【摘要】：目的探討IgA腎病的診斷和鑒別診斷的思路。方法回顧分析2例IgA腎病患兒的臨床資料,并復(fù)習(xí)相關(guān)文獻(xiàn)。結(jié)果2例患兒均為男性,年齡分別為6歲、7歲,臨床表現(xiàn)為大量蛋白尿(以白蛋白為主)、低白蛋白血癥、高膽固醇血癥及持續(xù)鏡下血尿,均符合腎病綜合征診斷,但激素及免疫抑制劑治療效果欠佳。腎臟病理免疫熒光結(jié)合光鏡檢查符合輕、中度系膜增生性IgA腎病(M1E0S0T0);電鏡提示腎小球基底膜病變(分層、斷裂、薄厚不均),Alport綜合征不除外。進(jìn)一步基因檢測證實(shí)存在COL4A5致病基因突變。結(jié)論 IgA腎病和Alport綜合征二者合并發(fā)生較為罕見,對于治療效果欠佳特別是有相關(guān)家族史的IgA腎病患兒,應(yīng)注意二者同時(shí)發(fā)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.
【作者單位】： 河北省兒童醫(yī)院腎臟免疫科;北京大學(xué)第一醫(yī)院兒科;
【分類號】：R726.9

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本文編號：2347682