Open AccessCystic fibrosis associated liver disease in children
Author(s) -
Joseph Valamparampil,
Girish Gupte
Publication year - 2021
Publication title -
world journal of hepatology
Language(s) - Uncategorized
Resource type - Journals
SCImago Journal Rank - 0.913
H-Index - 55
ISSN - 1948-5182
DOI - 10.4254/wjh.v13.i11.1727
Subject(s) - medicine , cystic fibrosis , cystic fibrosis transmembrane conductance regulator , liver transplantation , disease , pathogenesis , liver disease , lung transplantation , regulator , lung , pathology , gastroenterology , bioinformatics , transplantation , gene , genetics , biology
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.