2024
Portal Fibrosis and the Ductular Reaction: Pathophysiological Role in the Progression of Liver Disease and Translational Opportunities
Gupta V, Sehrawat T, Pinzani M, Strazzabosco M. Portal Fibrosis and the Ductular Reaction: Pathophysiological Role in the Progression of Liver Disease and Translational Opportunities. Gastroenterology 2024 PMID: 39251168, DOI: 10.1053/j.gastro.2024.07.044.Peer-Reviewed Original ResearchLiver diseasePortal fibrosisDuctular reactionPathological repairProgression of liver diseaseCholestatic liver diseaseTranslational opportunitiesChronic liver diseaseProgression of fibrosisCell typesChronic human liver diseaseHuman liver diseaseTumor microenvironmentTherapeutic advancesImmune modulationHistological abnormalitiesVascular changesLiver repairPathophysiological roleFibrosisTreatment prospectsPortal spacesMesenchymal cellsVascular cellsComplex crosstalkProminent role of gut dysbiosis in the pathogenesis of cystic fibrosis-related liver disease in mice
Bertolini A, Nguyen M, Zehra S, Taleb S, Bauer-Pisani T, Palm N, Strazzabosco M, Fiorotto R. Prominent role of gut dysbiosis in the pathogenesis of cystic fibrosis-related liver disease in mice. Journal Of Hepatology 2024, 81: 429-440. PMID: 38554847, PMCID: PMC11347101, DOI: 10.1016/j.jhep.2024.03.041.Peer-Reviewed Original ResearchCystic fibrosis-related liver diseaseCystic fibrosis transmembrane conductance regulatorCFTR-KO miceDefective cystic fibrosis transmembrane conductance regulatorCFTR-KOIntestinal permeabilityLiver diseaseGut-liver axisGut dysbiosisIncreased morbidityMortality of CF patientsAssociated with increased intestinal permeabilityLiver pathologyDevelopment of cholangiopathyCftr-knockout miceTransmembrane conductance regulatorIncreased intestinal permeabilityTargeted therapeutic strategiesFecal microbiota transferAttenuates liver diseaseExcessive inflammatory responseFITC-dextran assayPresence of neutrophilsActivation of pro-inflammatoryCFTR-knockoutEuropean Society for Organ Transplantation (ESOT) Consensus Statement on Outcome Measures in Liver Transplantation According to Value-Based Health Care
Carbone M, Neuberger J, Rowe I, Polak W, Forsberg A, Fondevila C, Mantovani L, Nardi A, Colli A, Rockell K, Schick L, Cristoferi L, Oniscu G, Strazzabosco M, Cillo U. European Society for Organ Transplantation (ESOT) Consensus Statement on Outcome Measures in Liver Transplantation According to Value-Based Health Care. Transplant International 2024, 36: 12190. PMID: 38332850, PMCID: PMC10850237, DOI: 10.3389/ti.2023.12190.Peer-Reviewed Original ResearchConceptsValue-based health careOutcome measuresEuropean Society of Organ TransplantationLiver transplantationDevelopment of outcome measuresConsensus statementOrgan transplantationValue-basedPatient-focusedAdvanced liver diseaseHealth careMultidisciplinary teamLiver diseaseTransplantationLife-savingEuropean Society for Organ TransplantationPatients
2022
Diabetes medications and risk of HCC
