남성 가성반음양
Clinical Experience of Male Pseudohermaphroditism
소속 상세정보
박승오/Park SO
윤종병/Yoon JB
KMID : 0358319820230081183
Abstract
Although the definition of male pseudohermaphroditism remains controversial, male pseudohermaphrodites will be defined as chromatin-negative individuals who have testis with the failure of normal development of the normal male.
Also included are those individuals who may have more than one cell line, but at least one cell line, containing a Y chromosome and no cell line having two X chromosomes. Gonadal histologic findings may be either testicular or streak, but not ovarian.
Male pseudohermaphroditism can result as a consequence of 1) absent M(llerian regression, 2) Inadequate synthesis of testosterone, 3) inadequate synthesis of dihydrotestosterone and 4) androgen receptor deficiency.
We have seen 5 cases of male pseudohermaphroditism with manifestations of penoscrotal or perineal hypospadias, pubertal virilization, cryptorchism or atrophied testes, and feminization.
Four cases had been reared as male and one case as female. All revealed chromatin-negative pattern in buccal smear and testes.
After the plausible discussion, two cases were decided to be reared as female and three were as male.
키워드
간성; male pseudohermaphroditism
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