肝移植治疗Gilbert综合征合并急性戊型肝炎导致的肝衰竭1例报告并文献复习  

Liver transplantation for liver failure due to Gilbert syndrome complicated with acute hepatitis E:a case report and review of literature

作　　者：张晓晶 金巧菲 刘寿荣 ZHANG Xiao-jing;JIN Qiao-fei;LIU Shou-rong(Hangzhou Xixi Hospital,Hangzhou,Zhejiang 310023,China)

机构地区：[1]杭州市西溪医院肝病二科,浙江杭州310023

出　　处：《中华医院感染学杂志》2025年第2期215-218,共4页Chinese Journal of Nosocomiology

基　　金：国家“十三五”艾滋病和病毒性肝炎等重大传染病防治专项子课题(2017ZX10302201-001);杭州市生物医药和健康产业发展扶持科技专项项目(2021WJCY366)。

摘　　要：目的总结Gilbert综合征合并急性戊型肝炎的临床表现、检验结果、病理特点、诊断及治疗,提高对Gilbert综合征合并病毒性肝炎的警觉和认识,减少漏诊和误诊。方法2022年10月14日杭州市西溪医院收治了一例以“乏力、肤黄尿黄1月”为主诉入院,症状进行性加重,肝功能进行性恶化,起初考虑急性黄疸型戊型病毒性肝炎,经保肝退黄药物、激素冲击治疗及7次人工肝支持治疗均不见好转。结果在排除其他病毒性肝炎、自身免疫性肝炎、药物性肝炎、肝内外梗阻等情况后,追问病史,患者自诉近数十年总胆红素一直波动在40~50μmol/L,以间接胆红素增高为主,但当时未查明原因,也未予关注,且其哥哥也一直有总胆红素轻度升高的情况,也以间接胆红素升高为主。最终通过基因检测确认患者基础即患有Gilbert综合征,最后通过肝移植治疗后得以康复。结论在Gilbert综合征的基础上合并病毒性肝炎容易引发肝衰竭,在临床诊疗中,应拓宽思路,甚至是罕见疾病导致患者黄疸的可能性,并加强对此病的警惕和认识,为患者做出更好地诊治。OBJECTIVE To summarize the clinical manifestations,results of clinical laboratory tests,pathological characteristics,diagnosis and treatment of the Gilbert syndrome patient complicated with acute hepatitis E so as to raise the awareness of Gilber syndrome complicated with viral hepatitis and reduce the missed diagnosis and misdiagnosis.METHODS One patient wad admitted to Hangzhou Xixi Hospital by chief complaints of′fatigue,yellow skin and yellow urine for 1 month′on October 14,2022.The symptom of the patient was progressively aggravated,the liver function was progressively exacerbated.The patient was initially considered as acute jaundice viral hepatitis E and was treated with liver protection and jaundice-removing drugs,hormone impact therapy and 7 times of artificial liver support therapy,and the symptom was not improved.RESULTS Other types of viral hepatitis,autoimmune hepatitis,drug-induced hepatitis and intrahepatic and extrahepatic obstruction were excluded,the patient told the medical history:the total bilirubin maintains fluctuating between 40 and 50μmol/L for several decades,dominating by the rise of indirect bilirubin,the causes failed to be identified,the brother of the patient always presents with slight rise of total bilirubin,also dominating by the rise of indirect bilirubin.The patient was ultimately diagnosed with primary Gilbert syndrome by genetic testing and was cured after liver transplantation.CONCLUSION The primary Gilbert syndrome complicated with viral hepatitis is prone to inducing the liver failure.It is necessary to broaden thinking during the clinical diagnosis and treatment,especially the possibility of jaundice induced by rare diseases,and intensify the vigilance and understanding of the disease so as to provide better diagnosis and treatment for the patient.

关 键 词：GILBERT综合征 总胆红素 间接胆红素 戊型病毒性肝炎 肝衰竭 

分 类 号：R512.6[医药卫生—内科学]