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Cystic fibrosis associated liver disease in children

Cystic fibrosis (CF) is an autosomal recessive disorder caused by mutations in the CF transmembrane conductance regulator gene. CF liver disease develops in 5%-10% of patients with CF and is the third leading cause of death among patients with CF after pulmonary disease or lung transplant complicati...

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Detalles Bibliográficos
Autores principales: Valamparampil, Joseph J, Gupte, Girish L
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Baishideng Publishing Group Inc 2021
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8637674/
https://www.ncbi.nlm.nih.gov/pubmed/34904041
http://dx.doi.org/10.4254/wjh.v13.i11.1727
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author Valamparampil, Joseph J
Gupte, Girish L
author_facet Valamparampil, Joseph J
Gupte, Girish L
author_sort Valamparampil, Joseph J
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description Cystic fibrosis (CF) is an autosomal recessive disorder caused by mutations in the CF transmembrane conductance regulator gene. CF liver disease develops in 5%-10% of patients with CF and is the third leading cause of death among patients with CF after pulmonary disease or lung transplant complications. We review the pathogenesis, clinical presentations, complications, diagnostic evaluation, effect of medical therapies especially CF transmembrane conductance regulator modulators and liver transplantation in CF associated liver disease.
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spelling pubmed-86376742021-12-12 Cystic fibrosis associated liver disease in children Valamparampil, Joseph J Gupte, Girish L World J Hepatol Minireviews Cystic fibrosis (CF) is an autosomal recessive disorder caused by mutations in the CF transmembrane conductance regulator gene. CF liver disease develops in 5%-10% of patients with CF and is the third leading cause of death among patients with CF after pulmonary disease or lung transplant complications. We review the pathogenesis, clinical presentations, complications, diagnostic evaluation, effect of medical therapies especially CF transmembrane conductance regulator modulators and liver transplantation in CF associated liver disease. Baishideng Publishing Group Inc 2021-11-27 2021-11-27 /pmc/articles/PMC8637674/ /pubmed/34904041 http://dx.doi.org/10.4254/wjh.v13.i11.1727 Text en ©The Author(s) 2021. Published by Baishideng Publishing Group Inc. All rights reserved. https://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. See: http://creativecommons.org/Licenses/by-nc/4.0/
spellingShingle Minireviews
Valamparampil, Joseph J
Gupte, Girish L
Cystic fibrosis associated liver disease in children
title Cystic fibrosis associated liver disease in children
title_full Cystic fibrosis associated liver disease in children
title_fullStr Cystic fibrosis associated liver disease in children
title_full_unstemmed Cystic fibrosis associated liver disease in children
title_short Cystic fibrosis associated liver disease in children
title_sort cystic fibrosis associated liver disease in children
topic Minireviews
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8637674/
https://www.ncbi.nlm.nih.gov/pubmed/34904041
http://dx.doi.org/10.4254/wjh.v13.i11.1727
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