2024
Pathophysiology of Cystic Fibrosis Liver Disease
Kasper V, Assis D. Pathophysiology of Cystic Fibrosis Liver Disease. Pediatric Pulmonology 2024, 59: s98-s106. PMID: 39105342, DOI: 10.1002/ppul.26869.Peer-Reviewed Original ResearchConceptsComplication of cystic fibrosisCF liver diseaseHeterogeneity of clinical manifestationsManifestations of CFDisease-modifying factorsCFTR mutationsHepatobiliary complicationsBiliary stricturesBiliary cholestasisClinical manifestationsCystic fibrosisLiver diseaseTherapeutic approachesCF careDisease modelsDiseasePPAR-Mediated Bile Acid Glucuronidation: Therapeutic Targets for the Treatment of Cholestatic Liver Diseases
Gallucci G, Hayes C, Boyer J, Barbier O, Assis D, Ghonem N. PPAR-Mediated Bile Acid Glucuronidation: Therapeutic Targets for the Treatment of Cholestatic Liver Diseases. Cells 2024, 13: 1296. PMID: 39120326, PMCID: PMC11312002, DOI: 10.3390/cells13151296.Peer-Reviewed Original ResearchPrimary biliary cholangitisPrimary sclerosing cholangitisPeroxisome proliferator-activated receptorCholestatic liver diseaseUrsodeoxycholic acidUridine 5'-diphospho-glucuronosyltransferaseObeticholic acidBile acid metabolismAdjunctive therapyIncomplete response to UDCAProgression of primary biliary cholangitisResponse to UDCALiver diseasePeroxisome proliferator-activated receptor agonistsSecond-line treatmentMarkers of cholestasisImpairment of bile flowTherapeutic targetTreatment of cholestatic liver diseasesRetention of bile acidsAlternative treatment strategiesProgression to fibrosisProliferator-activated receptorsAcid metabolismBiliary cholangitisProtocol for the development of a core outcome set for clinical trials in primary sclerosing cholangitis
Hussain N, Ma C, Hirschfield G, Walmsley M, Hanford P, Vesterhus M, Kowdley K, Bergquist A, Ponsioen C, Levy C, Assis D, Schramm C, Bowlus C, Trauner M, Aiyegbusi O, Jairath V, Trivedi P. Protocol for the development of a core outcome set for clinical trials in primary sclerosing cholangitis. BMJ Open 2024, 14: e080143. PMID: 38926149, PMCID: PMC11216047, DOI: 10.1136/bmjopen-2023-080143.Peer-Reviewed Original ResearchConceptsPrimary sclerosing cholangitisClinical trialsSclerosing cholangitisCore outcome setImmune-mediated liver diseasesPrimary sclerosing cholangitis treatmentOutcome measuresEvaluate novel therapiesSlow disease progressionPatient-reported outcome measuresMedical therapyNovel therapiesLiver fibrosisDisease progressionHistological assessmentLiver diseaseInternational two-round Delphi surveyImaging-based biomarkersIntervention trialsTherapyConsensus meetingHealthcare payersCholangitisSemistructured qualitative interviewsTwo-round Delphi survey
2023
Cystic fibrosis screening, evaluation, and management of hepatobiliary disease consensus recommendations
Sellers Z, Assis D, Paranjape S, Sathe M, Bodewes F, Bowen M, Cipolli M, Debray D, Green N, Hughan K, Hunt W, Leey J, Ling S, Morelli G, Peckham D, Pettit R, Philbrick A, Stoll J, Vavrina K, Allen S, Goodwin T, Hempstead S, Narkewicz M. Cystic fibrosis screening, evaluation, and management of hepatobiliary disease consensus recommendations. Hepatology 2023, 79: 1220-1238. PMID: 37934656, PMCID: PMC11020118, DOI: 10.1097/hep.0000000000000646.Peer-Reviewed Original ResearchConceptsAdvanced liver diseaseCystic fibrosisLiver diseaseHepatobiliary involvementHepatobiliary complicationsCF-associated liver diseasePubMed literature searchCystic fibrosis screeningScreening ultrasoundPortal hypertensionOutcome questionsLiver biopsyLiver failureMultilobular cirrhosisFibrosis screeningConsensus recommendationsFull-text reviewHepatobiliary diseaseCF FoundationLiterature searchThirty-oneCF outcomesMultidisciplinary committeeDiseaseComplicationsAdvancing diagnosis and management of liver disease in adults through exome sequencing
