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

World Journal of Hepatology 2023年第9期15卷 1033-1042页

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 Liver Transplant Department Walter Cantídio Teaching HospitalFortaleza 60430270CearáBrazil School of Medicine University of FortalezaFortaleza 60811905CearáBrazil Hospital São José CearáState Health DepartmentFortaleza 60455610CearáBrazil Department of Surgery Federal University of CearáFortaleza 60430140CearáBrazil Surgery Department São Carlos HospitalFortaleza 60130241CearáBrazil

BACKGROUND Primary sclerosing cholangitis(PSC)manifests within a broad ethnic and racial spectrum,reflecting different levels of access to health *** To evaluate the clinical profile,complications and survival rates of patients with PSC undergoing liver transplantation(LTx)at a Brazilian reference *** All patients diagnosed with PSC before or after LTx were *** medical records were reviewed for demographic and clinical variables,including outcomes and *** level of statistical significance was set at P<*** Our cohort represented 1.6%(n=34)of the 2113 patients receiving liver grafts at our service over the past two *** 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 *** patients had the classic form of *** women had PSC/autoimmune hepatitis overlap syndrome,and one patient had small-duct *** phosphatase levels at diagnosis and pre-LTx model for end-stage liver *** were significantly higher in *** 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 *** overall median post-LTx survival was 66 *** 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 simil

关键词： Primary sclerosing cholangitis Epidemiology Liver transplantation Survivor Clinical associations Pathological features

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