肝豆状核变性并发肝硬变40例临床分析

Hepatolenticular degeneration complicated with hepatocirrhosis:a clinical analysis of 40 cases

目的 :了解肝豆状核变性引起肝硬变患者的临床特点和诊治情况。方法 :回顾 4 0例肝豆状核变性临床资料 ,对合并肝硬变、脾功能亢进和门静脉高压症的情况进行分析和比较。结果 :① 4 0例中 2 1例以肝功能受损为首发症状。②肝脏受累的患者较年轻 ,平均年龄为 2 0 .3岁 ;神经系统受累的平均年龄为 2 3岁(P <0 .0 5 )。③肝脏大小平均为肋下 2 .2cm ,脾脏大小平均为肋下 2 .7cm ,WBC为 2 .8× 10 9/L ,PLT为 4 6×10 9/L。④X线钡餐或胃镜检查提示食管静脉曲张阳性率为 2 7.5 % ( 11/ 4 0 ) ,4 0例中发生上消化道出血者 3例( 7.5 % ) ,均有肝硬变表现 ,其中大出血 1例。结论 :肝硬变、脾功能亢进和门静脉高压症是本病最常见的合并症 ,脾切除手术能改善肝功能 。

Objective:To evaluate clinical feature,diagnosis and treatment of hepatolenticular degeneration with hepatocirrhosis.Methods:Retrospective analysis was made in 40 patients with a diagnosis of hepatolenticular degeneration with hepatic cirrhosis,hypersplenism,and/or portal hypertension.Results:①Hepatic function injury was the initial symptom in 21 of 40 patients.②The age of patients with hepatic function injury were younger(average 20.3 years old) than that of those with involvement of the nervous system(average 23 years old,P<0.05).③Liver was average 2.2 cm below subcostal margin,but spleen was average 2.7 cm below subcostal margin;WBC and PLT were 2.8×10 9/L and 46×10 9/L,respectively.④Esophageal varicosis was found in 11 patients(27.5%) through barium swallow test or fiberoptic gastroscopy,3(7.5%) patients with hepatocirrhosis were associated with upper alimentary tract hemorrhage,1 of whom presented with massive bleeding.Conclusion:Hepatic cirrhosis,hypersplenism,and portal hypertension are the most common complications of hepatolenticular degeneration,splenectomy can improve hepatic function and elevate the curative effect of hepatolenticular degeneration.