Zheng M, Hakim A, Konkwo C, Deaton A, Ward L, Genetics A, Silveira M, Assis D, Liapakis A, Jaffe A, Jiang Z, Curry M, Lai M, Cho M, Dykas D, Bale A, Mistry P, Vilarinho S. Advancing diagnosis and management of liver disease in adults through exome sequencing. EBioMedicine 2023, 95: 104747. PMID: 37566928, PMCID: PMC10433007, DOI: 10.1016/j.ebiom.2023.104747.Peer-Reviewed Original ResearchConceptsLiver diseaseWhole-exome sequencingUnknown etiologyTertiary referral academic medical centerReferral academic medical centerExome sequencingLiver disease patientsManagement of adultsAcademic health care centerComprehensive clinical evaluationHealth care centersAcademic medical centerGenetic variantsRare genetic variantsAdult patientsLiver centersHepatic steatosisDisease patientsClinical evaluationCare centerFamily historyMedical CenterClinical valueAdult medicinePatientsWED-283 Investigating the cholestatic pruritus of primary sclerosing cholangitis (ItCh-PSC): a study of patients participating in the consortium for autoimmune liver disease (CALiD)
Dean R, Yazdanfar M, Zepeda J, Levy C, Gordon S, Forman L, Lammert C, Assis D, Pratt D, Gungabissoon U, McGirr A, Mukherjee S, McLaughlin M, Bowlus C. WED-283 Investigating the cholestatic pruritus of primary sclerosing cholangitis (ItCh-PSC): a study of patients participating in the consortium for autoimmune liver disease (CALiD). Journal Of Hepatology 2023, 78: s388. DOI: 10.1016/s0168-8278(23)01040-1.Peer-Reviewed Original ResearchRecent Advances in the Management of Primary Sclerosing Cholangitis
Assis D, Bowlus C. Recent Advances in the Management of Primary Sclerosing Cholangitis. Clinical Gastroenterology And Hepatology 2023, 21: 2065-2075. PMID: 37084929, DOI: 10.1016/j.cgh.2023.04.004.Peer-Reviewed Original ResearchConceptsChronic cholestatic liver diseasePrimary sclerosing cholangitisInflammatory bowel diseasePrognostication of patientsCholestatic liver diseaseSclerosing cholangitisBowel diseaseLiver failureClinical featuresLiver diseaseMedical managementBiliary treeComplex pathophysiologyEffective therapyPharmacologic agentsRare natureCurrent conceptsDiseaseCholangitisFurther studiesCholangiocarcinomaPatientsPathophysiologyTherapyPrognosticationResearch gaps and opportunities in autoimmune hepatitis—Results of the international autoimmune hepatitis group research workshop 2022
Snijders R, Assis D, Oo Y, Sebode M, Taubert R, Willemse J, Tomsin B, Lohse A, Drenth J, Gevers T, Diseases T. Research gaps and opportunities in autoimmune hepatitis—Results of the international autoimmune hepatitis group research workshop 2022. Liver International 2023, 43: 1375-1384. PMID: 37035872, DOI: 10.1111/liv.15573.Peer-Reviewed Original ResearchConceptsInternational Autoimmune Hepatitis GroupAutoimmune hepatitisRare autoimmune liver diseaseMajor unmet clinical needClinical needAdult autoimmune hepatitisAutoimmune Hepatitis GroupAutoimmune liver diseaseLiver-related complicationsHealth-related qualityNew immunomodulatory therapiesInflammatory immune reactionsUnmet clinical needClinical trial designSpecific diagnostic testsQuality of lifeHepatitis groupNonselective immunosuppressionImmunomodulatory therapyLiver diseasePrognostic markerImmune reactionsPatient representativesTrial designCare delivery
