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
2011
Polycystic Liver Diseases: Congenital Disorders of Cholangiocyte Signaling
Strazzabosco M, Somlo S. Polycystic Liver Diseases: Congenital Disorders of Cholangiocyte Signaling. Gastroenterology 2011, 140: 1855-1859.e1. PMID: 21515270, PMCID: PMC3109236, DOI: 10.1053/j.gastro.2011.04.030.Peer-Reviewed Original ResearchConceptsPolycystic liver diseaseLiver cyst formationClinical featuresLiver diseaseMultiple cystsDisease progressionBiliary epitheliumLiver parenchymaProgressive enlargementCongenital diseaseCyst formationCholangiocyte physiologyCongenital disorderPotential targetGenetic defectsDiseaseProgressionDisordersInheritance patternSignalingIntracellular organellesDifferent entitiesTherapyKidneyPathway