Clinical analysis of 96 patients with Gilbert syndrome
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摘要: 目的总结Gilbert综合征的临床特点,便于诊断、鉴别诊断及治疗。方法总结了经肝组织活检明确诊断的Gilbert综合征96例,从家族史、临床表现、实验室检查、病理变化及治疗等多方面进行分析。结果患者中男85例,女11例。6.1%有明确家族史,70%发病年龄在10~30岁,其主要症状是皮肤巩膜黄染、尿黄。肝功能示:总胆红素(50.5±18.24)μmol/L,间接胆红素(36.19±16.86)μmol/L,90%患者ALT正常。肝组织病理显示肝细胞轻度水肿及少量炎性细胞浸润,肝细胞内可见棕黄色色素颗粒沉积。超声主要表现为肝脏回声粗欠均匀、脾大和慢性肝损害。应用苯巴比妥治疗有效率高于一般保肝治疗。结论 Gilbert综合征多见于青少年,男性多见。症状以黄染为主,胆红素轻中度升高,以间接胆红素升高为主。病理变化轻微,苯巴比妥疗效确切。
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关键词:
- 吉尔伯特病
Abstract: Objective To summarize the clinical features of Gilbert syndrome (GS) and to improve the diagnosis and treatment level.Methods The family history, clinical manifestations, laboratory examinations and pathological changes of hepatic tissues in 96 patients with GS diagnosed by liver biopsy were analyzed.Results The ratio of male to female is 85∶ 11;6.1% of GS patients have definite family history and 70% of them fall ill between 10-30 years old.The main symptoms of GS are skin and sclera yellow or dark urine and fatigue.Liver function test shows that total bilirubin is (50.5±18.24) μmol/L and the unconjugated bilirubin is (36.19±16.86) μmol/L.Total bilirubin of 97% patients are less than 85.5μmol/L and alanine aminotransferase (ALT) levels of 90% patients are normal.Liver pathological changes are mild with hepatocytes dropsy and denaturation, bile pigments depositing in the hepatocytes and inflammatory cells invading into the liver.Ultrasound visualize that the resonance of livers are coarse and poorly-distributed in 37 paients, splenomegaly and chronic hepatic lesion are shown in 30 and 23 patients respectively.The phenobarbital treatment is more effective than genera treatment.Conclusion GS attacks mainly adolescents, most of them are male.The levels of total bilirubin in GS patients increase less than 5 times upper limit of normal and unconjugated bilirubin rise mainly.Liver pathological changes of GS patients are mild.Phenobarbital is effective.-
Key words:
- Gilbert disease
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