2022
Fenofibrate Downregulates NF-κB Signaling to Inhibit Pro-inflammatory Cytokine Secretion in Human THP-1 Macrophages and During Primary Biliary Cholangitis
Gallucci GM, Alsuwayt B, Auclair AM, Boyer JL, Assis DN, Ghonem NS. Fenofibrate Downregulates NF-κB Signaling to Inhibit Pro-inflammatory Cytokine Secretion in Human THP-1 Macrophages and During Primary Biliary Cholangitis. Inflammation 2022, 45: 2570-2581. PMID: 35838934, PMCID: PMC10853883, DOI: 10.1007/s10753-022-01713-1.Peer-Reviewed Original ResearchConceptsPrimary biliary cholangitisPrimary sclerosing cholangitisAnti-inflammatory mechanismsChronic liver diseaseNF-κB signalingBiliary cholangitisLiver diseaseNF-κB p50IL-1βIL-8Peroxisome proliferator-activated receptor alphaPro-inflammatory cytokine secretionProliferator-activated receptor alphaIncomplete biochemical responseAnti-inflammatory effectsAddition of fenofibratePro-inflammatory cytokinesPPARα-dependent mannerHuman THP-1 macrophagesP65 protein expressionLabel therapeutic optionTHP-1 macrophagesTHP-1 cellsSclerosing cholangitisAdult patients
2021
Outcome of COVID‐19 in Patients With Autoimmune Hepatitis: An International Multicenter Study
Efe C, Dhanasekaran R, Lammert C, Ebik B, la Tijera F, Aloman C, Calışkan A, Peralta M, Gerussi A, Massoumi H, Catana AM, Torgutalp M, Purnak T, Rigamonti C, Aldana A, Khakoo N, Kacmaz H, Nazal L, Frager S, Demir N, Irak K, Ellik ZM, Balaban Y, Atay K, Eren F, Cristoferi L, Batıbay E, Urzua Á, Snijders R, Kıyıcı M, Akyıldız M, Ekin N, Carr RM, Harputluoğlu M, Hatemi I, Mendizabal M, Silva M, Idilman R, Silveira M, Drenth JPH, Assis DN, Björnsson E, Boyer JL, Invernizzi P, Levy C, Schiano TD, Ridruejo E, Wahlin S. Outcome of COVID‐19 in Patients With Autoimmune Hepatitis: An International Multicenter Study. Hepatology 2021, 73: 2099-2109. PMID: 33713486, PMCID: PMC8250536, DOI: 10.1002/hep.31797.Peer-Reviewed Original ResearchConceptsSevere COVID-19Chronic liver diseaseAutoimmune hepatitisLiver injuryMulticenter studyCOVID-19Causes of CLDPropensity score-matched cohortSevere COVID-19 outcomesContinuation of immunosuppressionMaintenance of immunosuppressionOutcomes of patientsIntensive care admissionUse of antiviralsInternational multicenter studyCOVID-19 outcomesCOVID-19 diagnosisContinued immunosuppressionCare admissionCause mortalityIndependent predictorsMedian ageLiver diseaseMechanical ventilationRetrospective study
2020
Diagnosis and Management of Autoimmune Hepatitis in Adults and Children: 2019 Practice Guidance and Guidelines From the American Association for the Study of Liver Diseases
