Liver transplant in patients with primary sclerosing cholangitis:A retrospective cohort from Northeastern Brazil  

作　　者：Louyse Teixeira de Souza Freitas Elodie Bomfim Hyppolito Victor Leonardo Barreto Luiz Humberto JataíCastelo Júnior Bianca Carneiro de Melo Jorge Frederico César Tahim de Sousa Brasil Háteras Malthus Barbosa Marzola Clébia Azevedo Lima Raquel Mendes Celedonio Gustavo Rêgo Coelho Jose Huygens Parente Garcia 

机构地区：[1]Liver Transplant Department,Walter Cantídio Teaching Hospital,Fortaleza 60430270,Ceará,Brazil [2]School of Medicine,University of Fortaleza,Fortaleza 60811905,Ceará,Brazil [3]Hospital São José,CearáState Health Department,Fortaleza 60455610,Ceará,Brazil [4]Department of Surgery,Federal University of Ceará,Fortaleza 60430140,Ceará,Brazil [5]Surgery Department,São Carlos Hospital,Fortaleza 60130241,Ceará,Brazil

出　　处：《World Journal of Hepatology》2023年第9期1033-1042,共10页世界肝病学杂志（英文版）（电子版）

基　　金：reviewed and approved by the ethics committee of the Federal University of Cearáand filed under Approval No.98627218.6.2018.5045.

摘　　要：BACKGROUND Primary sclerosing cholangitis(PSC)manifests within a broad ethnic and racial spectrum,reflecting different levels of access to health care.AIM To evaluate the clinical profile,complications and survival rates of patients with PSC undergoing liver transplantation(LTx)at a Brazilian reference center.METHODS All patients diagnosed with PSC before or after LTx were included.The medical records were reviewed for demographic and clinical variables,including outcomes and survival.The level of statistical significance was set at P<0.05.RESULTS Our cohort represented 1.6%(n=34)of the 2113 patients receiving liver grafts at our service over the past two decades.Most were male(n=19;56%).The average age(40±14 years)was similar for men and women(P=0.347).The mean follow-up time from diagnosis to LTx was 68 mo.Most patients had the classic form of PSC.Three women had PSC/autoimmune hepatitis overlap syndrome,and one patient had small-duct PSC.Alkaline phosphatase levels at diagnosis and pre-LTx model for end-stage liver disease.scores were significantly higher in males.Inflammatory bowel research(IBD)was investigated by colonoscopy in 26/34(76%)and was present in most cases(18/26;69%).IBD was less common in women than in men(44.4%vs.55.6%)(P=0.692).Cholangiocarcinoma(CCA)was diagnosed in 2/34(5.9%)patients by histopathology of the explant(survival:3 years 6 mo,and 4 years 11 mo).Two patients had complications requiring a second LTx(one after 7 d due to hepatic artery thrombosis and one after 17 d due to primary graft dysfunction).Five patients(14.7%)developed biliary stricture.The overall median post-LTx survival was 66 mo.Most deaths occurred in the first year(infection n=2,primary liver graft dysfunction n=3,unknown cause n=1).The 1-year and 5-year survival rates of this cohort were 82.3%and 70.6%,respectively,matching the mean overall survival rates of LTx patients at our center(87.1%and 69.43%,respectively)(P=0.83).CONCLUSION Survival after 1 and 5 years was similar to that of other LTx indications.The observe

关 键 词：Primary sclerosing cholangitis EPIDEMIOLOGY Liver transplantation SURVIVOR Clinical associations Pathological features 

分 类 号：R725.7[医药卫生—儿科]