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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。

Prenatal treatment of congenital adrenal hyperplasia resulting from 21-hydroxylase deficiency.

作者信息

David M, Forest M G

出版信息

J Pediatr. 1984 Nov;105(5):799-803. doi: 10.1016/s0022-3476(84)80310-8.

DOI:10.1016/s0022-3476(84)80310-8
PMID:6334149
Abstract

In an attempt to prevent in utero virilization of female fetuses with 21-hydroxylase deficiency, six mothers at risk were treated with either hydrocortisone (n = 1) or dexamethasone (n = 5) in early pregnancy. Treatment was continued to term in the two pregnancies in which the diagnosis of an affected female fetus was confirmed. In patient 1 (hydrocortisone treatment) fetal adrenal suppression was only partial but the external genitalia were only slightly abnormal. In patient 2 (dexamethasone treatment) fetal adrenal suppression was achieved and the external genitalia were normal at birth. These encouraging results open a new prospect for treating congenital adrenal hyperplasia in utero.

摘要

为防止21-羟化酶缺乏导致女性胎儿在子宫内男性化,对6名有风险的母亲在孕早期使用氢化可的松(n = 1)或地塞米松(n = 5)进行治疗。在确诊为受影响女性胎儿的两例妊娠中,治疗持续至足月。在患者1(氢化可的松治疗)中,胎儿肾上腺抑制仅为部分抑制,但外生殖器仅有轻微异常。在患者2(地塞米松治疗)中,实现了胎儿肾上腺抑制,出生时外生殖器正常。这些令人鼓舞的结果为子宫内治疗先天性肾上腺皮质增生症开辟了新前景。

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Prenatal treatment of congenital adrenal hyperplasia resulting from 21-hydroxylase deficiency.21-羟化酶缺乏所致先天性肾上腺皮质增生症的产前治疗。
J Pediatr. 1984 Nov;105(5):799-803. doi: 10.1016/s0022-3476(84)80310-8.
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Prenatal treatment of congenital adrenal hyperplasia due to 21-hydroxylase deficiency.21-羟化酶缺乏所致先天性肾上腺皮质增生症的产前治疗。
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