遗传性肾炎(附4例分析与家系调查)

HEREDITARY NEPHRITIS(ALPORT'S SYNDROME)

本文对4例遗传性肾炎(Alport综合征)进行分析。经观察发现4例(100％)首发症状均为血尿,同时全部伴有眼—耳—肾病变。伴临床耳聋的肾炎者,无论男女病情均重,预后极差。家系调查结果:显性遗传1例,稀有的隐性遗传3例,国内尚未见报导。本文着重分析遗传性肾炎的临床表现,就其早期诊断线索和遗传方式提出讨论。

The analysis of hereditary nephritis 4 cases in paper, all hematurta in first symptom of 4 cases (100%), which all with Eye-Ear Kidney pathogery. Among nephritis with clinical deafness, Male and Femal both the prognonis is worst and serious. Family investigation result in, X-Chromosom sex linked inheritance dominant in 1 cases, Rarely inheritance recessive in 3 cases (autosomal recessive inheritance in 2 case, X-chromosom sex linked recessive inheritance in 1 case), no report in china. Make a special effort to analysis chinical feature, the discussed inheritance patter and diagnosis indication rapid of hereditary nephritis in article.