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基底膜肾病:基于超微结构发现对Alport综合征和无症状血尿的新分类。

Basement membrane nephropathy: a new classification for Alport's syndrome and asymptomatic hematuria based on ultrastructural findings.

作者信息

Yum M, Bergstein J M

出版信息

Hum Pathol. 1983 Nov;14(11):996-1003. doi: 10.1016/s0046-8177(83)80180-4.

Abstract

Renal biopsy specimens from 19 patients with asymptomatic hematuria, normal glomeruli on light microscopic examination, and negative immunofluorescence were studied to characterize the ultrastructural changes of the glomerular basement membrane. Three groups of patients were identified. Four patients (group 1) had type I basement membrane nephropathy, characterized by marked thickening and lamellation of the basement membrane in a pattern resembling that of Alport's syndrome. Seven patients (group 2) had type II basement membrane nephropathy, which was characterized by extensive attenuation of the basement membrane with only occasional small areas of lamellation or fragmentation. Eight patients (group 3) had moderate variability in basement membrane thickness with no other structural alterations. The authors propose that this pathologic classification be used in patients with asymptomatic hematuria as a basis for long-term clinical investigations, in hopes of distinguishing the natural history of each group.

摘要

对19例无症状血尿患者的肾活检标本进行了研究,这些患者的肾小球在光镜检查下正常,免疫荧光检查为阴性,以确定肾小球基底膜的超微结构变化。确定了三组患者。4例患者(第1组)患有I型基底膜肾病,其特征是基底膜显著增厚和分层,其模式类似于Alport综合征。7例患者(第2组)患有II型基底膜肾病,其特征是基底膜广泛变薄,仅偶尔有小面积的分层或断裂。8例患者(第3组)基底膜厚度有中度变化,无其他结构改变。作者建议将这种病理分类用于无症状血尿患者,作为长期临床研究的基础,以期区分每组的自然病程。

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