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本文报告三例以血尿为首发症状的肝豆状核变性.除肾小球功能检查外并进行肾穿活检,表现有典型IgA肾病改变,用化染色(kubeanicacid)显示在肾小管上皮细胞内有铜颗粒沉积,而肾小球毛细血管袢及系膜区未见铜沉积.分析此例肾的受累除文献强调的肾小管受累外,可能因肝损伤后对血液中IgA或含IgA的复合物清除能力下降,而致IgA沉积于肾小球系膜区,最终引起继发性IgA肾病.
This article reports three cases of hepatolenticular degeneration with hematuria as the first symptom. In addition to glomerular function tests and renal biopsy, there are typical changes in IgA nephropathy, staining (kubeanicacid) showed deposition of copper particles in renal tubular epithelial cells, and glomerular capillary loop and mesangial area have not See copper deposition. Analysis of this case of renal involvement in addition to the literature emphasize the tubular involvement, may be due to liver injury IgA or IgA-containing compounds in the blood scavenging capacity decreased, leaving IgA deposition in the mesangial area, eventually causing secondary Sex IgA nephropathy.