| 遗传性血管性水肿患者血清补体测定的诊断价值 |
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| 引用本文： | 钱雪冶,陆明兰,吴学愚,黄鹤年,张天仁,宣熙华.遗传性血管性水肿患者血清补体测定的诊断价值[J].复旦学报(医学版),1988(4). |
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| 作者姓名： | 钱雪冶 陆明兰 吴学愚 黄鹤年 张天仁 宣熙华 |
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| 作者单位： | 上海医科大学耳鼻喉科研究所
(钱雪冶，吴学愚，黄鹤年)，上海生物制品研究所
(陆明兰，张天仁)，上海医科大学耳鼻喉科研究所(宣熙华) |
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| 摘 要： | 本文报道6例遗传性血管性水肿(HAE)患者的家系调查,发现两个高发病家系,18例患者其中4例死于喉阻塞。对此6例患者和家系调查中3例患者血清中补体含量测定结果表明C_1酯酶抑制剂(C_(1-INH)明显低下,可作为诊断HAE的特异性指标;C_4亦明显低于正常,可作为一种初筛试验;B因子、C_3和C_(1?)在正常范围内,提示旁路途径未受影响和HAE是一种遗传性疾病。本文还报道了HAE患者在发病期、缓解期和治疗期间血清中C_(1-INA)、C_4含量的动态变化。
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| 关 键 词： | 遗传性血管性水肿 补体 C_1酯酶抑制剂 |
DETECTION OF SERUM COMPLEMENT IN DIAGNOSTIC VALUE OF HEREDITARY ANGIOEDEMA |
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| Qian Xueyu,Lu Minglan Wu Xueyu,Huang Henian,Zhang Tianren,Xuan Xuhua.DETECTION OF SERUM COMPLEMENT IN DIAGNOSTIC VALUE OF HEREDITARY ANGIOEDEMA[J].Fudan University Journal of Medical Sciences,1988(4). |
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| Authors: | Qian Xueyu Lu Minglan Wu Xueyu Huang Henian Zhang Tianren Xuan Xuhua |
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| Abstract: | Hereditary angioedema (HAE) is a complement C1 esterase inhibitor (C1-INH) defect disorder. It is characterized by recurrent acute edema of the extremities, the respiratory tract and the gastroenterio tract. Acute laryngeal edema usually produces laryngeal obstruction. Six cases of this disease are reported in this paper. Familial analysis revealed that two families there were a high incidences and eighteen patients, four of them had died of laryngeal obstruction. Laboratory examination showed a remarkable decrease of C1-INH and C4; B factor, C3, C1q were normal. This means that the pass way is not affected, and HAE is confirmed as a hereditary disease., This paper reports chang of C1-INH and C4 in paroxysm and resume of hereditary angioedema. |
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| Keywords: | hereditary angioedema complement C1 esterase inhibitor |
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