肝豆状核变性的肝影像学表现

Imaging Manifestations of Liver in Hepatolenticular Degeneration

目的 :探讨肝豆状核变性的肝脏影像特征及早期诊断的可能性。方法 :对 17例有临床症状 ,经眼角膜检查、血铜检测、CT表现及进行驱铜治疗后所确诊的肝豆状核变性患者进行回顾性分析。结果 :17例中 15例有结节性肝硬化表现 ,1例出现肝脂肪变性伴门脉高压征象 ;肝内结节呈弥漫分布 ,稍高或高密度 ,边界清楚 ;随病程发展结节逐渐增大 ,密度增高 ,肝脏体积缩小。结论 :年青患者尤其是少儿期出现结节性肝硬化或脂肪变性 ,在排除病毒感染后应考虑本病的可能 ,肝影像学表现是早期诊断无神经系统锥体外系症状的肝豆状核变性的重要依据之一。

Objective:To investigate the imaging characteristics of liver and the feasibility of early diagnosis in hepatolenticular degeneration (HLD).Methods:Seventeen patients with HLD diagnosed by clinical symptoms,keratoscopy,serum copper concentration,imaging manifestations and effective therapy were analyzed retrospectively.Results:15 cases were found nodular cirrhosis and one case had liver degeneration with portal hypertension,These nodules were diffuse and multiple with well-defined margin and high or moderate density,the nodules became more bigger and more dense and the liver became more smaller with progress of the diease.Conclusion:HLD should be taken into account when nodular cirrhosis of liver degeneration without virus infections is found in young adults especially in chlidren.It's a valuable diagnostic evidence for HLD before the extrapyramidal system symptoms appear.It's easy to confirm the diagnosis of HLD when these abnormal imaging features are found in the liver and brain at the same time.