Mack CL, Adams D, Assis DN, Kerkar N, Manns MP, Mayo MJ, Vierling JM, Alsawas M, Murad MH, Czaja AJ. Diagnosis and Management of Autoimmune Hepatitis in Adults and Children: 2019 Practice Guidance and Guidelines From the American Association for the Study of Liver Diseases. Hepatology 2020, 72: 671-722. PMID: 31863477, DOI: 10.1002/hep.31065.Peer-Reviewed Original ResearchConceptsAutoimmune hepatitisLiver diseaseAmerican AssociationPractice guidanceHepatitisDiseaseDiagnosisFenofibrate Improves Liver Function and Reduces the Toxicity of the Bile Acid Pool in Patients With Primary Biliary Cholangitis and Primary Sclerosing Cholangitis Who Are Partial Responders to Ursodiol
Ghonem NS, Auclair AM, Hemme CL, Gallucci GM, de la Rosa Rodriguez R, Boyer JL, Assis DN. Fenofibrate Improves Liver Function and Reduces the Toxicity of the Bile Acid Pool in Patients With Primary Biliary Cholangitis and Primary Sclerosing Cholangitis Who Are Partial Responders to Ursodiol. Clinical Pharmacology & Therapeutics 2020, 108: 1213-1223. PMID: 32480421, PMCID: PMC7886378, DOI: 10.1002/cpt.1930.Peer-Reviewed Original ResearchMeSH KeywordsAdultAgedBile Acids and SaltsBiomarkersCholangitis, SclerosingCytokinesDrug Therapy, CombinationFemaleFenofibrateHumansInflammation MediatorsLiverLiver Cirrhosis, BiliaryLiver Function TestsMaleMiddle AgedPPAR alphaPrincipal Component AnalysisRetrospective StudiesTreatment OutcomeUrsodeoxycholic AcidYoung AdultConceptsPrimary sclerosing cholangitisPrimary biliary cholangitisBile acid metabolismSclerosing cholangitisBiliary cholangitisBile acidsAcid metabolismPeroxisome proliferator-activated receptor alphaProliferator-activated receptor alphaRetrospective observational studyBeneficial clinical effectsCholestatic liver diseasePro-inflammatory cytokinesBile acid metabolitesHealthy control subjectsBile acid poolSerum alkaline phosphataseAminotransferase abnormalitiesUrsodiol therapyFenofibrate therapyPartial respondersBile acid precursorsClinical effectsFenofibrate treatmentLiver disease
2019
The Contribution of B Cells in Autoimmune Liver Diseases
Taylor SA, Assis DN, Mack CL. The Contribution of B Cells in Autoimmune Liver Diseases. Seminars In Liver Disease 2019, 39: 422-431. PMID: 31226726, PMCID: PMC6800599, DOI: 10.1055/s-0039-1688751.BooksConceptsAutoimmune liver diseaseT cell activationB cellsLiver diseaseT cellsCell activationB-cell mechanismsRegulatory T cellsProduction of autoantibodiesAutoreactive T cellsAutoreactive B cellsGeneration of cytokinesAutoimmune hepatitisCell mechanismsAntigen presentationDisease pathogenesisAutoantibodiesDiseaseCellsActivationVast majorityHepatitisCholangiopathyAutoimmunityCytokinesClinical utility of genomic analysis in adults with idiopathic liver disease
Hakim A, Zhang X, DeLisle A, Oral EA, Dykas D, Drzewiecki K, Assis DN, Silveira M, Batisti J, Jain D, Bale A, Mistry PK, Vilarinho S. Clinical utility of genomic analysis in adults with idiopathic liver disease. Journal Of Hepatology 2019, 70: 1214-1221. PMID: 31000363, PMCID: PMC6526061, DOI: 10.1016/j.jhep.2019.01.036.Peer-Reviewed Original ResearchConceptsIdiopathic liver diseaseUnexplained liver diseaseManagement of adultsWhole-exome sequencingLiver diseaseAdult patientsUnknown etiologyHeterozygous variantsUse of WESAmelioration of dyslipidemiaDaily insulin requirementLeptin replacement therapyUtility of WESChronic liver diseaseNon-alcoholic steatohepatitisAcademic health care centerHealth care centersHomozygous pathogenic variantUnrelated adult patientsNon-oncological diseasesDisease preventive measuresInsulin requirementsLean patientsDevastating complicationLiver aminotransferases