Torres M, Jaffe A, Perry R, Marabotto E, Strazzabosco M, Giannini EG. Diabetes medications and risk of HCC. Hepatology 2022, 76: 1880-1897. PMID: 35239194, PMCID: PMC9790535, DOI: 10.1002/hep.32439.Peer-Reviewed Reviews, Practice Guidelines, Standards, and Consensus StatementsConceptsType 2 diabetes mellitusLiver diseaseRisk of HCCDiabetic medicationsDiabetes mellitusGlucagon-like peptide-1 receptor agonistsSodium-glucose cotransporter 2 inhibitorsGlucose cotransporter 2 inhibitorsPeptide-1 receptor agonistsCotransporter 2 inhibitorsAchievement of euglycemiaInsulin-sensitizing activityLiver cancer tumorigenesisDiabetes medicationsDrug armamentariumInsulin resistanceReceptor agonistRisk factorsInsulin secretionCurrent evidenceNew agentsCancer tumorigenesisGlucose absorptionEndocrinology perspectiveHepatic carcinogenesisHolistic management of hepatocellular carcinoma: The hepatologist's comprehensive playbook
Jaffe A, Taddei TH, Giannini EG, Ilagan‐Ying Y, Colombo M, Strazzabosco M. Holistic management of hepatocellular carcinoma: The hepatologist's comprehensive playbook. Liver International 2022, 42: 2607-2619. PMID: 36161463, PMCID: PMC10878125, DOI: 10.1111/liv.15432.Peer-Reviewed Original ResearchConceptsLiver diseaseHepatocellular carcinomaLiver functionRisk factorsRelevant prognostic roleChronic hepatitis BResidual liver functionChronic liver diseaseManagement of patientsCare of patientsCommon risk factorsAppropriate surveillance strategiesSuccessful cancer treatmentC infectionCommon complicationHepatitis BMetabolic syndromePrognostic roleSignificant morbidityLongitudinal careCurrent careCancer recurrenceHCC managementLiver cancerHCC treatmentMetabolic Syndrome and Liver Cancer
Jaffe A, Strazzabosco M. Metabolic Syndrome and Liver Cancer. 2022, 87-104. DOI: 10.1007/978-3-030-41683-6_51.Peer-Reviewed Original ResearchHepatitis B virusNonalcoholic fatty liver diseaseMetabolic syndromeRisk factorsHepatocellular carcinomaLiver diseaseLiver cancerParticular hepatitis B virusIncidence of HCCAdvanced liver diseaseIndependent risk factorFatty liver diseaseType 2 diabetesUnderlying pathogenic mechanismsHepatic manifestationHCC incidenceViral hepatitisAdvanced fibrosisSignificant morbidityDiabetes leadsTime patientsB virusHCC riskHigh prevalenceLower incidenceBile acids and their receptors: modulators and therapeutic targets in liver inflammation
Bertolini A, Fiorotto R, Strazzabosco M. Bile acids and their receptors: modulators and therapeutic targets in liver inflammation. Seminars In Immunopathology 2022, 44: 547-564. PMID: 35415765, PMCID: PMC9256560, DOI: 10.1007/s00281-022-00935-7.Peer-Reviewed Reviews, Practice Guidelines, Standards, and Consensus StatementsConceptsBile acidsLiver diseaseTherapeutic targetAutoimmune liver diseaseCholestatic liver diseaseBile acid receptorAbsorption of lipidsFat-soluble vitaminsLiver inflammationInflammatory diseasesImmunomodulatory propertiesAcid receptorsInflammationDiseaseReceptorsClinical applicationLiverNutrient metabolismPathwayInflammatory pathways and cholangiocarcioma risk mechanisms and prevention
Cadamuro M, Strazzabosco M. Inflammatory pathways and cholangiocarcioma risk mechanisms and prevention. Advances In Cancer Research 2022, 156: 39-73. PMID: 35961707, PMCID: PMC10916841, DOI: 10.1016/bs.acr.2022.02.001.Peer-Reviewed Original ResearchConceptsDevelopment of cholangiocarcinomaNonalcoholic fatty liver diseaseAdequate therapeutic treatmentPrimary sclerosing cholangitisFatty liver diseasePro-inflammatory mechanismsMain risk factorsProdromal diseaseSclerosing cholangitisCaroli's diseaseMetabolic syndromeChronic cholangiopathiesLiver diseasePoor prognosisInflammatory pathwaysBiliary treeCCA developmentRisk factorsImmune responseImmunological responseTherapeutic targetCholangiocarcinomaFluke infestationRole of cellExtrahepatic areas
