21-羟化酶缺陷症的围妊娠期管理研究进展  

Peripregnancy management of 21-hydroxylase deficiency

作　　者：郭伟红[1] 何庆[1] Guo Weihong;He Qing(Department of Endocrinology and Metabolism,Tianjin Medical University General Hospital,Tianjin 300052,China)

机构地区：[1]天津医科大学总医院内分泌代谢科,天津300052

出　　处：《国际内分泌代谢杂志》2023年第2期128-131,共4页International Journal of Endocrinology and Metabolism

基　　金：天津市医学重点学科(专科)建设项目(TJYXZDXK-030A)。

摘　　要：21-羟化酶缺陷症是先天性肾上腺皮质增生症中最常见的类型,近年随着人们对生育要求的提高,21-羟化酶缺陷症女性患者围妊娠期的管理受到广泛关注。为了减轻未出生婴儿的男性化程度并提高活产率,孕前病情控制及遗传咨询、妊娠期药物剂量及种类的调整及监测标志物的选择、分娩时糖皮质激素应激剂量的调整、治疗中如何避免肾上腺危象、产后和哺乳期药物剂量调整及非经典先天性肾上腺皮质增生症围妊娠期的治疗等均需进一步规范。本文将针对相关管理策略和治疗的现状及研究进展进行综述。21-hydroxylase deficiency is the most common form of congenital adrenal cortex (CAH).In recent years,with the improvement of fertility requirements,perinatal management of female patients with congenital adrenal hyperplasia (CAH) has attracted more and more attention.In order to reduce the degree of masculinity of the affected unborn babies and improve the live birth rate,disease control and genetic counseling before pregnancy,adjustment of drug dosage and type during pregnancy and selection of monitoring markers,adjustment of glucocorticoid dose during delivery,how to avoid adrenal crisis in treatment,drug dose adjustment during postpartum and lactation and treatment of non-classical CAH during pregnancy need to be further standardized.This article reviews the current status and research progress of related manage strategies and treatments.

关 键 词：21-羟化酶缺陷症 先天性肾上腺皮质增生症 围妊娠期 糖皮质激素 

分 类 号：R714.2[医药卫生—妇产科学]