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目的 :报告 2 1例以肝损为主肝豆状核变性 ,进一步提高原因不明肝病和肝豆状核变性诊治工作。方法 :收集我院近 8年来 2 1例以肝损为主的肝豆状核变性 ,对其临床和实验检查改变特征进行总结、分析。结果 :以肝损为主肝豆状核变性有以下特点 :本病男、女均可发生 ,主要在 5～ 15岁发病 ,近 90 % ,小于 5岁及 15岁以上少见。肝损害中肝硬化占 80 %以上。绝大多数有不同程度的肝外脏器受损 ,如贫血占 80 .9% ,椎体外系症状占 6 1.92 8.6 %有家族史。角膜 K—F环 ,血清铜 ,铜兰蛋白 ,铜氧化酶 ,尿铜测定等均有重要诊断、鉴别意义。结论 :对原因不明肝病 ,特别是小儿肝病 ,只要提高对本病认识 ,根据临床特征和实验检查 ,诊断该病并无困难。必要时可作肝活检进一步防止误诊、漏诊
Objective: To report 21 cases of hepatolenticular degeneration with liver damage as the main cause, to further improve the diagnosis and treatment of liver disease and hepatolenticular degeneration. Methods: Twenty-one cases of hepatolenticular degeneration with hepatic impairment were collected in our hospital in the recent 8 years. The clinical and experimental characteristics of the changes were collected and analyzed. Results: The main characteristic of hepatic degeneration was hepatomegaly: The disease can occur in both males and females, mainly in the age of 5 to 15 years, nearly 90%, less than 5 years old and over 15 years old rare. Liver cirrhosis accounts for more than 80%. Most have different degrees of extrahepatic organ damage, such as anemia accounted for 80.9%, extrapyramidal symptoms accounted for 6 1.92 8.6% had a family history. Corneal K-F ring, serum copper, copper blue protein, copper oxidase, urine copper determination are important diagnostic and differential significance. Conclusion: For unknown causes of liver disease, especially in children with liver disease, as long as the understanding of the disease increased, based on clinical features and laboratory tests, diagnosis of the disease is not difficult. Liver biopsy can be further necessary to prevent misdiagnosis, missed diagnosis