2021
The Neglected Role of Bile Duct Epithelial Cells in NASH
Cadamuro M, Lasagni A, Sarcognato S, Guido M, Fabris R, Strazzabosco M, Strain AJ, Simioni P, Villa E, Fabris L. The Neglected Role of Bile Duct Epithelial Cells in NASH. Seminars In Liver Disease 2021, 42: 034-047. PMID: 34794182, DOI: 10.1055/s-0041-1739455.Peer-Reviewed Original ResearchConceptsNonalcoholic fatty liver diseaseNonalcoholic steatohepatitisLiver diseaseInsulin resistancePrevalent liver diseaseBile duct epithelial cellsFatty liver diseaseSubset of patientsCommon pathogenetic mechanismDuct epithelial cellsMultiple biological effectsFibro-inflammationHepatic manifestationNAFLD patientsPortal fibrosisMetabolic syndromeBile ductDuctular reactionDisease progressionPathogenetic mechanismsLiver cancerMetabolic alterationsProgenitor cell compartmentEpithelial cellsDiseaseAn international genome-wide meta-analysis of primary biliary cholangitis: Novel risk loci and candidate drugs
Cordell H, Fryett J, Ueno K, Darlay R, Aiba Y, Hitomi Y, Kawashima M, Nishida N, Khor S, Gervais O, Kawai Y, Nagasaki M, Tokunaga K, Tang R, Shi Y, Li Z, Juran B, Atkinson E, Gerussi A, Carbone M, Asselta R, Cheung A, de Andrade M, Baras A, Horowitz J, Ferreira M, Sun D, Jones D, Flack S, Spicer A, Mulcahy V, Byan J, Han Y, Sandford R, Lazaridis K, Amos C, Hirschfield G, Seldin M, Invernizzi P, Siminovitch K, Ma X, Nakamura M, Mells G, Consortia P, Consortium C, Siminovitch K, Mason A, Vincent C, Xie G, Zhang J, Consortium C, Tang R, Ma X, Li Z, Shi Y, Group I, Affronti A, Almasio P, Alvaro D, Andreone P, Andriulli A, Azzaroli F, Battezzati P, Benedetti A, Bragazzi M, Brunetto M, Bruno S, Calvaruso V, Cardinale V, Casella G, Cazzagon N, Ciaccio A, Coco B, Colli A, Colloredo G, Colombo M, Colombo S, Cristoferi L, Cursaro C, Crocè L, Crosignani A, D’Amato D, Donato F, Elia G, Fabris L, Fagiuoli S, Ferrari C, Floreani A, Galli A, Giannini E, Grattagliano I, Lampertico P, Lleo A, Malinverno F, Mancuso C, Marra F, Marzioni M, Massironi S, Mattalia A, Miele L, Milani C, Morini L, Morisco F, Muratori L, Muratori P, Niro G, O’Donnell S, Picciotto A, Portincasa P, Rigamonti C, Ronca V, Rosina F, Spinzi G, Strazzabosco M, Tarocchi M, Tiribelli C, Toniutto P, Valenti L, Vinci M, Zuin M, Consortium J, Nakamura H, Abiru S, Nagaoka S, Komori A, Yatsuhashi H, Ishibashi H, Ito M, Migita K, Ohira H, Katsushima S, Naganuma A, Sugi K, Komatsu T, Mannami T, Matsushita K, Yoshizawa K, Makita F, Nikami T, Nishimura H, Kouno H, Kouno H, Ota H, Komura T, Nakamura Y, Shimada M, Hirashima N, Komeda T, Ario K, Nakamuta M, Yamashita T, Furuta K, Kikuchi M, Naeshiro N, Takahashi H, Mano Y, Tsunematsu S, Yabuuchi I, Shimada Y, Yamauchi K, Sugimoto R, Sakai H, Mita E, Koda M, Tsuruta S, Kamitsukasa H, Sato T, Masaki N, Kobata T, Fukushima N, Ohara Y, Muro T, Takesaki E, Takaki H, Yamamoto T, Kato M, Nagaoki Y, Hayashi S, Ishida J, Watanabe Y, Kobayashi M, Koga M, Saoshiro T, Yagura M, Hirata K, Tanaka A, Takikawa H, Zeniya M, Abe M, Onji M, Kaneko S, Honda M, Arai K, Arinaga-Hino T, Hashimoto E, Taniai M, Umemura T, Joshita