目的 探讨IgA肾病的诊断和鉴别诊断的思路。方法 回顾分析2例IgA肾病患儿的临床资料，并复习相关文献。结 果 2例患儿均为男性，年龄分别为6岁、 7岁，临床表现为大量蛋白尿（以白蛋白为主）、低白蛋白血症、高胆固醇血症及持续镜下 血尿，均符合肾病综合征诊断，但激素及免疫抑制剂治疗效果欠佳。肾脏病理免疫荧光结合光镜检查符合轻、中度系膜增生性IgA 肾病（M1E0S0T0）； 电镜提示肾小球基底膜病变（分层、断裂、薄厚不均），Alport综合征不除外。进一步基因检测证实存在COL4A5 致病基因突变。结论 IgA肾病和Alport综合征二者合并发生较为罕见，对于治疗效果欠佳特别是有相关家族史的IgA肾病患儿， 应注意二者同时发生的可能。

Objective  To explore the diagnosis and differential diagnosis of IgA nephropathy. Methods  The clinical data of 2 children with IgA nephropathy were retrospectively analyzed. The pertinent literatures were reviewed. Results  In 2 males aged 6 and 7 years, the clinical features were a large amount of proteinuria (mainly albumin), low serum albumin, high cholesterol, and persistent microscopic hematuria, which were in line with the diagnosis of nephrotic syndrome. The effects of hormone and immunosuppressive therapy were poor. Renal pathology immunofluorescence and light microscopy findings were in accord with mild to moderate mesangial proliferative IgA nephropathy (M1E0S0T0). Electron microscope showed glomerular basement membrane lesions (layering, breakage, and uneven thickness), which could not exclude Alport syndrome. Further gene detection confirmed a pathogenic mutation of COL4A5. Conclusions  It is rare that IgA nephropathy is combined IgA nephropathy at the same time. Attention should by paid to those who had a poor effect of treatment or had a related family history in IgA patients because it is possible that IgA nephropathy and IgA nephropathy may occurred at the same time.