摘要
A 16-year-old "female" patient presented as hypertension,hypokalemia,male pseudohermaphroditism,lowered gonadal steroids and cortisol,elevated adre nocorticotropic hormone and pituitary gonadotropin,and 46 XY karyotype.The pat ient was diagnosed as 17α-hydroxylase deficiency,a rare case of congenital ad r enal hyperplasia."She" chose to remain female appearance and social gender af te r negotiation with the parents.Cryptor-chidism of both inguinal canals was surgi cally removed for preventing canceration.After the surgery,a very small daily dose of dexamethasone(0.187 5 mg at bedtime)was enough to control hypertension and hypokalemia,and the therapy of conjugated estrogens(Premarin)was given t o promote the development of female characters.After 6 months of treatment,nor motension and normokalemia remained,and pubarche and mammogenesis emerged.
A 16-year-old ＂female＂ patient presented as hypertension, hypokalemia, male pseudohermaphroditism, lowered gonadal steroids and cortisol, elevated adrenocorticotropic hormone and pituitary gonadotropin, and 46 XY karyotype. The patient was diagnosed as 17α-hydroxylase deficiency, a rare case of congenital adrenal hyperplasia. ＂She＂ chose to remain female appearance and social gender after negotiation with the parents. Cryptor-chidism of both inguinal canals was surgically removed for preventing canceration. After the surgery, a very small daily dose of dexamethasone （0. 187 5 mg at bedtime） was enough to control hypertension and hypokalemia, and the therapy of conjugated estrogens （Premarin） was given to promote the development of female characters. After 6 months of treatment, normotension and normokalemia remained, and pubarche and mammogenesis emerged.
出处
《北京大学学报（医学版）》
CAS
CSCD
北大核心
2008年第2期221-222,共2页
Journal of Peking University:Health Sciences
