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快速进展性IgA肾病

Rapidly progressive IgA nephropathy.

作者信息

Welch T R, McAdams A J, Berry A

机构信息

Department of Pediatrics, University of Cincinnati School of Medicine, OH.

出版信息

Am J Dis Child. 1988 Jul;142(7):789-93. doi: 10.1001/archpedi.1988.02150070103038.

Abstract

Five children had rapidly progressive glomerulonephritis, determined by biopsy specimen and terminating in end-stage renal disease. All had mesangial deposition of IgA and C3 in the pattern typically seen with IgA nephropathy (Berger's disease). These children ranged in age from 7 to 13 years; four were boys. Severe hypertension was present in all, and three had a nephrotic syndrome. Other than hypertension and findings related to renal insufficiency or nephrotic syndrome, no clinical or laboratory finding was a consistent marker distinguishing these patients from those with uncomplicated IgA nephropathy, and no therapy proved useful in halting the rapid decline in renal function. The disease has not recurred in the kidney transplant of any of the five children.

摘要

五名儿童患有快速进展性肾小球肾炎,通过活检标本确诊,最终发展为终末期肾病。所有患儿均有IgA和C3在系膜区沉积,呈IgA肾病(伯杰氏病)典型表现。这些儿童年龄在7至13岁之间;四名是男孩。所有人均有严重高血压,三名患有肾病综合征。除高血压以及与肾功能不全或肾病综合征相关的表现外,没有临床或实验室检查结果可作为将这些患者与单纯性IgA肾病患者区分开来的一致标志物,且没有任何治疗方法被证明对阻止肾功能快速下降有效。这五名儿童中没有一人的肾移植出现疾病复发。

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