儿童IgA肾病合并Alport综合征2例报告并文献复习
发布时间：2018-11-21 17:21
【摘要】：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。
[Abstract]:Objective to explore the diagnosis and differential diagnosis of IgA nephropathy. Methods the clinical data of 2 children with IgA nephropathy were retrospectively analyzed and the related literatures were reviewed. Results the two cases were all male, aged 6 years and 7 years, respectively. The clinical manifestations of these patients were large amounts of proteinuria (mainly albumin), hypoalbuminemia, hypercholesterolemia and hematuria under continuous microscope, all of which were in accordance with the diagnosis of nephrotic syndrome. But the effect of hormone and immunosuppressant was not good. Renal pathological immunofluorescence combined with light microscopy was consistent with mild and moderate Mesangial proliferative IgA nephropathy (M1E0S0T0), and electron microscopy showed that glomerular basement membrane lesions (stratification, rupture, uneven thickness and), Alport syndrome) were not excluded. Further gene detection confirmed the existence of COL4A5 pathogenic gene mutation. Conclusion the combination of IgA nephropathy and Alport syndrome is rare. It is necessary to pay attention to the possibility of both occurring at the same time for children with IgA nephropathy.
【作者单位】： 河北省儿童医院肾脏免疫科;北京大学第一医院儿科;
【分类号】：R726.9
本文编号：2347682
[Abstract]:Objective to explore the diagnosis and differential diagnosis of IgA nephropathy. Methods the clinical data of 2 children with IgA nephropathy were retrospectively analyzed and the related literatures were reviewed. Results the two cases were all male, aged 6 years and 7 years, respectively. The clinical manifestations of these patients were large amounts of proteinuria (mainly albumin), hypoalbuminemia, hypercholesterolemia and hematuria under continuous microscope, all of which were in accordance with the diagnosis of nephrotic syndrome. But the effect of hormone and immunosuppressant was not good. Renal pathological immunofluorescence combined with light microscopy was consistent with mild and moderate Mesangial proliferative IgA nephropathy (M1E0S0T0), and electron microscopy showed that glomerular basement membrane lesions (stratification, rupture, uneven thickness and), Alport syndrome) were not excluded. Further gene detection confirmed the existence of COL4A5 pathogenic gene mutation. Conclusion the combination of IgA nephropathy and Alport syndrome is rare. It is necessary to pay attention to the possibility of both occurring at the same time for children with IgA nephropathy.
【作者单位】： 河北省儿童医院肾脏免疫科;北京大学第一医院儿科;
【分类号】：R726.9
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1 谭国军;李国昀;;Alport综合征2例报告[J];云南医药;2008年04期
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