Clinical characteristics and analysis of 4 cases of hepatic amyloidosis
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摘要:
目的对已确诊病例的临床特点进行总结和分析,以提高对本病的认识,减少误诊率。方法经肝、肾组织学检查及特异性组织学染色明确诊断为肝淀粉样变性的4例住院患者。住院期间检测肝功、血脂、尿常规等化验,腹部彩超、腹部CT或腹部核磁,肝脏、肾脏的活体组织病理检查及特异性组织学染色(刚果红染色)。结果患者均有肝大,肝功生化检查AKP及r-GT明显增高,低蛋白血症,高脂血症。肝脏彩超和CT显示肝脏增大,肝密度低,回声细密,类似脂肪肝的表现。肝穿及肾穿病理见大量粉红色淀粉样物质沉积,刚果红染色(+),病理结论为肝、肾淀粉样变性。结论对临床上高度怀疑为肝淀粉样变性的患者,作肝肾活体组织检查及特异性组织学染色(刚果红染色),可证实为肝淀粉样变性。
Abstract:Objective To analyze the clinical features of patients diagnosed as hepatic amyloid degeneration to improve the current understanding of the disease and decrease misdiagnosis.Methods A total of 4 patients were diagnosed with hepatic amyloid degeneration based on the pathology results gained from the aspiration samples of liver or kidney and specific dyeing (Congo red dye) .Liver function tests, blood fat, routine urine tests, abdominal ultrasound and CT or MRI of the patients were performed.Results All patients had hepatomegaly, significant elevation of AKP and r-GT, hypoproteinemia and hyperlipidemia.Hepatic ultrasound and CT showed an enlarged liver and with low density, and the hepatic echo was fine and closely similar with the features of fatty liver.Pathologic results of liver or kidney aspirate showed sedimentation of pink amyloid matters.Congo red dye test was positive and pathological consequence was hepatic and renal amyloidosis.Conclusion Liver or kidney aspirate examination and specific dyeing (Congo red dye) is necessary in patients suspected with hepatic amyloid degeneration.
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Key words:
- Hepatic amyloid degeneration /
- clinical features /
- Congo red dye test
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