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儿童 IgA 肾病合并薄基底膜肾病的临床表现。

Clinical manifestations of IgA nephropathy combined with thin glomerular basement membrane nephropathy in children.

机构信息

Department of Pediatrics, Kyungpook National University, School of Medicine, Daegu, Korea.

Department of Pathology, Kyungpook National University, School of Medicine, Daegu, Korea.

出版信息

Kidney Res Clin Pract. 2013 Sep;32(3):111-4. doi: 10.1016/j.krcp.2013.07.001. Epub 2013 Sep 4.

Abstract

BACKGROUND

Immunoglobulin A nephropathy (IgAN) and thin glomerular basement membrane nephropathy (TBMN) are the most common causes of persistent hematuria during childhood. The objective of this study is to determine the difference in clinical features and laboratory findings between pediatric patients with IgA deposited TBMN and IgAN alone.

METHODS

Between January 2000 and March 2009, 95 children were diagnosed with IgAN by renal biopsy. Clinical features and laboratory findings of patients with isolated IgAN and with IgAN plus TBMN were compared; the children diagnosed with IgAN were compared to 127 children who had been diagnosed with TBMN alone during the same period.

RESULTS

There were 71 (74.7%) of a total 95 patients that were diagnosed with isolated IgAN (Group1); in 24 (25.3%) of the 95 patients IgAN was combined with TBMN (Group 2). There was marked difference in the gender distribution between Group 2 and isolated TBMN patients. The degree of proteinuria and pathologic severity was higher in Group 1 compared with Group 2. Gross hematuria was present in both groups. There were no distinguishing features in the other laboratory parameters.

CONCLUSION

Patients with both IgAN and TBMN seem to have similar clinical features to patients with isolated IgAN; however, the latter tend to have better pathologic and laboratory findings, compared to the patients with IgAN alone.

摘要

背景

免疫球蛋白 A 肾病(IgAN)和薄基底膜肾病(TBMN)是儿童持续性血尿的最常见原因。本研究旨在确定 IgA 沉积于 TBMN 且单纯 IgAN 患儿的临床特征和实验室检查结果的差异。

方法

2000 年 1 月至 2009 年 3 月期间,95 例儿童经肾活检诊断为 IgAN。比较单纯 IgAN 与 IgAN 合并 TBMN 患儿的临床特征和实验室检查结果;并将 IgAN 患儿与同期单纯 TBMN 患儿(127 例)进行比较。

结果

95 例患儿中,71 例(74.7%)被诊断为单纯 IgAN(组 1);95 例患儿中有 24 例(25.3%)IgAN 合并 TBMN(组 2)。组 2 的性别分布与单纯 TBMN 患儿存在显著差异。组 1 的蛋白尿程度和病理严重程度高于组 2。两组均有肉眼血尿。其他实验室参数无明显差异。

结论

IgAN 合并 TBMN 患儿的临床特征与单纯 IgAN 患儿相似,但与单纯 IgAN 患儿相比,后者的病理和实验室检查结果更好。

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