先天性肾上腺皮质增生症21羟化酶缺陷症合并妊娠的临床及生化特点  

Clinical and Biochemical Features of Pregnant Women With Congenital Adrenal Hyperplasia Due to 21-hydroxylase Deficiency

作　　者：卢琳[1] 曾正陪[1] 陆召麟[1] 童安莉[1] 陈适[1] 王永慧[1] 付春莉[1] 陶红[2] 张波[3] 

机构地区：[1]中国医学科学院北京协和医学院北京协和医院卫生部内分泌重点室验室,北京100730 [2]北京安贞医院 [3]中日友好医院

出　　处：《内科急危重症杂志》2010年第1期13-16,共4页Journal of Critical Care In Internal Medicine

摘　　要：目的:分析21OHD女性患者妊娠及其妊娠期间的临床生化特点。方法:对来我院就诊的88例21OHD育龄期女性患者调查其妊娠分娩的情况。结果:在此期间就诊的女性已婚确诊21OHD患者为54名,19名患者共妊娠34次,其中活产婴儿16个,自然流产9次。新生儿男性5例,女性11例。妊娠后血17羟孕酮升高倍数的中位数为8.06倍,6例患者妊娠后改变了糖皮质激素的用法。结论:21OHD患者的妊娠率下降,妊娠期间调整糖皮质激素用法需要个体化。Objective： To investigate the clinical and biochemical features of the pregnant women with the 21- hydroxylase deficiency （21OHD）. Methods： The history of pregnancy and delivery were reviewed in 88 female patients with 21 OHD. Results： There were 54 married women were confirmed with 21 OHD. There were 34 preg- nancies among 19 female patients, only 16 were normal deliveries with 11 healthy girls and 5 healthy boys. Sponta neous abortion was observed in 9 pregnancies. The median of serum 17 alpha-hydroxyprogesterone was elevated 8. 06 folds after pregnancy. During the gestational period, the dosage of glucocorticoid was changed in 6 pregnan- cies. Conclusions.. The rate of pregnant was moderately reduced in treated adult women with 21 OHD. The dosage of glueocorticoid should be carefully titrated during the pregnancy individually.

关 键 词：先天性肾腺皮质增生 21羟化酶缺陷症 妊娠 

分 类 号：R586[医药卫生—内分泌]