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緒言
男性化現象は種々の疾患で観察されるが,中でも先天性副腎皮質過形成(Congenital Adrenal Hyperplasia, CAHと略)による副腎性器症侯群(Adrenogenital Syndrome, AGSと略)は,副腎皮質におけるCortisol生合成に関与する酵素の障害による疾患として知られている。最近当教室においてもAGSの1例を経験したので報告する。
A 4 year old girl was admitted to the urological department of Niigata University Hospital on Feb-ruary 27, 1969, with the chief complaint of enlarged clitoris and past history of genital abnormality, which was noticed at birth.
Physical development was over the average of a 4 year old girl and bone development was eqivalent to 13 years of age. Physical examination revealed enlarged clitoris with fusion of labium and the exter-nal urethral orifice was noticed at the base of clitoris, inspite that the vaginal outlet was indefinite. Urogenital sinus was outlined by urethrography.
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