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 ResearchMeSH KeywordsCystic FibrosisCystic Fibrosis Transmembrane Conductance RegulatorHumansLiver DiseasesMutationConceptsComplication of cystic fibrosisCF liver diseaseHeterogeneity of clinical manifestationsManifestations of CFDisease-modifying factorsCFTR mutationsHepatobiliary complicationsBiliary stricturesBiliary cholestasisClinical manifestationsCystic fibrosisLiver diseaseTherapeutic approachesCF careDisease modelsDisease
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 committeeDiseaseComplications
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 Research
2017
Gallbladder and bile duct disease in Cystic Fibrosis
Assis DN, Debray D. Gallbladder and bile duct disease in Cystic Fibrosis. Journal Of Cystic Fibrosis 2017, 16: s62-s69. PMID: 28986023, DOI: 10.1016/j.jcf.2017.07.006.Peer-Reviewed Reviews, Practice Guidelines, Standards, and Consensus StatementsConceptsBile duct diseaseCystic fibrosisHigh-quality careDuct diseaseExtra-pulmonary manifestationsBile duct pathologyEfficient clinical evaluationHepatobiliary disturbancesCommon complicationClinical presentationBiliary diseaseDuct pathologyBiliary treeClinical evaluationBiliary disordersUndetected pathologyGallbladderDiverse disordersPatientsDiseaseCareFibrosisPathologyDisordersComplications
2015
Gastrointestinal Disorders in Cystic Fibrosis
Assis DN, Freedman SD. Gastrointestinal Disorders in Cystic Fibrosis. Clinics In Chest Medicine 2015, 37: 109-118. PMID: 26857772, DOI: 10.1016/j.ccm.2015.11.004.BooksConceptsCystic fibrosisDistal intestinal obstruction syndromeCommon GI complicationsIntestinal obstruction syndromeCare of patientsGI complicationsGastrointestinal manifestationsObstruction syndromeGI disordersGI malignanciesIntestinal overgrowthPulmonary functionClinical presentationGastrointestinal disordersPancreatic insufficiencyInnovative therapiesRecent approvalModulating agentsFibrosisDisordersComplicationsPatientsMalignancySyndromeTherapy