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先天性肾上腺皮质增生症的产前诊断与治疗

Prenatal diagnosis and treatment of congenital adrenal hyperplasia.

作者信息

Speiser P W, New M I

机构信息

Division of Pediatric Endocrinology and Metabolism, North Shore University Hospital, Manhasset, NY 11030.

出版信息

J Pediatr Endocrinol. 1994 Jul-Sep;7(3):183-91. doi: 10.1515/jpem.1994.7.3.183.

DOI:10.1515/jpem.1994.7.3.183
PMID:7820211
Abstract

Congenital adrenal hyperplasia (CAH) due to 21-hydroxylase deficiency is associated with hormonal imbalance which predisposes affected females to prenatal development of genital ambiguity. Because the disease is usually not lethal and can be treated with glucocorticoids, affected pregnancies are seldom terminated. Dexamethasone can be administered to the pregnant mother and is effective in correcting the fetus's adrenal hormone imbalance during gestation. Nearly a decade's experience with prenatal treatment of CAH indicates that the risk-benefit ratio is favorable for mother and fetus with careful medical supervision of gestationally administered dexamethasone.

摘要

由于21-羟化酶缺乏导致的先天性肾上腺皮质增生症(CAH)与激素失衡有关,这使受影响的女性在产前易出现生殖器模糊不清的情况。因为这种疾病通常不会致命,且可用糖皮质激素治疗,所以受影响的妊娠很少被终止。地塞米松可给予孕妇,在孕期能有效纠正胎儿的肾上腺激素失衡。近十年对CAH进行产前治疗的经验表明,在对孕期给予地塞米松进行仔细医学监测的情况下,对母亲和胎儿而言风险效益比是有利的。

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引用本文的文献

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Prenatal diagnosis and treatment of steroid 21-hydroxylase deficiency (congenital adrenal hyperplasia).类固醇21-羟化酶缺乏症(先天性肾上腺皮质增生症)的产前诊断与治疗
Indian J Pediatr. 2000 Nov;67(11):813-8. doi: 10.1007/BF02726225.