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[雄激素治疗遗传性血管性水肿]

[Treatment of hereditary angioneurotic edema with androgens].

作者信息

Lagrue G, Moisy M, Intrator L, Belghiti D, Sobel A

出版信息

Nouv Presse Med. 1978 Sep 30;7(33):2927-31.

PMID:724439
Abstract

Hereditary angioedema (HANE) is a rare, life-threatening disease due to the deficiency of C1 inhibitor (C1 Inh). Androgen therapy has been recently shown to be effective for prophylaxis of Hane attacks. Since life-long androgen therapy may be hazardous, this study was designed to define the minimal doses required for effectiveness. Ten patients from six different families were treated during cumulative 73 months by danazol and/for methandrostenolone. One tablet/day of either drug was the minimal requirement to prevent any attack in all patients. When 3 tablets/day were given, complement abnormalities were simultaneously rapidly reversed. When 1 tablet/day was given the biological effect was barely detectable, except for C2. Serum C2 levels may, therefore, represent the best criteria of androgen therapy effectiveness. Thus, an excellent clinical result can be obtained with much lower doses of androgens than previously stated. This result seemed important with respect to the serious dose-dependent risk of androgens.

摘要

遗传性血管性水肿(HANE)是一种由于C1抑制物(C1 Inh)缺乏导致的罕见的、危及生命的疾病。最近研究表明雄激素疗法对预防HANE发作有效。由于终身雄激素治疗可能有风险,本研究旨在确定有效所需的最小剂量。来自六个不同家庭的10名患者接受了达那唑和/或甲雄烯醇酮累计73个月的治疗。两种药物中的任何一种每天一片是预防所有患者发作的最低要求。当每天服用3片时,补体异常同时迅速逆转。当每天服用1片时,除C2外,几乎检测不到生物学效应。因此,血清C2水平可能是雄激素治疗效果的最佳标准。因此,与之前所述相比,使用低得多的雄激素剂量就能获得优异的临床效果。鉴于雄激素严重的剂量依赖性风险,这一结果似乎很重要。

相似文献

1
[Treatment of hereditary angioneurotic edema with androgens].
Nouv Presse Med. 1978 Sep 30;7(33):2927-31.
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Treatment of hereditary angioedema with danazol. Reversal of clinical and biochemical abnormalities.
N Engl J Med. 1976 Dec 23;295(26):1444-8. doi: 10.1056/NEJM197612232952602.
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[Formation of IgG antibodies to C1 inhibitor as the cause of life-threatening angioedema].
Dtsch Med Wochenschr. 1987 Mar 27;112(13):503-6. doi: 10.1055/s-2008-1068084.

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