| 遗传性血管性水肿和慢性肾小球肾炎 |
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| 引用本文： | 张宏誉,黄庆元,毕增琪,张茸,何海娟. 遗传性血管性水肿和慢性肾小球肾炎[J]. 中国医学科学院学报, 1988, 0(6) |
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| 作者姓名： | 张宏誉 黄庆元 毕增琪 张茸 何海娟 |
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| 作者单位： | 协和医院 北京(张宏誉，黄庆元，毕增琪，张茸)，协和医院 北京(何海娟) |
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| 摘 要： | C_1抑制物的遗传缺陷是遗传性血管性水肿(HAE)的基本特征。因C_1抑制物缺乏所致补体过度活化可引起肾脏疾患。本院大约有10％的HAE病例合并有慢性肾小球肾炎。本文报告一个HAE家系,在四代中发现14名患者,其中2例于数年前死于喉水肿。存活的12例中,有3例发生慢性肾小球肾炎。其中1例做了肾活检,病理诊断为系膜增生性肾小球肾炎,其母患HAE并因慢性肾小球肾炎导致慢性肾功能衰竭,其表兄患HAE伴蛋白尿及管型尿。HAE的发作可加重肾脏疾患,故应予以长疗程的丹那唑治疗。
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| 关 键 词： | 遗传性血管性水肿 C_1抑制物 慢性肾小球肾炎 |
Hereditary Anqioedema with Chronic Glomerulonephritis |
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| Zhang Hongyu,et al. Hereditary Anqioedema with Chronic Glomerulonephritis[J]. Acta Academiae Medicinae Sinicae, 1988, 0(6) |
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| Authors: | Zhang Hongyu et al |
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| Abstract: | Hereditary angioedema (HAE) is characterized by an inherited defect of C1 inhibitor (C1INH). Excessive complement activation caused by lack of C1INH may initiate the glomerulonephritis. About 10% of patients with HAE in PUMC Hospital were accompanied by chronic glomerulonephritis. A family with HAE is reported in this paper: fourteen patients with HAE in the family were found. Two of them died of laryngeal edema. Three cases of the remaining 12 developed chronic glomerulonephritis. A renal biopsy was performed on one patient. The specimen demonstrated mensangial proliferative glomerulonephritis. The mother suffered from HAE, with chronic renal failure secondary to chronic glomerulonephritis. A cousin with HAE developed prot-einuria and cylindruria. In all cases, renal condition deteriorates after attacks. Long term danazol therapy is considered to be the treatment of choice. |
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| Keywords: | hereditary angioedema C1 inhibitor chronic glomerulonephritis |
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