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Pretransplant Evaluation and Liver Transplantation Outcome in PBC Patients

Primary biliary cholangitis (PBC) is an autoimmune chronic cholestatic liver disease characterized by progressive cholangiocyte and bile duct destruction leading to fibrosis and finally to liver cirrhosis. The presence of disease-specific serological antimitochondrial antibody (AMA) together with el...

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Autores principales: Mijic, Maja, Saric, Ivona, Delija, Bozena, Lalovac, Milos, Sobocan, Nikola, Radetic, Eva, Martincevic, Dora, Filipec Kanizaj, Tajana
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Hindawi 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9337972/
https://www.ncbi.nlm.nih.gov/pubmed/35910038
http://dx.doi.org/10.1155/2022/7831165
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author Mijic, Maja
Saric, Ivona
Delija, Bozena
Lalovac, Milos
Sobocan, Nikola
Radetic, Eva
Martincevic, Dora
Filipec Kanizaj, Tajana
author_facet Mijic, Maja
Saric, Ivona
Delija, Bozena
Lalovac, Milos
Sobocan, Nikola
Radetic, Eva
Martincevic, Dora
Filipec Kanizaj, Tajana
author_sort Mijic, Maja
collection PubMed
description Primary biliary cholangitis (PBC) is an autoimmune chronic cholestatic liver disease characterized by progressive cholangiocyte and bile duct destruction leading to fibrosis and finally to liver cirrhosis. The presence of disease-specific serological antimitochondrial antibody (AMA) together with elevated alkaline phosphatase (ALP) as a biomarker of cholestasis is sufficient for diagnosis. Ursodeoxycholic acid (UDCA) is the first treatment option for PBC. Up to 40% of patients have an incomplete response to therapy, and over time disease progresses to liver cirrhosis. Several risk scores are proposed for better evaluation of patients before and during treatment to stratify patients at increased risk of disease progression. GLOBE score and UK PBC risk score are used for the evaluation of UDCA treatment and Mayo risk score for transplant-free survival. Liver transplantation (LT) is the only treatment option for end-stage liver disease. More than 10 years after LT, 40% of patients experience recurrence of the disease. A liver biopsy is required to establish rPBC (recurrent primary biliary cholangitis). The only treatment option for rPBC is UDCA, and data show biochemical and clinical improvement, plus potential beneficial effects for use after transplantation for the prevention of rPBC development. Additional studies are required to assess the full impact of rPBC on graft and recipient survival and for treatment options for rPBC.
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spelling pubmed-93379722022-07-30 Pretransplant Evaluation and Liver Transplantation Outcome in PBC Patients Mijic, Maja Saric, Ivona Delija, Bozena Lalovac, Milos Sobocan, Nikola Radetic, Eva Martincevic, Dora Filipec Kanizaj, Tajana Can J Gastroenterol Hepatol Review Article Primary biliary cholangitis (PBC) is an autoimmune chronic cholestatic liver disease characterized by progressive cholangiocyte and bile duct destruction leading to fibrosis and finally to liver cirrhosis. The presence of disease-specific serological antimitochondrial antibody (AMA) together with elevated alkaline phosphatase (ALP) as a biomarker of cholestasis is sufficient for diagnosis. Ursodeoxycholic acid (UDCA) is the first treatment option for PBC. Up to 40% of patients have an incomplete response to therapy, and over time disease progresses to liver cirrhosis. Several risk scores are proposed for better evaluation of patients before and during treatment to stratify patients at increased risk of disease progression. GLOBE score and UK PBC risk score are used for the evaluation of UDCA treatment and Mayo risk score for transplant-free survival. Liver transplantation (LT) is the only treatment option for end-stage liver disease. More than 10 years after LT, 40% of patients experience recurrence of the disease. A liver biopsy is required to establish rPBC (recurrent primary biliary cholangitis). The only treatment option for rPBC is UDCA, and data show biochemical and clinical improvement, plus potential beneficial effects for use after transplantation for the prevention of rPBC development. Additional studies are required to assess the full impact of rPBC on graft and recipient survival and for treatment options for rPBC. Hindawi 2022-07-22 /pmc/articles/PMC9337972/ /pubmed/35910038 http://dx.doi.org/10.1155/2022/7831165 Text en Copyright © 2022 Maja Mijic et al. https://creativecommons.org/licenses/by/4.0/This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
spellingShingle Review Article
Mijic, Maja
Saric, Ivona
Delija, Bozena
Lalovac, Milos
Sobocan, Nikola
Radetic, Eva
Martincevic, Dora
Filipec Kanizaj, Tajana
Pretransplant Evaluation and Liver Transplantation Outcome in PBC Patients
title Pretransplant Evaluation and Liver Transplantation Outcome in PBC Patients
title_full Pretransplant Evaluation and Liver Transplantation Outcome in PBC Patients
title_fullStr Pretransplant Evaluation and Liver Transplantation Outcome in PBC Patients
title_full_unstemmed Pretransplant Evaluation and Liver Transplantation Outcome in PBC Patients
title_short Pretransplant Evaluation and Liver Transplantation Outcome in PBC Patients
title_sort pretransplant evaluation and liver transplantation outcome in pbc patients
topic Review Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9337972/
https://www.ncbi.nlm.nih.gov/pubmed/35910038
http://dx.doi.org/10.1155/2022/7831165
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