2019
Pathophysiology of Cystic Fibrosis Liver Disease: A Channelopathy Leading to Alterations in Innate Immunity and in Microbiota
Fiorotto R, Strazzabosco M. Pathophysiology of Cystic Fibrosis Liver Disease: A Channelopathy Leading to Alterations in Innate Immunity and in Microbiota. Cellular And Molecular Gastroenterology And Hepatology 2019, 8: 197-207. PMID: 31075352, PMCID: PMC6664222, DOI: 10.1016/j.jcmgh.2019.04.013.Peer-Reviewed Reviews, Practice Guidelines, Standards, and Consensus StatementsConceptsCF-associated liver diseaseLiver diseaseCystic fibrosisInnate immunityCystic fibrosis liver diseaseEpithelial innate immunityCystic fibrosis transmembrane conductance regulatorFibrosis transmembrane conductance regulatorNonpulmonary causesCF adultsTransmembrane conductance regulatorLiver complicationsMutations of CFTRPediatric populationAltered microbiotaIntestinal diseaseBile secretionCF mortalityDiseaseNew drugsConductance regulatorPotential targetLife expectancyBasic defectPathophysiology
1998
Liver and Biliary Problems in Cystic Fibrosis
Colombo C, Battezzati P, Strazzabosco M, Podda M. Liver and Biliary Problems in Cystic Fibrosis. Seminars In Liver Disease 1998, 18: 227-235. PMID: 9773423, DOI: 10.1055/s-2007-1007159.Peer-Reviewed Original ResearchConceptsCF-associated liver diseaseLiver diseaseCF patientsCystic fibrosisEarly-stage liver diseaseEssential fatty acid statusExtra-hepatic causesBile duct damageStage liver diseaseBile acid therapyHepatic excretory functionBile acid compositionFatty acid statusUseful therapeutic approachImpaired secretory functionQuality of lifeSpecific CFTR mutationsCholangiocyte pathobiologyHepatic assessmentLiver biochemistryCumulative incidenceDuct damageLiver histologyBiliary problemsMajor complications