【摘要】 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．更多还原

【Abstract】 Wilson’s disease (WD), an autosomal recessive disorder of copper metabolism,presents with hepatic, neurologic,or psychi-atric symptomatology. In some patients,the kidney may be affected and mainly mani-fests as abnormalities of renal tubular func-tion. MD presenting as gross hematuria and IgA-deposition in glomerular mesangium is rare in the literatures.A 8-year-old girl complining of gross hematuria, puffiness of eyelids for 3 days was admitted to our hospital. Her past med-ical history included hepatomegaly since 7-year-old, elevated serum transaminases and negative serum hepatitis markers (hepatitis A,B and C).Examination showed kayser-Fleischer rings,enlarged liver 4 cm below the costal margin, proteinuria (392 mg/24h ), hema-turia (RBC＞ 10/HP ), markedly deformed morphologically, defetive urinary acidifica-tion, elevated serum transaminases (ALT 175 IU/L, AST 194 ~ 59l IU/L) and low serum ceruloplasmin. Renal biopsy showed mild diffuse mesandial proliferative glomerulonephritis, IF exam showed IgA (+ + +),C3 (+ + ),lgG(-),IgM (- ) staining in mesangial area. EM exam showed dense deposit in mesangium, with kubeanic acid stain cupper granule deposi-tion were found in the tubular celIs but not the mesangial area In this patient, the predominat IgA staining in the mesangium was thought to be resided from defect in the clearance for IgA or IgA-cormplex by liver damage in WD. This was a case of WD associated with secondary lgA-Nephropathy.更多还原