2021
Fibrocystic 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. PMID: 33824930, PMCID: PMC7838530, DOI: 10.21037/tgh-2020-04.Peer-Reviewed Original ResearchFibrocystic liver diseaseAutosomal recessive polycystic kidney diseaseIntrahepatic bile ductsBile ductPeribiliary fibrosisLarge intrahepatic bile ductsSmall intrahepatic bile ductsBile duct dilationEmbryonic ductal plateTargeted medical therapyRenal function impairmentMain clinical manifestationsRecessive polycystic kidney diseaseSpectrum of disordersHepatic disease 1Polycystic kidney diseaseBiliary microhamartomasLiver transplantationPortal hypertensionRecurrent cholangitisBiliary changesBiliary cystsDuct dilationMedical therapySurgical treatment
2016
Macrophage recruitment by fibrocystin‐defective biliary epithelial cells promotes portal fibrosis in congenital hepatic fibrosis
Locatelli L, Cadamuro M, Spirlì C, Fiorotto R, Lecchi S, Morell C, Popov Y, Scirpo R, De Matteis M, Amenduni M, Pietrobattista A, Torre G, Schuppan D, Fabris L, Strazzabosco M. Macrophage recruitment by fibrocystin‐defective biliary epithelial cells promotes portal fibrosis in congenital hepatic fibrosis. Hepatology 2016, 63: 965-982. PMID: 26645994, PMCID: PMC4764460, DOI: 10.1002/hep.28382.Peer-Reviewed Original ResearchMeSH KeywordsAnimalsAntigens, NeoplasmChemokinesClodronic AcidCollagenDisease Models, AnimalEpithelial CellsGenetic Diseases, InbornIntegrinsLiver CirrhosisMacrophagesMiceMyofibroblastsReceptors, Cell SurfaceSnail Family Transcription FactorsTranscription FactorsTransforming Growth Factor beta1Tumor Necrosis Factor-alphaConceptsCongenital hepatic fibrosisMacrophage recruitmentPortal hypertensionPortal fibrosisHepatic fibrosisLiver fibrosisCell dysfunctionBile duct changesRange of chemokinesLow-grade inflammationProgressive liver fibrosisDuctal plate malformationEpithelial cell dysfunctionGrowth factor-β1Biliary epithelial cellsBiliary fibrosisLiver failureMacrophage infiltratesLiver cystsDuct changesProinflammatory cytokinesPeribiliary fibrosisBiliary epitheliumDisease progressionM1 phenotype