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 surveyAutoimmune hepatitis: Brighton Collaboration case definition and guidelines for data collection, analysis, and presentation of immunisation safety data
Kochhar S, Assis D, Mack C, Izurieta H, Muratori L, Munoz A, Nordenberg D, Gidudu J, Blau E, Vierling J. Autoimmune hepatitis: Brighton Collaboration case definition and guidelines for data collection, analysis, and presentation of immunisation safety data. Vaccine 2024, 42: 1812-1825. PMID: 38368225, DOI: 10.1016/j.vaccine.2024.01.021.Peer-Reviewed Original ResearchAutoimmune hepatitisCase definitionExclusion of alternative diagnosesBrighton CollaborationLevel of diagnostic certaintyBrighton Collaboration case definitionStandardised case definitionsAdverse eventsMiddle-income countriesAlternative diagnosesClinical trialsBackground incidenceSafety dataProcess expertsStudy of safetyClinical case managementDiagnostic certaintyHealthcare accessControl groupData collectionCOVID-19 vaccineExpert consensusCase managementBiochemical testsSystematic reviewAfrican 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 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 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 patientsEffects of immunosuppressive drugs on COVID-19 severity in patients with autoimmune hepatitis.
Efe C, Lammert C, Taşçılar K, Dhanasekaran R, Ebik B, Higuera-de la Tijera F, Calışkan AR, Peralta M, Gerussi A, Massoumi H, Catana AM, Purnak T, Rigamonti C, Aldana AJG, Khakoo N, Nazal L, Frager S, Demir N, Irak K, Melekoğlu-Ellik Z, Kacmaz H, Balaban Y, Atay K, Eren F, Alvares-da-Silva MR, Cristoferi L, Urzua Á, Eşkazan T, Magro B, Snijders R, Barutçu S, Lytvyak E, Zazueta GM, Demirezer-Bolat A, Aydın M, Heurgue-Berlot A, De Martin E, Ekin N, Yıldırım S, Yavuz A, Bıyık M, Narro GC, Kıyıcı M, Akyıldız M, Kahramanoğlu-Aksoy E, Vincent M, Carr RM, Günşar F, Reyes EC, Harputluoğlu M, Aloman C, Gatselis NK, Üstündağ Y, Brahm J, Vargas NCE, Güzelbulut F, Garcia SR, Aguirre J, Anders M, Ratusnu N, Hatemi I, Mendizabal M, Floreani A, Fagiuoli S, Silva M, Idilman R, Satapathy SK, Silveira M, Drenth JPH, Dalekos GN, Assis DN, Björnsson E, Boyer JL, Yoshida EM, Invernizzi P, Levy C, Montano-Loza AJ, Schiano TD, Ridruejo E, Wahlin S. Effects of immunosuppressive drugs on COVID-19 severity in patients with autoimmune hepatitis. Liver International : Official Journal Of The International Association For The Study Of The Liver 2022, 42: 607-614. PMID: 34846800, DOI: 10.1111/liv.15121.Peer-Reviewed Original ResearchMindfulness-based stress reduction may decrease stress, disease activity, and inflammatory cytokine levels in patients with autoimmune hepatitis
Alrabadi LS, Dutton A, Rabiee A, Roberts SJ, Deng Y, Cusack L, Silveira MG, Ciarleglio M, Bucala R, Sinha R, Boyer JL, Assis DN. Mindfulness-based stress reduction may decrease stress, disease activity, and inflammatory cytokine levels in patients with autoimmune hepatitis. JHEP Reports 2022, 4: 100450. PMID: 35434588, PMCID: PMC9011026, DOI: 10.1016/j.jhepr.2022.100450.Peer-Reviewed Original ResearchQuality of lifeMindfulness-based stress reductionInflammatory cytokine levelsDisease activityCytokine levelsAutoimmune hepatitisAlanine aminotransferaseAdult patientsTRIAL REGISTRATIONPeripheral blood cytokine levelsScore improvementMBSR programPilot studyBlood cytokine levelsSerum alanine aminotransferaseClinical trial registrationSignificant dose reductionPerceived stressExploratory pilot studyALT levelsSteroid requirementsStress reductionCytokine mediatorsALT reductionMedication needsPREDICTORS AND OUTCOMES OF BIOLOGIC/IMMUNOSUPPRESSIVE THERAPY FOR PSC-ASSOCIATED IBD
Sayed A, Silveira M, Assis D, Deng Y, Gaidos J, Proctor D, Al-Bawardy B. PREDICTORS AND OUTCOMES OF BIOLOGIC/IMMUNOSUPPRESSIVE THERAPY FOR PSC-ASSOCIATED IBD. Gastroenterology 2022, 162: s76. DOI: 10.1053/j.gastro.2021.12.161.Peer-Reviewed Original Research