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21-羟化酶缺乏所致先天性肾上腺皮质增生症的产前治疗:法国多中心研究的第88次更新

Prenatal treatment in congenital adrenal hyperplasia due to 21-hydroxylase deficiency: up-date 88 of the French multicentric study.

作者信息

Forest M G, Bétuel H, David M

机构信息

INSERM U 34, Hôpital Debrousse, Lyon, France.

出版信息

Endocr Res. 1989;15(1-2):277-301. doi: 10.1080/07435808909039101.

DOI:10.1080/07435808909039101
PMID:2667968
Abstract

A multicentric study of prenatal treatment of congenital adrenal hyperplasia (CAH) resulting from 21-hydroxylase deficiency in 43 pregnancies at risk for CAH is presented. The mothers were given dexamethasone per os, 0.5 mg either 12-hourly or 8-hourly. From the analysis of the results obtained in the present study and review of the literature, it would appear that the first condition for successful prevention of female virilization in utero (a total of 6 cases) is to start treatment as early as possible, no later than the 7th week. The dose of dexamethasone should be related to maternal size: 20 micrograms/kg/day (in 2 or 3 fractioned) doses would seem to be both efficient and safe. Adrenal suppression of both maternal and fetal adrenal function should be controlled by appropriate hormonal determinations. Finally, the advantages of early prenatal diagnosis or no prenatal diagnosis are discussed.

摘要

本文介绍了一项多中心研究,该研究针对43例有先天性肾上腺皮质增生症(CAH)风险的妊娠进行了21-羟化酶缺乏所致先天性肾上腺皮质增生症的产前治疗。母亲口服地塞米松,每12小时或每8小时服用0.5毫克。从本研究获得的结果分析以及文献综述来看,成功预防子宫内女性男性化(共6例)的首要条件似乎是尽早开始治疗,不迟于第7周。地塞米松的剂量应与母亲的体型相关:20微克/千克/天(分2或3次服用)的剂量似乎既有效又安全。应通过适当的激素测定来控制对母亲和胎儿肾上腺功能的肾上腺抑制。最后,讨论了早期产前诊断或不进行产前诊断的优缺点。

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Prenatal treatment in congenital adrenal hyperplasia due to 21-hydroxylase deficiency: up-date 88 of the French multicentric study.21-羟化酶缺乏所致先天性肾上腺皮质增生症的产前治疗:法国多中心研究的第88次更新
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