25例Gilbert综合征患者临床及肝组织病理特点分析

Clinical and pathological features of 25 patients with Gilbert syndrome

目的总结分析Gilbert综合征患者临床及肝组织病理特点。方法回顾性分析2006-2008年我院收治的25例Gilbert综合征患者的临床及肝脏病理资料。结果 25例患者中男性17例(68%),发病年龄18～30岁者15例(60%);合并HBV感染(4例)、药物性肝炎(2例)、酒精性肝炎(1例)等常见肝病7例(28%);多数病例临床症状轻微,17例(68%)表现为黄疸、疲乏,2例(8%)脾大。按照发病年龄分为<18岁组,18～30岁组,>30岁组,其总胆红素分别为121.4±27.9μmol/L、56.6±13.8μmol/L、60.7±28.1μmol/L,间接胆红素分别为87.9±35.4μmol/L、42.9±27.0μmol/L、42.0±31.6μmol/L,黄疸分布与发病年龄呈负相关(P<0.01)。光镜下,100%患者肝脏组织可见中央静脉周围肝细胞胞浆内棕褐色颗粒沉积。结论 Gilbert综合征青壮年男性多见,临床表现间接胆红素上升为主的黄疸,青少年发病可以表现为中重度黄疸;其肝脏病理可见中央静脉周围肝细胞胞浆内棕褐色颗粒沉积;常见肝病原因(如HBV感染,药物性/酒精性肝炎)合并Gilbert综合征值得临床重视。

Objectives To study the clinical and pathological features of Gilbert syndrome.Methods The clinical and pathological data of 25 patients with Gilbert syndrome were retrospectively analyzed.Results 17 cases（68%） were males.15 cases（60%） aged 18 to 30 years old,7 cases（29%） were complicated with other liver diseases（4 cases with hepatitis B,2 with drug-induced liver injury and 1 with alcoholic hepatitis）.Most cases had mild symptoms,17 cases（68%） with jaundice and fatigue,2 cases（8%） with splenomegaly.The total bilirubin were 121.4±27.9μmol/L,56.6±13.8μmol/L,60.7±28.1μmol/L and the indirect bilirubin were 87.9±35.4μmol/L,42.9±27.0μmol/L,42.0±31.6μmol/L in patients aged less than 18 years,18 to 30 years and older than 30 years,respectively.The bilirubin had an inverse relation with age（P0.01）.Dark brown granules around the central vein were observed under light microscope in all cases.Conclusions Gilbert syndrome was more commonly seen in young males and the indirect bilirubin elevated remarkably.The pathological examination revealed dark brown granules around the central vein.This disease is often complicated with hepatitis B,drug-induced liver injury and alcoholic hepatitis.