ō·hər′maf·rə‚did‚iz·əm] (medicine) androgyny male pseudohermaphroditism male pseu·do·her·maph·ro·dit·ism [MIM261550, MIM264300, MIM*312100] pseudohermaphroditism in which the gonads are male and the karyotype is XY but with genital anomalies. male pseudohermaphroditism A 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. Clinical findings 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. Pathogenesis 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 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. male pseudohermaphroditism 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 AcronymsSeemiles per hour
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