S, Nakao K, Ichikawa T, Shibata H, Yamagiwa S, Seike M, Honda K, Sakisaka S, Takeyama Y, Harada M, Senju M, Yokosuka O, Kanda T, Ueno Y, Kikuchi K, Ebinuma H, Himoto T, Yasunami M, Murata K, Mizokami M, Kawata K, Shimoda S, Miyake Y, Takaki A, Yamamoto K, Hirano K, Ichida T, Ido A, Tsubouchi H, Chayama K, Harada K, Nakanuma Y, Maehara Y, Taketomi A, Shirabe K, Soejima Y, Mori A, Yagi S, Uemoto S, Egawa H, Tanaka T, Yamashiki N, Tamura S, Sugawara Y, Kokudo N, Consortium P, Atkinson E, de Andrade M, Chalasani N, Luketic V, Odin J, Chopra K, Baras A, Horowitz J, Abecasis G, Cantor M, Coppola G, Economides A, Lotta L, Overton J, Reid J, Shuldiner A, Beechert C, Forsythe C, Fuller E, Gu Z, Lattari M, Lopez A, Schleicher T, Padilla M, Toledo K, Widom L, Wolf S, Pradhan M, Manoochehri K, Ulloa R, Bai X, Balasubramanian S, Barnard L, Blumenfeld A, Eom G, Habegger L, Hawes A, Khalid S, Maxwell E, Salerno W, Staples J, Jones M, Mitnaul L, Consortium U, Sturgess R, Healey C, Yeoman A, Gunasekera A, Kooner P, Kapur K, Sathyanarayana V, Kallis Y, Subhani J, Harvey R, McCorry R, Rooney P, Ramanaden D, Evans R, Mathialahan T, Gasem J, Shorrock C, Bhalme M, Southern P, Tibble J, Gorard D, Jones S, Mells G, Mulcahy V, Srivastava B, Foxton M, Collins C, Elphick D, Karmo M, Porras-Perez F, Mendall M, Yapp T, Patel M, Ede R, Sayer J, Jupp J, Fisher N, Carter M, Koss K, Shah J, Piotrowicz A, Scott G, Grimley C, Gooding I, Williams S, Tidbury J, Lim G, Cheent K, Levi S, Mansour D, Beckley M, Hollywood C, Wong T, Marley R, Ramage J, Gordon H, Ridpath J, Ngatchu T, Grover V, Shidrawi R, Abouda G, Corless L, Narain M, Rees I, Brown A, Taylor-Robinson S, Wilkins J, Grellier L, Banim P, Das D, Heneghan M, Curtis H, Matthews H, Mohammed F, Aldersley M, Srirajaskanthan R, Walker G, McNair A, Sharif A, Sen S, Bird G, Prince M, Prasad G, Kitchen P, Barnardo A, Oza C, Sivaramakrishnan N, Gupta P, Shah A, Evans C, Saha S, Pollock K, Bramley P, Mukhopadhya A, Barclay S, McDonald N, Bathgate A, Palmer K, Dillon J, Rushbrook S, Przemioslo R, McDonald C, Millar A, Tai C, Mitchell S, Metcalf J, Shaukat S, Ninkovic M, Shmueli U, Davis A, Naqvi A, Lee T, Ryder S, Collier J, Klass H, Cramp M, Sharer N, Aspinall R, Ghosh D, Douds A, Booth J, Williams E, Hussaini H, Christie J, Mann S, Thorburn D, Marshall A, Patanwala I, Ala A, Maltby J, Matthew R, Corbett C, Vyas S, Singhal S, Gleeson D, Misra S, Butterworth J, George K, Harding T, Douglass A, Mitchison H, Panter S, Shearman J, Bray G, Roberts M, Butcher G, Forton D, Mahmood Z, Cowan M, Das D, Ch’ng C, Rahman M, Whatley G, Wesley E, Mandal A, Jain S, Pereira S, Wright M, Trivedi P, Gordon F, Unitt E, Palejwala A, Austin A, Vemala V, Grant A, Higham A, Brind A, Mathew R, Cox M, Ramakrishnan S, King A, Whalley S, Fraser J, Thomson S, Bell A, Wong V, Kia R, Gee I, Keld R, Ransford R, Gotto J, Millson C. An international genome-wide meta-analysis of primary biliary cholangitis: Novel risk loci and candidate drugs. Journal Of Hepatology 2021, 75: 572-581. PMID: 34033851, PMCID: PMC8811537, DOI: 10.1016/j.jhep.2021.04.055.Peer-Reviewed Original ResearchConceptsChronic liver diseaseBiliary cholangitisLiver diseaseSmall intrahepatic bile ductsTreatment of PBCPrimary biliary cholangitisIntrahepatic bile ductsAdditional risk lociRisk lociPattern recognition receptorsLicensed medicationsT helperAutoimmune destructionEast Asian cohortsAutoimmune disordersBile ductAsian cohortEuropean cohortInadequate responseNovel therapiesDrug efficacy screeningHealthy individualsGenome-wide association studiesNumerous risk lociRecognition receptorsRare and undiagnosed liver diseases: challenges and opportunities
