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
2019
Fibrocystic Liver Disease
Cristoferi L, Morana G, Strazzabosco M, Fabris L. Fibrocystic Liver Disease. 2019, 201-218. DOI: 10.1007/978-3-319-96400-3_11.Peer-Reviewed Original ResearchHepatorenal fibrocystic diseaseCongenital hepatic fibrosisFibrocystic liver diseaseLiver diseaseCaroli's diseaseCholedochal cystEnd-stage liver diseaseBiliary duct dilationEmbryonic ductal plateAutosomal recessive polycystic kidney diseaseIntrahepatic bile ductsRecessive polycystic kidney diseasePolycystic kidney diseaseLiver transplantationDuct dilationHepatic involvementMultidisciplinary managementRenal diseaseMultiorgan involvementClinical manifestationsBile ductFibrocystic diseaseKidney diseaseBiliary treeHepatic fibrosis
2018
Fibropolycystic Liver Diseases and Congenital Biliary Abnormalities
Hadžić N, Strazzabosco M. Fibropolycystic Liver Diseases and Congenital Biliary Abnormalities. 2018, 308-327. DOI: 10.1002/9781119237662.ch16.Peer-Reviewed Original ResearchFibropolycystic liver diseaseLiver diseasePolycystic kidney diseaseKidney diseaseAutosomal recessive polycystic kidney diseaseAutosomal dominant polycystic kidney diseaseFibrotic liver diseasePolycystic liver diseaseRecessive polycystic kidney diseaseDominant polycystic kidney diseaseCongenital biliary abnormalitiesBiliary microhamartomasPathogenetic pointCholedochal cystBiliary tractBiliary abnormalitiesBile ductCystic lesionsRenal abnormalitiesClinical reasonsCongenital anomaliesLiver parenchymaDiseaseHeterogeneous groupAbnormalities
2013
Protein kinase a‐dependent pSer675‐β‐catenin, a novel signaling defect in a mouse model of congenital hepatic fibrosis
Spirli C, Locatelli L, Morell CM, Fiorotto R, Morton SD, Cadamuro M, Fabris L, Strazzabosco M. Protein kinase a‐dependent pSer675‐β‐catenin, a novel signaling defect in a mouse model of congenital hepatic fibrosis. Hepatology 2013, 58: 1713-1723. PMID: 23744610, PMCID: PMC3800498, DOI: 10.1002/hep.26554.Peer-Reviewed Original ResearchConceptsAutosomal recessive polycystic kidney diseaseCongenital hepatic fibrosisCaroli's diseaseΒ-cateninHepatic fibrosisRac-1 inhibitionIntrahepatic bile ductsRecessive polycystic kidney diseasePotential therapeutic targetPolycystic kidney diseaseStimulation of cAMPRac-1 activityE-cadherin expressionBile ductKidney diseaseLiver pathologyCystic dysplasiaMouse modelTherapeutic targetTranscriptional activityNuclear translocationDiseasePKA blockerCholangiocytesFibrosis