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肝豆状核变性又称wilson病,其发病率为6～10/100万,但临床并不少见。由于其临床表现多样,误诊率高。本文报告16例误诊教训,以为鉴戒。 临床资料 16例中男10例,女6例;发病年龄8岁～15岁。其中1例有肝病家族史,均无明确肝豆状核变性家族史。首次就诊时间最短6天,最长4年,发病至确诊时间为2个月～4年。主要症状和体征:以肝脏损害为首发者7例,表现为腹胀、腹水6例(其中伴下肢浮肿4例,发热、腹痛2例),黄疸7例,肝肿大5例,脾肿大4例,消化道出血2例。以神经系统损害为首发症状者5例,手震颤2例,语言障碍(语言
Wilson’s disease, also known as wilson disease, the incidence of 6 to 10/1 million, but the clinical is not uncommon. Due to its diverse clinical manifestations, the misdiagnosis rate is high. This article reports 16 cases of misdiagnosis lessons, that learning. 16 cases of clinical data in 10 males and 6 females; the age of onset of 8 years old to 15 years. One case of a family history of liver disease, no clear family history of Wilson’s disease. The first visit to the shortest 6 days, up to 4 years, the onset to the diagnosis of time is 2 months to 4 years. The main symptoms and signs: Liver damage as the first seven cases, manifested as abdominal distension, ascites in 6 cases (including 4 cases of edema in lower limbs, fever, abdominal pain in 2 cases), jaundice in 7 cases, hepatomegaly in 5 cases, splenomegaly Cases, gastrointestinal bleeding in 2 cases. 5 cases of neurological damage as the first symptom, hand tremor in 2 cases, language disorders (language