Cystic fibrosis associated liver disease in children

Autor: Girish L Gupte, Joseph J Valamparampil
Jazyk: angličtina
Rok vydání: 2021
Předmět:
Zdroj: World Journal of Hepatology
ISSN: 1948-5182
Popis: 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.
Databáze: OpenAIRE