实用肝脏病杂志Issue(5)：486-490,5.DOI:10.3969/j.issn.1672-5069.2015.05.010
肝豆状核变性与慢性乙型肝炎重度患者临床特征分析
Clinical features of patients with Wilson’s disease and hyperbilirubinemia：Comparison to patients with ;chronic hepatitis B of severe degree
摘要
Abstract
Objective To explore the clinical features of patients with Wilson’s disease and hyperbiliru-binemia. Method Twenty-four patients with Wilson’s disease and twenty-eight with chronic hepatitis B of severe degree were included in this study. The manifestation and routine laboratory tests were collected. The peripheral blood ATP7B gene mutations in exon 8,12,13 and 16 were analyzed by direct sequencing. Results The pa-tients with WD had the features of significantly younger (33.2 vs. 41.8 yr old,P=0.049),longer delay diagnosis time (8.2 vs. 1 month,P<0.001) as compared to patients with hepatitis B;the total urine copper in 24 hours was significantly elevated [(919.83±1017.15)μg vs. (204.79±191.85)μg,P<0.001];the patients with WD had signifi-cantly higher serum glutamyltranspeptidase level [(175.74±245.99) U/L] as compared with in patients with hepati-tis B[(133.44±115.95) U/L,P=0.004],which had a positive correlation with 24 hours total urine copper drainage (r=0.552,P=0.012);all the patients with WD were positive for ATP7B mutation,while only 4 patients with hep-atitis B positive. Conclusion Serum copper and ceruloplasmin levels may be useful for screening patients with WD and jaundice,and the 24 hour urine copper excretion is an important index for differential diagnosis from se-vere chronic hepatitis B.关键词
肝豆状核变性/黄疸/临床特征/鉴别诊断Key words
Wilson’s Disease/Hyperbilirubinemia/Clinical features/Differential diagnosis引用本文复制引用
陈韬,叶昊,方文敏,吴亮,黄元成..肝豆状核变性与慢性乙型肝炎重度患者临床特征分析[J].实用肝脏病杂志,2015,(5):486-490,5.基金项目
华中科技大学自主创新基金重点项目 （）
