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 modelsDiseaseProtocol 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 surveyAfrican American race does not confer an increased risk of clinical events in patients with primary sclerosing cholangitis.
Yazdanfar M, Zepeda J, Dean R, Wu J, Levy C, Goldberg D, Lammert C, Prenner S, Reddy K, Pratt D, Forman L, Assis D, Lytvyak E, Montano-Loza A, Gordon S, Carey E, Ahn J, Schlansky B, Korzenik J, Karagozian R, Hameed B, Chandna S, Yu L, Bowlus C. African American race does not confer an increased risk of clinical events in patients with primary sclerosing cholangitis. Hepatology Communications 2024, 8 PMID: 38285883, PMCID: PMC10830082, DOI: 10.1097/hc9.0000000000000366.Peer-Reviewed Original ResearchConceptsPrimary sclerosing cholangitisTransplant-free survivalInflammatory bowel diseaseHepatic decompensationNon-Hispanic whitesSclerosing cholangitisIncreased risk of clinical eventsNatural history of primary sclerosing cholangitisAssociated with transplant-free survivalHistory of primary sclerosing cholangitisAssociated with hepatic decompensationBowel diseaseProgression to hepatic decompensationRisk of clinical eventsDecompensation-free survivalMayo risk scoreAbnormal liver testsPerformance of prognostic modelsAfrican American raceRates of inflammatory bowel diseaseDeath/liver transplantationAA patientsLiver testsDiagnostic delayAA race
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 medicinePatientsRecent 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 deliveryReply: Insurance should cover vancomycin for primary sclerosing cholangitis
Bowlus C, Arrivé L, Bergquist A, Deneau M, Forman L, Ilyas S, Lunsford K, Martinez M, Sapisochin G, Shroff R, Tabibian J, Assis D. Reply: Insurance should cover vancomycin for primary sclerosing cholangitis. Hepatology 2023, 77: e176-e177. PMID: 36695291, DOI: 10.1097/hep.0000000000000305.Peer-Reviewed Original ResearchOutcomes of immunomodulator and advanced therapies for primary sclerosing cholangitis-associated inflammatory bowel disease
Sayed A, Assis D, Silveira M, Deng Y, Ciarleglio M, Gaidos J, Proctor D, Al-Bawardy B. Outcomes of immunomodulator and advanced therapies for primary sclerosing cholangitis-associated inflammatory bowel disease. European Journal Of Gastroenterology & Hepatology 2023, 35: 270-274. PMID: 36708297, DOI: 10.1097/meg.0000000000002510.Peer-Reviewed Original ResearchConceptsPrimary sclerosing cholangitisInflammatory bowel diseasePSC-IBDEndoscopic healingTherapy groupAdvanced therapiesClinical remissionBowel diseaseAcute ascending cholangitisRate of cholangitisSmall bowel involvementThird of patientsAscending cholangitisSclerosing cholangitisAdult patientsBowel involvementSecondary outcomesMedian agePrimary outcomeRetrospective studyHigh riskCholangitisPatientsTherapyLarger studyReply: Living donor liver transplantation for people with PSC
Forman L, Sapisochin G, Assis D, Arrivé L, Bergquist A, Bowlus C, Deneau M, Ilyas S, Lunsford K, Martinez M, Shroff R, Tabibian J. Reply: Living donor liver transplantation for people with PSC. Hepatology 2023, 77: e97-e98. PMID: 36732294, DOI: 10.1097/hep.0000000000000210.Peer-Reviewed Original Research
2022
Update in Advancing the Gastrointestinal Frontier in Cystic Fibrosis
Vélez C, Freedman S, Assis D. Update in Advancing the Gastrointestinal Frontier in Cystic Fibrosis. Clinics In Chest Medicine 2022, 43: 743-755. PMID: 36344078, DOI: 10.1016/j.ccm.2022.07.001.Peer-Reviewed Original ResearchNoninvasive biomarkers for the diagnosis and management of autoimmune hepatitis
Harrington C, Krishnan S, Mack CL, Cravedi P, Assis DN, Levitsky J. Noninvasive biomarkers for the diagnosis and management of autoimmune hepatitis. Hepatology 2022, 76: 1862-1879. PMID: 35611859, PMCID: PMC9796683, DOI: 10.1002/hep.32591.Peer-Reviewed Original ResearchConceptsAutoimmune hepatitisNormal serum transaminasesPediatric autoimmune hepatitisFirst-line treatmentAcute liver failureImmune cell phenotypesPredictors of responseHigh clinical valueNovel candidate biomarkersPersonalized therapeutic managementSystemic corticosteroidsCurrent regimensMedication titrationHistological activityMost patientsClinical courseLiver failureLiver injurySerum transaminasesMedication dosingTherapeutic managementUnclear etiologyHepatic fibrosisClinical valueElevated riskAASLD practice guidance on primary sclerosing cholangitis and cholangiocarcinoma
Bowlus CL, Arrivé L, Bergquist A, Deneau M, Forman L, Ilyas SI, Lunsford KE, Martinez M, Sapisochin G, Shroff R, Tabibian JH, Assis DN. AASLD practice guidance on primary sclerosing cholangitis and cholangiocarcinoma. Hepatology 2022, 77: 659-702. PMID: 36083140, DOI: 10.1002/hep.32771.Peer-Reviewed Original ResearchFenofibrate 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 studyRole of Biliary Organoids in Cholestasis Research and Regenerative Medicine
Soroka CJ, Roberts SJ, Boyer JL, Assis DN. Role of Biliary Organoids in Cholestasis Research and Regenerative Medicine. Seminars In Liver Disease 2021, 41: 206-212. PMID: 33957696, DOI: 10.1055/s-0041-1728663.Peer-Reviewed Original ResearchConceptsHuman cholestatic diseasesCholestatic diseaseBiliary organoidsStudy of pathophysiologyCholestasis ResearchBiliary treeDisease stageIndividual patientsTranslational studiesPrimary cholangiocytesPersonalized approachBiliary tissueApplication of organoidsStandardization of terminologyTranslational medicineDiseaseOrganoidsMedicineOrganoid technologyPatientsPathophysiologyOral Vancomycin or Ursodeoxycholic Acid for Pediatric Primary Sclerosing Cholangitis? The Uncontroversial Need for Randomized Controlled Trials
Assis DN, Levy C. Oral Vancomycin or Ursodeoxycholic Acid for Pediatric Primary Sclerosing Cholangitis? The Uncontroversial Need for Randomized Controlled Trials. Hepatology 2021, 73: 887-889. PMID: 33403699, DOI: 10.1002/hep.31702.Peer-Reviewed Original Research
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 ResearchImmunosuppressive Treatment Regimens in Autoimmune Hepatitis: Systematic Reviews and Meta‐Analyses Supporting American Association for the Study of Liver Diseases Guidelines
Vierling JM, Kerkar N, Czaja AJ, Mack CL, Adams D, Assis D, Manns MP, Mayo MJ, Nayfeh T, Majzoub AMM, Alzuabi MA, Ding J, Haffar S, Murad MH, Alsawas M. Immunosuppressive Treatment Regimens in Autoimmune Hepatitis: Systematic Reviews and Meta‐Analyses Supporting American Association for the Study of Liver Diseases Guidelines. Hepatology 2020, 72: 753-769. PMID: 32500593, DOI: 10.1002/hep.31407.Peer-Reviewed Original ResearchFenofibrate 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