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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。
Objective To summarize the clinical and hepatic histopathological features of patients with Gilbert’s syndrome. Methods The clinical and liver pathology data of 25 patients with Gilbert’s syndrome treated in our hospital from 2006 to 2008 were retrospectively analyzed. Results Among the 25 patients, 17 (68%) were male and 15 (60%) were aged 18-30. HBV infection (4), drug induced hepatitis (2), alcoholic hepatitis (1) Seven cases (28%) were common liver diseases. The clinical symptoms were mild in most cases. Jaundice and fatigue were found in 17 cases (68%), and splenomegaly was found in 2 cases (8%). The total bilirubin was 121.4 ± 27.9μmol / L, 56.6 ± 13.8μmol / L and 60.7 ± 28.1μmol / L, respectively, according to the age of onset in the group <18 years old, 18-30 years old, and> 30 years old Bilirubin were 87.9 ± 35.4μmol / L, 42.9 ± 27.0μmol / L and 42.0 ± 31.6μmol / L, respectively. The distribution of jaundice was negatively correlated with the age of onset (P <0.01). Light microscopy, 100% of patients with liver tissue visible around the central vein hepatocytes cytoplasm tan particles deposition. Conclusions Gilbert syndrome is more common in young and middle-aged males, with clinical manifestations of jaundice, which is mainly indirect bilirubin rise. Juvenile onset may be moderate to severe jaundice. The liver pathology shows the deposition of brown granules in the cytoplasm of hepatocytes around the central veins. Common liver diseases Cause (such as HBV infection, drug / alcoholic hepatitis) Gilbert syndrome merits clinical attention.