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Combined liver-kidney transplantation for rare diseases

Combined liver and kidney transplantation (CLKT) is indicated in patients with failure of both organs, or for the treatment of end-stage chronic kidney disease (ESKD) caused by a genetic defect in the liver. The aim of the present review is to provide the most up-to-date overview of the rare conditi...

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Autores principales: Knotek, Mladen, Novak, Rafaela, Jaklin-Kekez, Alemka, Mrzljak, Anna
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Baishideng Publishing Group Inc 2020
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7643210/
https://www.ncbi.nlm.nih.gov/pubmed/33200012
http://dx.doi.org/10.4254/wjh.v12.i10.722
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author Knotek, Mladen
Novak, Rafaela
Jaklin-Kekez, Alemka
Mrzljak, Anna
author_facet Knotek, Mladen
Novak, Rafaela
Jaklin-Kekez, Alemka
Mrzljak, Anna
author_sort Knotek, Mladen
collection PubMed
description Combined liver and kidney transplantation (CLKT) is indicated in patients with failure of both organs, or for the treatment of end-stage chronic kidney disease (ESKD) caused by a genetic defect in the liver. The aim of the present review is to provide the most up-to-date overview of the rare conditions as indications for CLKT. They are major indications for CLKT in children. However, in some of them (e.g., atypical hemolytic uremic syndrome or primary hyperoxaluria), CLKT may be required in adults as well. Primary hyperoxaluria is divided into three types, of which type 1 and 2 lead to ESKD. CLKT has been proven effective in renal function replacement, at the same time preventing recurrence of the disease. Nephronophthisis is associated with liver fibrosis in 5% of cases and these patients are candidates for CLKT. In alpha 1-antitrypsin deficiency, hereditary C3 deficiency, lecithin cholesterol acyltransferase deficiency and glycogen storage diseases, glomerular or tubulointerstitial disease can lead to chronic kidney disease. Liver transplantation as a part of CLKT corrects underlying genetic and consequent metabolic abnormality. In atypical hemolytic uremic syndrome caused by mutations in the genes for factor H, successful CLKT has been reported in a small number of patients. However, for this indication, CLKT has been largely replaced by eculizumab, an anti-C5 antibody. CLKT has been well established to provide immune protection of the transplanted kidney against donor-specific antibodies against class I HLA, facilitating transplantation in a highly sensitized recipient.
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spelling pubmed-76432102020-11-15 Combined liver-kidney transplantation for rare diseases Knotek, Mladen Novak, Rafaela Jaklin-Kekez, Alemka Mrzljak, Anna World J Hepatol Review Combined liver and kidney transplantation (CLKT) is indicated in patients with failure of both organs, or for the treatment of end-stage chronic kidney disease (ESKD) caused by a genetic defect in the liver. The aim of the present review is to provide the most up-to-date overview of the rare conditions as indications for CLKT. They are major indications for CLKT in children. However, in some of them (e.g., atypical hemolytic uremic syndrome or primary hyperoxaluria), CLKT may be required in adults as well. Primary hyperoxaluria is divided into three types, of which type 1 and 2 lead to ESKD. CLKT has been proven effective in renal function replacement, at the same time preventing recurrence of the disease. Nephronophthisis is associated with liver fibrosis in 5% of cases and these patients are candidates for CLKT. In alpha 1-antitrypsin deficiency, hereditary C3 deficiency, lecithin cholesterol acyltransferase deficiency and glycogen storage diseases, glomerular or tubulointerstitial disease can lead to chronic kidney disease. Liver transplantation as a part of CLKT corrects underlying genetic and consequent metabolic abnormality. In atypical hemolytic uremic syndrome caused by mutations in the genes for factor H, successful CLKT has been reported in a small number of patients. However, for this indication, CLKT has been largely replaced by eculizumab, an anti-C5 antibody. CLKT has been well established to provide immune protection of the transplanted kidney against donor-specific antibodies against class I HLA, facilitating transplantation in a highly sensitized recipient. Baishideng Publishing Group Inc 2020-10-27 2020-10-27 /pmc/articles/PMC7643210/ /pubmed/33200012 http://dx.doi.org/10.4254/wjh.v12.i10.722 Text en ©The Author(s) 2020. Published by Baishideng Publishing Group Inc. All rights reserved. http://creativecommons.org/licenses/by-nc/4.0/ This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial.
spellingShingle Review
Knotek, Mladen
Novak, Rafaela
Jaklin-Kekez, Alemka
Mrzljak, Anna
Combined liver-kidney transplantation for rare diseases
title Combined liver-kidney transplantation for rare diseases
title_full Combined liver-kidney transplantation for rare diseases
title_fullStr Combined liver-kidney transplantation for rare diseases
title_full_unstemmed Combined liver-kidney transplantation for rare diseases
title_short Combined liver-kidney transplantation for rare diseases
title_sort combined liver-kidney transplantation for rare diseases
topic Review
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7643210/
https://www.ncbi.nlm.nih.gov/pubmed/33200012
http://dx.doi.org/10.4254/wjh.v12.i10.722
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