Fabris L, Strazzabosco M. Rare and undiagnosed liver diseases: challenges and opportunities. Translational Gastroenterology And Hepatology 2021, 6: 18-18. PMID: 33824922, PMCID: PMC7838528, DOI: 10.21037/tgh-2020-05.Peer-Reviewed Original ResearchUndiagnosed liver diseaseLiver diseaseFibrocystic liver disease: novel concepts and translational perspectives
Lasagni A, Cadamuro M, Morana G, Fabris L, Strazzabosco M. Fibrocystic liver disease: novel concepts and translational perspectives. Translational Gastroenterology And Hepatology 2021, 6: 26-26. DOI: 10.21037/tgh-2019-rld-13.Peer-Reviewed Original ResearchCongenital Cystic Lesions of the Biliary Tree
Lasagni A, Morana G, Strazzabosco M, Fabris L, Cadamuro M. Congenital Cystic Lesions of the Biliary Tree. 2021, 19-46. DOI: 10.1007/978-3-030-65908-0_2.Peer-Reviewed Original ResearchFibropolycystic liver diseasePolycystic liver diseaseFibrocystic liver diseaseHepatorenal fibrocystic diseaseIntrahepatic bile ductsCholedochal cystLiver diseaseBile ductBiliary treeLarge intrahepatic bile ductsSmall intrahepatic bile ductsBile duct dilationRenal function impairmentEarly surgical interventionOnly curative approachCongenital cystic lesionsExtrahepatic bile ductDuctal plate malformationRecessive polycystic kidney diseasePotential therapeutic targetSpectrum of disordersGrowth of cystsPolycystic kidney diseaseBiliary microhamartomasLiver transplantationMetabolic Syndrome and Liver Cancer
Jaffe A, Strazzabosco M. Metabolic Syndrome and Liver Cancer. 2021, 1-19. DOI: 10.1007/978-3-030-37482-2_51-1.Peer-Reviewed Original ResearchHepatitis B virusNonalcoholic fatty liver diseaseMetabolic syndromeRisk factorsHepatocellular carcinomaLiver diseaseLiver cancerParticular hepatitis B virusIncidence of HCCAdvanced liver diseaseIndependent risk factorFatty liver diseaseType 2 diabetesUnderlying pathogenic mechanismsHepatic manifestationHCC incidenceViral hepatitisAdvanced fibrosisSignificant morbidityDiabetes leadsTime patientsB virusHCC riskHigh prevalenceLower incidence
2020
Health related quality of life in chronic liver diseases
Cortesi PA, Conti S, Scalone L, Jaffe A, Ciaccio A, Okolicsanyi S, Rota M, Fabris L, Colledan M, Fagiuoli S, Belli LS, Cesana G, Strazzabosco M, Mantovani LG. Health related quality of life in chronic liver diseases. Liver International 2020, 40: 2630-2642. PMID: 32851764, DOI: 10.1111/liv.14647.Peer-Reviewed Original ResearchConceptsChronic liver diseaseLiver diseaseGeneral populationImpact of CLDLower EQ-5D VAS scoresMajor chronic liver diseaseEQ-5D VAS scoreChronic hepatitis stageHRQoL of patientsNAFLD/NASHUtility index scoresHealth-related qualityGeneral Italian populationDifferent QOL domainsAnxiety/depressionQuality of lifeVAS scoresHRQoL dataHepatitis stagePossible confoundersEQ-5DHigh riskItalian patientsHRQoLIndex scoreSurveillance for Hepatocellular Carcinoma in Patients with Non-Alcoholic Fatty Liver Disease: Universal or Selective?
