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。
Aim:[WT5BZ]To improve clinical diagnosis and treatment of unknown origin hepatic disorder and hepatolenticular degeneration.[WT5HZ]Methods:[WT5BZ]Collecting 21 cases of Wilsons 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 Wilsons 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]
出处
《河北医学》
CAS
2001年第1期38-40,共3页
Hebei Medicine
