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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。
This article reports the pedigree investigation of 6 patients with hereditary angioedema (HAE) and found that two of the high-incidence pedigrees, 4 of 18 patients, died of laryngeal obstruction. The results of serum complement determination in 6 patients and 3 patients in the pedigree survey showed that C_ (1-INH) was significantly lower, which could be used as a specific index for diagnosis of HAE. C_4 was also significantly lower than normal, Can be used as a preliminary screening test; B factor, C_3 and C_ (1?) Within the normal range, suggesting that the bypass pathway is not affected and HAE is a genetic disease.This article also reported HAE patients during the onset, remission Dynamic Changes of C_ (1-INA) and C_4 Contents in Serum during and after Treatment.