不同年龄段肝豆状核变性患者肝损害的超声表现

Ultrasonic manifestations of liver damage in patients with Wilson’s disease at different ages

目的 分析不同年龄段肝豆状核变性患者的声像图表现,总结其肝损害的超声特征,为临床诊断及治疗提供依据。方法 选取肝豆状核变性患者247例,根据年龄分为未成年组(≤17岁)59例、青年组(18~44岁)168例及中老年组(≥45岁)20例。所有患者均行腹部超声检查观测肝脏及脾脏形态、大小、包膜、实质回声、有无腹腔积液,以及肝内血管、门静脉、脾静脉走行及内径。由两名具有5年及10年以上检查经验的超声医师分别对图像进行独立分析并根据其肝损害超声表现进行分型;采用Kappa检验分析两名超声医师判定结果的一致性,比较各组肝损害超声分型的差异。结果 两名超声医师判断各组肝损害超声分型的一致性较好(Kappa=0.717,P<0.001)。入组247例肝豆状核变性患者中,肝损害超声分型主要为肝纤维化或早期肝硬化型(43.72%)、晚期肝硬化型(41.30%)。未成年组、青年组及中老年组肝损害超声分型比较差异有统计学意义(P<0.05);其中青年组和中老年组分别以肝纤维化或早期肝硬化型、晚期肝硬化型为主(43.45%、48.21%和45.00%、55.00%),且晚期肝硬化型占比均高于未成年组,差异均有统计学意义(均P<0.05);未成年组以脂肪肝型、肝纤维化或早期肝硬化型为主(25.42%、44.07%),其中脂肪肝型占比高于青年组和中老年组,差异均有统计学意义(均P<0.05)。结论 不同年龄段肝豆状核变性患者的肝损害超声表现有一定特征,未成年患者肝损害分型以脂肪肝型、肝纤维化或早期肝硬化型为主,青年、中老年患者肝损害分型以肝纤维化或早期肝硬化型、晚期肝硬化型为主。

Objective To analyze the ultrasonic manifestations in patients with Wilson’s disease(WD)at different ages,to summarize the ultrasonic characteristics of liver damage,and to provide reference for clinical diagnosis and treatment.Methods A total of 247 WD patients were selected and divided into the juvenile group(≤17 years old,59 cases),the young group(18~44 years old,168 cases)and the middle-old-aged group(≥45 years old,20 cases)according to age.All patients underwent abdominal ultrasonography to observe the shape,size,capsule and parenchymal echo of liver and spleen,as well as the course and inner diameter of hepatic,portal and splenic veins.Two sonographers with more than 5 and 10 years of examination experience independently analyzed the images and classified the liver damage according to the ultrasonic manifestations.Kappa test was used to analyze the consistency of the judgment results of the two sonographers,and the differences of ultrasonic classification of liver damage among all groups were compared.Results The consistency of ultrasonic classification of liver damage judged by two sonographers in each group was good(Kappa=0.717,P<0.001).Among the 247 WD patients,the main ultrasonic manifestations of liver damage were liver fibrosis or early cirrhosis(43.72%)and late cirrhosis(41.30%).There were significant differences in ultrasonic manifestations of liver damage among juvenile group,the young group and middle-old-aged group(all P<0.05).The ultrasonic manifestations of liver damage in the young group and middle-old-aged group were mainly liver fibrosis or early cirrhosis and late cirrhosis(43.45%,48.21%and 45.00%,55.00%),and the proportion of late cirrhosis in the two groups were higher than that in the juvenile group,and the differences were statistically significant(both P<0.05).The ultrasonic manifestations of liver damage in juvenile group were mainly fatty liver type,liver fibrosis or early cirrhosis type(25.42%and 44.07%),and the proportion of fatty liver type was higher than that in the young group and the middle-old-aged group,and the differences were statistically significant(both P<0.05).Conclusion The ultrasonic manifestations of liver damage in patients with WD at different ages have certain characteristics.The juvenile patients mainly have fatty liver,liver fibrosis or early cirrhosis of liver damage,and the young and middle-old-aged patients mainly have liver fibrosis or early cirrhosis and late cirrhosis of liver damage.