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遗传性肾病(阿尔波特综合征):临床数据与肾小球基底膜改变的相关性

Hereditary nephropathy (Alport syndrome): correlation of clinical data with glomerular basement membrane alterations.

作者信息

Rumpelt H J

出版信息

Clin Nephrol. 1980 May;13(5):203-7.

PMID:7398144
Abstract

In 32 patients from 27 families affected with hereditary neophropathy (Alport syndrome) the glomerular basement membranes were examined electron microscopically and the percentage of characteristically split and thin basement membrane portions was determined. The clinical course was more severe in males which corresponded with a higher rate of basement membrane alterations: on an average in males 61% split and 6% thin but only 18% split and 21% thin in females. The splitting lesion increased with age in males but not so in females. There were also indications for a possible positive correlation of the splitting lesion and the grade of proteinuria. Compared with the splitting lesion basement membrane thinning seemed to be of minor importance.

摘要

在来自27个患有遗传性肾病(奥尔波特综合征)家庭的32名患者中,对肾小球基底膜进行了电子显微镜检查,并确定了特征性分裂和薄基底膜部分的百分比。男性的临床病程更为严重,这与基底膜改变的发生率较高相对应:男性平均61%分裂和6%变薄,而女性仅18%分裂和21%变薄。男性的分裂病变随年龄增加,而女性则不然。也有迹象表明分裂病变与蛋白尿程度可能呈正相关。与分裂病变相比,基底膜变薄似乎不太重要。

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