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先天性异常与先天性肾上腺增生症。

Congenital anomalies and congenital adrenal hyperplasia.

作者信息

Collipp P J, Irani N G, Plachte F

出版信息

Calif Med. 1966 Apr;104(4):278-83.

PMID:5909641
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC1516380/
Abstract

Among 74 patients with congenital adrenal hyperplasia observed at Childrens Hospital, Los Angeles, in a 25-year period, 36 had the simple virilizing type and 38 the salt-losing type. During the same time, seven children with virilizing adrenal tumors were observed at the hospital. While virilization and dehydration were the most common presenting symptoms, some of the children first came to medical attention because of other symptoms, and 11 of them died before adrenal hyperplasia had been diagnosed. Twenty-eight additional congenital cardiovascular, genitourinary, and gastrointestinal anomalies were found in 16 of these 74 children. With proper management, the patients tolerated such stresses as surgical operation and infections without difficulty.

摘要

在洛杉矶儿童医院25年间观察的74例先天性肾上腺皮质增生症患者中,36例为单纯男性化型,38例为失盐型。同期,该院观察到7例男性化肾上腺肿瘤患儿。虽然男性化和脱水是最常见的首发症状,但有些患儿最初因其他症状就医,其中11例在先天性肾上腺皮质增生症被诊断之前死亡。在这74例患儿中的16例还发现了另外28种先天性心血管、泌尿生殖和胃肠道异常。经过适当治疗,患者能够顺利耐受手术和感染等应激情况。

相似文献

1
Congenital anomalies and congenital adrenal hyperplasia.先天性异常与先天性肾上腺增生症。
Calif Med. 1966 Apr;104(4):278-83.
2
[Clinical forms of congenital adrenal hyperplasia in the light of recent studies].[从近期研究看先天性肾上腺皮质增生症的临床类型]
Maroc Med. 1971 Mar;51(544):147-55.
3
Congenital adrenal hyperplasia: problems with developmental anomalies of the external genitalia and sex assignment.先天性肾上腺皮质增生症:外生殖器发育异常及性别指定问题。
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Androgens levels in plasma and urine of patients with virilizing congenital adrenal hyperplasia.先天性肾上腺皮质增生症男性化患者血浆和尿液中的雄激素水平。
Proc R Soc Med. 1970 Oct;63(10):1039-40. doi: 10.1177/003591577006301032.
5
[Adrenogenital syndrome, with special reference to virilism in congenital adrenal hyperplasia].
Naika. 1970 Jun;25(6):1124-7.
6
Androstenedione and its conversion to plasma testosterone in congenital adrenal hyperplasia.先天性肾上腺皮质增生症中雄烯二酮及其向血浆睾酮的转化。
J Clin Invest. 1967 Jun;46(6):1003-9. doi: 10.1172/JCI105589.
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[Congenital adrenal cortex hyperplasia--the adrenogenital syndrome].[先天性肾上腺皮质增生——肾上腺性征异常综合征]
Tidsskr Nor Laegeforen. 1970 Nov 1;90(21):2015-9.
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Diagnosis and management of congenital adrenal hyperplasia.先天性肾上腺皮质增生症的诊断与管理
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本文引用的文献

1
The adrenogenital syndrome with deficiency of 3 beta-hydroxysteroid dehydrogenase.伴有3β-羟类固醇脱氢酶缺乏的肾上腺生殖器综合征
J Clin Invest. 1962 Nov;41(11):2086-92. doi: 10.1172/JCI104666.
2
The adrenogenital syndrome.肾上腺性征异常综合征
N Engl J Med. 1963 Jun 6;268:1283-9 contd. doi: 10.1056/NEJM196306062682308.
3
Treatment of adrenogenital syndrome. Intramuscular therapy with long-acting prednisteroids.
JAMA. 1962 Jun 23;180:1017-20. doi: 10.1001/jama.1962.03050250023005.
4
Virilizing adrenal tumors in children.儿童肾上腺男性化肿瘤
AMA J Dis Child. 1959 Jan;97(1):78-86. doi: 10.1001/archpedi.1959.02070010080007.
5
Congenital defects in adrenal steroid synthesis.肾上腺类固醇合成的先天性缺陷。
Pediatrics. 1958 Jun;21(6):1031-33.
6
Virilizing adrenal hyperplasia; a genetic and hormonal study.男性化肾上腺增生;一项遗传学与激素研究。
J Clin Invest. 1956 Feb;35(2):213-22. doi: 10.1172/JCI103266.
7
Congenital adrenocortical hyperplasia with disturbed electrolyte regulation; dysadrenocorticism.
Pediatrics. 1955 Dec;16(6):875-901.