CAJ | 학술논문

目的：报告21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21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61.928.6％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。
목적：보고21례이간손위주간두상핵변성，진일보제고원인불명간병화간두상핵변성진치공작。방법：수집아원근8년래21례이간손위주적간두상핵변성，대기림상화실험검사개변특정진행총결、분석。결과：이간손위주간두상핵변성유이하특점：본병남、녀균가발생，주요재5～15세발병，근90％，소우5세급15세이상소견。간손해중간경화점80％이상。절대다수유불동정도적간외장기수손，여빈혈점80.9％，추체외계증상점61.928.6％유가족사。각막K-F배，혈청동，동란단백，동양화매，뇨동측정등균유중요진단、감별의의。결론：대원인불명간병，특별시소인간병，지요제고대본병인식，근거림상특정화실험검사，진단해병병무곤난。필요시가작간활검진일보방지오진、루진。
Aim：To improve clinical diagnosis and treatment of unknown origin hepatic disorder and hepatolenticular degeneration．Methods：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．Results：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 Cu2＋，ceruloplasmin，copperoxidase in serum，and Cu2＋ in urine．Conclusion：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．