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[21-羟化酶缺乏所致先天性肾上腺皮质增生症患儿的肾素-血管紧张素及垂体-肾上腺系统]

[Renin-angiotensin and hypophyseo-adrenal systems in children suffering from congenital adrenocortical hyperplasia resulting from 21-hydroxylase deficiency].

作者信息

Kasatkina E P, Tiul'pakov A N, Ibragimova G V, Litvinova N N

出版信息

Probl Endokrinol (Mosk). 1991 Nov-Dec;37(6):p66.

PMID:1664952
Abstract

A study was made of the activity of plasma renin (APR), and the blood levels of ACTH, 17-hydroxyprogesterone (17-OHP) and aldosterone (A) in 50 children suffering from congenital adrenocortical hyperplasia as a result of 21-hydroxylase deficiency; 38 girls and 12 boys aged 1.5 mos. to 14 years were divided into 2 groups: (1) 35 with a salt losing type of disease; (2) 15 with a common virile type of disease. ARP in the 1st group did not exceed the control values and was unaccompanied by adequate secretion of A. Moderate ARP in parallel with a high level of A was noted in the 2nd group. Mineral corticoid therapy resulted in reduced ARP and A concentration, and a tendency to a decrease in the levels of ACTH and 17-OHP was noted. Variations in ARP and in the level of A did not manifest themselves clinically and were unaccompanied by electrolytic disorders. In the authors' opinion, a choice of adequate therapy for both types of disease must be based on the results of investigation of the above hormonal parameters.

摘要

对50例因21 -羟化酶缺乏导致先天性肾上腺皮质增生的儿童进行了血浆肾素活性(APR)、促肾上腺皮质激素(ACTH)、17 -羟孕酮(17 - OHP)和醛固酮(A)血水平的研究;38名女孩和12名男孩,年龄在1.5个月至14岁之间,分为两组:(1)35例为失盐型疾病;(2)15例为常见的男性化型疾病。第一组的ARP未超过对照值,且无A的充分分泌伴随。第二组观察到中度ARP与高水平的A同时出现。盐皮质激素治疗导致ARP和A浓度降低,并注意到ACTH和17 - OHP水平有下降趋势。ARP和A水平的变化在临床上未表现出来,也无电解质紊乱伴随。作者认为,两种类型疾病的适当治疗选择必须基于上述激素参数的调查结果。

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