Clinical Analysis of 19 Patients with 21 Hydroxylase Deficiency
- VernacularTitle:19例21羟化酶缺陷症临床分析
- Author:
chang-qin, LIU
;
jun, YANG
;
ting-wei, SU
;
shou-yue, SUN
;
li-hao, SUN
;
yong-ju, ZHAO
;
jian-min, LIU
;
xiao-ying, LI
;
guang, NING
- Publication Type:Journal Article
- Keywords:
congenital adrenal hyperplasia;
21 hydroxylase deficiency;
17-hydroxy progesterone
- From:Journal of Shanghai Jiaotong University(Medical Science)
2006;0(01):-
- CountryChina
- Language:Chinese
-
Abstract:
Objective Clinical data of 19 Chinese patients with 21 hydroxylase deficiency (21OHD) were analyzed to improve the diagnosis and treatment level. Methods Clinical features and laboratory data were collected from 19 patients with 21OHD before and after treatment. Results In male patients, the average age of early appearance of secondary sexual character was (9.3?2.8)yrs, and excess androgen resulted in phallic enlargement. Primary amenorrhea was the most common complaint in female(87.5%), and the signs included a varying degree of labioscrotal fusion and clitoral enlargement. The average level of 17-hydroxy progesterone(17OHP) was (63.42?35.07) ?g/L, and adrenocorticotrophic hormone(ACTH), dehydroepiandrosterone(sodium) sulfate(DHEAS) and testosterone(T) were obviously elevated. CT scan showed bilateral adrenal hyperplasia. The level of 17OHP was significantly decreased after treatment[(63.42?35.07) ?g/L vs (3.15?2.71) ?g/L](P