Torres M, Bodini G, Furnari M, Marabotto E, Zentilin P, Strazzabosco M, Giannini EG. Surveillance for Hepatocellular Carcinoma in Patients with Non-Alcoholic Fatty Liver Disease: Universal or Selective? Cancers 2020, 12: 1422. PMID: 32486355, PMCID: PMC7352281, DOI: 10.3390/cancers12061422.Peer-Reviewed Original ResearchLiver diseaseHepatocellular carcinomaRisk factorsHCC surveillanceNAFLD patientsNon-alcoholic fatty liver diseasePrevalence of HCCFrequent primary liver cancerNon-alcoholic fatty liverNon-cirrhotic NAFLDNon-cirrhotic subjectsCause of cirrhosisTreatment of HBVChronic liver diseaseFatty liver diseasePrimary liver cancerCancer-related deathMain risk factorsGood clinical judgmentEarly HCC detectionDeaths/yearNew screening toolNAFLD epidemicHCV infectionLiver ultrasonographyThe Emerging Role of Macrophages in Chronic Cholangiopathies Featuring Biliary Fibrosis: An Attractive Therapeutic Target for Orphan Diseases
Cadamuro M, Girardi N, Gores GJ, Strazzabosco M, Fabris L. The Emerging Role of Macrophages in Chronic Cholangiopathies Featuring Biliary Fibrosis: An Attractive Therapeutic Target for Orphan Diseases. Frontiers In Medicine 2020, 7: 115. PMID: 32373615, PMCID: PMC7186419, DOI: 10.3389/fmed.2020.00115.Peer-Reviewed Original ResearchBiliary fibrogenesisBiliary fibrosisChronic liver diseaseCongenital hepatic fibrosisEffective therapeutic approachHepatic stellate cellsPotential novel targetAttractive therapeutic targetMost cholangiopathiesChronic cholangiopathiesLiver diseasePortal fibroblastsHepatic fibrosisModern hepatologyLiver fibrosisBiliary epitheliumDisease progressionCell effectorsTherapeutic approachesCholangiopathyStellate cellsTherapeutic targetFibrosisOrphan diseaseNovel target
2019
Clinical outcome indicators in chronic hepatitis B and C: A primer for value‐based medicine in hepatology
Strazzabosco M, Cortesi P, Conti S, Okolicsanyi S, Rota M, Ciaccio A, Cozzolino P, Fornari C, Gemma M, Scalone L, Cesana G, Fabris L, Colledan M, Fagiuoli S, Ideo G, Zavaglia C, Perricone G, Munari L, Mantovani L, Belli L. Clinical outcome indicators in chronic hepatitis B and C: A primer for value‐based medicine in hepatology. Liver International 2019, 40: 60-73. PMID: 31654608, PMCID: PMC10916792, DOI: 10.1111/liv.14285.Peer-Reviewed Original ResearchMeSH KeywordsAgedAntiviral AgentsCarcinoma, HepatocellularFemaleGastroenterologyHepatitis B, ChronicHepatitis C, ChronicHumansItalyLiver CirrhosisLiver NeoplasmsLongitudinal StudiesMaleMiddle AgedProspective StudiesQuality Indicators, Health CareSurvival AnalysisTreatment OutcomeValue-Based Health InsuranceConceptsClinical outcome indicatorsClinical practiceOutcome indicatorsChronic hepatitis BClinical practice settingMajor health problemCurrent international guidelinesNew effective treatmentsValue of careMETHODS/RESULTSHCV patientsHCV infectionHepatitis BClinical outcomesLiver diseaseMulticentre studyEffective treatmentHealth problemsInternational guidelinesCare deliveryValue-based medicineCostly treatmentPractice settingsHBVPatientsPathobiology of inherited biliary diseases: a roadmap to understand acquired liver diseases
Fabris L, Fiorotto R, Spirli C, Cadamuro M, Mariotti V, Perugorria MJ, Banales JM, Strazzabosco M. Pathobiology of inherited biliary diseases: a roadmap to understand acquired liver diseases. Nature Reviews Gastroenterology & Hepatology 2019, 16: 497-511. PMID: 31165788, PMCID: PMC6661007, DOI: 10.1038/s41575-019-0156-4.Peer-Reviewed Reviews, Practice Guidelines, Standards, and Consensus StatementsConceptsCystic fibrosis-related liver diseaseFibropolycystic liver diseaseLiver diseasePolycystic liver diseaseBiliary repairAlagille syndromeEpithelial toll-like receptor 4Toll-like receptor 4Acquired liver diseasesGut-derived productsPrimary sclerosing cholangitisDuct epithelial cellsSclerosing cholangitisΒ-catenin signalingPortal fibrosisBiliary diseaseIL-1βUnknown etiologyDependent cytokinesReceptor 4Peribiliary inflammationRole of NotchCholangiopathyNovel treatmentsCyst growthPathophysiology 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