先天性肝纤维化致肝功能衰竭行肝移植术1例报告及文献复习

Liver transplantation for congenital hepatic fibrosis-induced liver function failure:A case report and literature review

目的:分析先天性肝纤维化(CHF)患者的临床特点、典型病理特征和治疗方法,提高该类疾病患者的预后。方法:收集1例CHF因肝功能衰竭行肝移植术患者的临床资料,结合相关文献,分析CHF的分型、临床特点、诊断和治疗。结果:患者,39岁,男性,以上消化道出血起病,曾行胃镜提示食管静脉重度曲张,以门静脉高压为突出表现;入院后影像学检查提示肝硬化、脾肿大和右肾囊肿;肝功能改变不明显,凝血功能差,并发肝功能衰竭,经内科治疗后病情一直无好转,随后该患者行同种异体原位肝移植术。术后肝脏病理诊断为CHF。患者移植术后1个月复查各项实验室指标均正常,术后随访1年患者恢复较好。结论:CHF以肝纤维化、门静脉高压症和肾囊性病变为特征。肝穿刺活检为CHF诊断的金标准,临床治疗主要是针对并发症,肝移植术是根本治疗方法。

Objective:To analyze the clinical characteristics,the typical pathological features and treatment methods of the patients with congenital hepatic fibrosis(CHF),and to improve the prognosis of the patients.Methods:The clinical data of a patient with CHF underwent liver transplantation for liver function failure were collected,and the relevant literatures were reviewed;the classification,clinical characteristics,diagnosis and treatment of CHF were analyzed.Results:The patient was a 39-year-old man and prensented with upper gastrointestinal bleeding.The results of gastroscope examination showed the severe varicose veins of the esophagus,which was marked by portal hypertension.The imaging examination after admission showed liver cirrhosis,splenomegaly and right renal cyst;the patient had mild liver function change,poor coagulation function,and liver function failure.After medical treatment,the condition was not improved,and the patient underwent allogeneic orthotopic liver transplantation.The postoperative liver pathology indicated CHF;all the laboratory indicators were normal after 1 month of transplantation.The patient recovered better 1 year after operation.Conclusion:CHF is characterized by liver fibrosis,portal hypertension and renal cystic lesions.Liver biopsy is the gold standard for its diagnosis,clinical treatment is mainly for the complications,and liver transplantation is the fundamental treatment.