单纯男性化先天性肾上腺皮质增生症合并正常生育1例并文献复习  

Successful Pregnancy in A Women Patient with Simple Virilizing Congenital Adrenal Hyperplasia and Review of the Literature

作　　者：江依群 肖宁[2] 周兴[2] 

机构地区：[1]广州女子医院,广东广州510370 [2]广州医科大学第二附属医院

出　　处：《中国医学创新》2014年第33期119-121,共3页Medical Innovation of China

基　　金：2014年惠州市科技计划项目(2014Y209)

摘　　要：目的:提高对先天性肾上腺皮质增生症(CAH)的认知,探讨其诊治方法,了解最近研究进展。方法:对1例单纯男性化CAH合并正常生育患者临床资料进行分析,并结合文献复习。结果:患者行激素替代联合外阴整形术、阴道扩大术。术后18个月自然受孕,术后28个月顺产1正常男婴。结论:CAH的早期、规律、系统治疗对于改善患者生活质量和成年后生育能力尤为关键,治疗目前多以激素替代联合外生殖器整形术为主。Objective：To improve the acknowledge about congenital adrenal hyperplasia（CAH）, and to discuss the treatment of CAH, and to update the latest development of it.Method：The data of a patient with simple virilizing congenital adrenal hyperplasia was collected and analyzed, and related literature was reviewed.Result：Glucocorticoids replacement therapy combined with reconstruction of external genitalia and dilation of vagina was performed. In followup stage, successful fertility and natural delivery for 18 and 28 months, postoperatively and respectively, the male infant was normal.Conclusion：It is benefit to employ the early, regular and systemic therapy, which can play a decisive role for improvement of life quality and post-pubertal fertility ability, for patients with CAH. Nowdays, hormone replacement therapy combined with external genitalia reconstruction is recommend in treatments of CAH.

关 键 词：先天性肾上腺皮质增生症 女性假两性畸形 诊断 治疗 

分 类 号：R586[医药卫生—内分泌]