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遗传性血管性水肿发作的预防。

Prophylaxis of attacks of hereditary angioedema.

作者信息

Rothbach C, Green R L, Levine M I, Fireman P

出版信息

Am J Med. 1979 Apr;66(4):681-3. doi: 10.1016/0002-9343(79)91184-7.

Abstract

Danazol, an attentuated androgen, has been suggested as an effective agent for the prophylaxis of attacks of hereditary angioedema. Four patients, with a clinical history of hereditary angioedema and a demonstrated depression of the serum inhibitor of the first component of complement (C1 INH) and the fourth component of complement (C4), were entered into a study to determine the minimum effective dose of this agent. All four of the patients had been experiencing attacks at least monthly, but they had only six attacks during a total of 60 patient months of Danazol therapy. The minimum effective dose varied from 100 to 400 mg/day. The drug appeared to work by increasing the level of serum C1 INH which reached the normal range in two of four patients. Side effects were only the anticipated menstrual irregularities in the female patients. Danazol appears to be an efficacious drug for prophylaxis of hereditary angioedema.

摘要

达那唑,一种弱雄激素,已被认为是预防遗传性血管性水肿发作的有效药物。对4例有遗传性血管性水肿临床病史且已证实血清补体第一成分抑制剂(C1 INH)和补体第四成分(C4)降低的患者进行了一项研究,以确定该药物的最小有效剂量。所有4例患者至少每月发作一次,但在总共60个患者月的达那唑治疗期间仅发作6次。最小有效剂量为每日100至400毫克。该药物似乎通过提高血清C1 INH水平起作用,4例患者中有2例的C1 INH水平达到正常范围。副作用仅为女性患者预期的月经不调。达那唑似乎是预防遗传性血管性水肿的有效药物。

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