【摘要】 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、漏诊更多还原

【Abstract】 Aim:[WT5BZ]To improve clinical diagnosis and treatment of unknown origin hepatic disorder and hepatolenticular degeneration.[WT5HZ]Methods:[WT5BZ]Collecting 21 cases of Wilsons disease which mainly exhibit as hepatocyte lesion from our hospital during the past 8 years.Analyzing their changes of clinical and experimental characteristics.[WT5HZ]Results:[WT5BZ]The main characteristis of Wilson’s disease with hepatic disorder as the main expression can be concluded as follows:It attacks among both males and females.Nearly 90% occurences take place in age of 5-15(whereas few are observed outside this range).More than 80% indicates liver cirrhosis.Most of them have additional Visceral injuries in a certain degree,e.g.80% has anemai and 61.9% has symptom of extrapyramidal system,28.6% of this disease has family history.In discrimination and diagnosis ,it is significant to check the K F ring on cornea,to detect the Cu 2+ ,ceruloplasmin,copperoxidase in serum,and Cu 2+ in urine.[WT5HZ]Conclusion:[WT5BZ]Based on the aforementioned clinical and experimental charateristics,It is not diffcult to diagnose the Wilsons disease,especially for children,as long as enough patience and attention is paid.It is recommendable to perform live biopsy to ensure the diagnosis if necessary. [WT5HZ]更多还原