[Partial androgen insensitivity syndrome].
There were studied 13 individuals with partial androgen insensitivity syndrome (PAIS) and we observed hypertrophy of clitoris in 92.3%, female pubic hair in 69.2%, complete absence of vagina in 69.2% and normal female development of breasts in 38.5%. In the partial form of AIS more frequent than in the complete form surgery for inguinal hernia, for hypertrophy of clitoris and for artificial vagina was necessary. The discussed form of this male hermaphroditism is caused by rare and untypical mutations in the androgen receptor gene and a differential diagnosis must be performed by exclusion above all the anorchism, Mayer-Rokitansky-Küstner syndrome and congenital adrenal hypertrophy. Among all cases of AIS the frequency of PAIS appeared 28.3%.
Baron
J
J
Instytutu Ginekologii i Połoznictwa AM w Poznaniu.
D000293:Adolescent; D000328:Adult; D013734:Androgen-Insensitivity Syndrome / Q000175:diagnosis* / Q000628:therapy; D002648:Child; D002987:Clitoris / Q000473:pathology; D005260:Female; D006801:Humans; D006984:Hypertrophy; D008297:Male; D008875:Middle Aged; D014621:Vagina / Q000002:abnormalities*
Niepełny lub cześciowy zespół braku wrazliwości na androgeny.