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Wilson病又名肝豆状核变性。临床以神经症状、肝症状及角膜色素环为三大主征。若以肝症状开始,则被误诊为肝炎者不少。作者对确诊的200例肝豆状核变性住院患者中有肝肿大及肝功能损害(其中3例尸检证明)的76例,分析如下。 临床资料 一、首发症状 本组有症状的198例临床型患者中,22例(11.11％)以肝症状起病较国内其他报道为少。 二、完成期的临床症状 本组仅79例有肝症状(38.38％)。76例中,男50例,女26例;15岁以下发病者35例(46.05％)。 三、肝脾肿大及腹水 肝脏肿大75例,脾脏肿大40例,腹水12例。其中4例误诊为门脉高压症而行脾切除。确诊前曾在外院误诊为急、慢性肝炎者
Wilson’s disease, also known as hepatolenticular degeneration. Clinical neurological symptoms, liver symptoms and corneal pigment ring for the three main symptoms. If the start of the liver symptoms, were misdiagnosed as hepatitis a lot. The authors identified 76 patients with hepatomegaly and impaired liver function (of which 3 were autopsy proven) of 200 patients with hepatolenticular degeneration confirmed as follows. Clinical data First, the first symptom? Of the 198 cases of clinical symptoms in this group, 22 cases (11.11%) of liver symptoms less than other reports of domestic. Second, the completion of the clinical symptoms of this group, only 79 cases of liver symptoms (38.38%). In 76 cases, there were 50 males and 26 females, and 35 cases (46.05%) were under 15 years of age. Third, hepatosplenomegaly and ascites liver enlargement in 75 cases, 40 cases of splenomegaly, ascites in 12 cases. 4 cases misdiagnosed as portal hypertension and splenectomy. Before the diagnosis of misdiagnosis in the hospital as acute and chronic hepatitis