2017
FRI-393 Macrophage migration inhibitory factor (MIF) modulates T-cell proliferation and hepatic inflammation in a model of autoimmune liver disease
Roberts S, Leng L, Soroka C, Boyer J, Bucala R, Assis D. FRI-393 Macrophage migration inhibitory factor (MIF) modulates T-cell proliferation and hepatic inflammation in a model of autoimmune liver disease. Journal Of Hepatology 2017, 66: s363-s364. DOI: 10.1016/s0168-8278(17)31067-x.Peer-Reviewed Original Research
2015
Fibrates and cholestasis
Ghonem NS, Assis DN, Boyer JL. Fibrates and cholestasis. Hepatology 2015, 62: 635-643. PMID: 25678132, PMCID: PMC4515188, DOI: 10.1002/hep.27744.BooksConceptsPrimary sclerosing cholangitisPrimary biliary cirrhosisCholestatic liver diseaseUDCA therapyLiver diseaseUrsodeoxycholic acidNuclear receptorsProgression of PBCHepatic transportersPeroxisome proliferator-activated receptor alphaProliferator-activated receptor alphaToxic bile constituentsApical sodium-dependent bile salt transporterOnly therapeutic optionCholestatic liver disordersBile acid homeostasisBile acid synthesisBile salt transportersMultidrug resistance protein 3Cytochrome P450PPARα effectLiver transplantationSclerosing cholangitisBiliary cirrhosisLiver failure
2013
The role of macrophage migration inhibitory factor in autoimmune liver disease
Assis DN, Leng L, Du X, Zhang CK, Grieb G, Merk M, Garcia AB, McCrann C, Chapiro J, Meinhardt A, Mizue Y, Nikolic‐Paterson D, Bernhagen J, Kaplan MM, Zhao H, Boyer JL, Bucala R. The role of macrophage migration inhibitory factor in autoimmune liver disease. Hepatology 2013, 59: 580-591. PMID: 23913513, PMCID: PMC3877200, DOI: 10.1002/hep.26664.Peer-Reviewed Original ResearchMeSH KeywordsAdultAgedAged, 80 and overAntigens, Differentiation, B-LymphocyteBiomarkersBiopsyCase-Control StudiesCohort StudiesFemaleGene FrequencyHepatitis, AutoimmuneHistocompatibility Antigens Class IIHumansIntramolecular OxidoreductasesLiverLiver Cirrhosis, BiliaryMacrophage Migration-Inhibitory FactorsMaleMicrosatellite RepeatsMiddle AgedPhenotypePolymorphism, Single NucleotideConceptsMacrophage migration inhibitory factorPrimary biliary cirrhosisAutoimmune hepatitisMigration inhibitory factorMIF receptorHealthy controlsInhibitory factorAutoimmune liver diseaseMIF promoter polymorphismsHepatic stellate cellsEnzyme-linked immunosorbentCATT7 alleleImmunopathogenic basisMIF expressionMIF locusBiliary cirrhosisLiver diseaseInflammatory phenotypeReceptor profileStellate cellsPromoter polymorphismPatientsSerum samplesCD74Single nucleotide polymorphisms
2010
Testing and management of thrombocytopenia and coagulopathy in the pre- and postliver transplant patient.
Assis DN, Schilsky ML. Testing and management of thrombocytopenia and coagulopathy in the pre- and postliver transplant patient. Minerva Gastroenterology 2010, 56: 331-43. PMID: 21037549.Peer-Reviewed Original ResearchConceptsPost-transplant periodLiver diseaseAdvanced liver diseaseManagement of coagulopathyAcute liver failureManagement of thrombocytopeniaImportant clinical skillSuperimposed complicationsHepatic dysfunctionTransplant patientsLiver failureCoagulation changesTransplant periodPathophysiologic changesBlood productsCoagulation testingCoagulopathyThrombocytopeniaPatientsClinical skillsDiseasePreSepsisComplicationsDysfunction