2025
WS16.05Impact of elexacaftor/tezacaftor/ivacaftor on glucose tolerance in adolescents with cystic fibrosis. Data from the MODUL-CF study
Bonnel A, Galderisi A, Weiss L, Sermet-Gaudelus I, Besancon A, Letierce A, Sahki D, Group M. WS16.05Impact of elexacaftor/tezacaftor/ivacaftor on glucose tolerance in adolescents with cystic fibrosis. Data from the MODUL-CF study. Journal Of Cystic Fibrosis 2025, 24: s33. DOI: 10.1016/j.jcf.2025.03.586.Peer-Reviewed Original ResearchOral glucose tolerance testAbnormal glucose toleranceNormal glucose toleranceCF-related diabetesBaseline oral glucose tolerance testAbnormal glucose tolerance groupCystic fibrosisGlucose toleranceNormal glucose tolerance groupEffective CFTR modulatorsOGTT glucoseBMI z-scoreYear of treatmentImpaired fasting glucoseImpaired glucose toleranceGlucose tolerance testForced expiratory volumeCFTR modulatorsPulmonary infectionDecrease recurrenceBaseline characteristicsRelated diabetesFasting glucoseTolerance testInsulin treatmentEffect of Elexacaftor/Tezacaftor/Ivacaftor on Glucose Tolerance in Adolescents With Cystic Fibrosis
Galderisi A, Weiss L, Besançon A, Stremler N, Reix P, Wizla N, Lustre A, Rames C, Tatopoulos A, Perisson C, Dalphin M, Troussier F, Houdouin V, Bessaci K, Cosson L, Gabsi A, Corvol H, Deneuville E, Storni V, Ramel S, Bui S, Heraud M, Remus N, Huet F, Scalbert M, Mely L, Gachelin E, Giannantonio M, Letierce A, Sahki D, Marguet C, Bonnel A, Sermet-Gaudelus I. Effect of Elexacaftor/Tezacaftor/Ivacaftor on Glucose Tolerance in Adolescents With Cystic Fibrosis. The Journal Of Clinical Endocrinology & Metabolism 2025, dgaf099. PMID: 39977216, DOI: 10.1210/clinem/dgaf099.Peer-Reviewed Original ResearchOral glucose tolerance testBaseline oral glucose tolerance testAbnormal glucose toleranceNormal glucose toleranceAbnormal glucose tolerance groupCystic fibrosisGlucose toleranceNormal glucose tolerance groupEffective CFTR modulatorsCF-related diabetesInsulin secretionImpaired fasting glucoseGlucose tolerance testImpaired glucose toleranceForced expiratory volumeBMIz-scoreCFTR modulatorsBaseline characteristicsRelated diabetesFasting glucoseTolerance testTherapeutic strategiesInsulin treatmentExpiratory volumeGlucose levels
2024
CCR2+ monocytes are dispensable to resolve acute pulmonary Pseudomonas aeruginosa infections in WT and cystic fibrosis mice
Öz H, Braga C, Gudneppanavar R, Di Pietro C, Huang P, Zhang P, Krause D, Egan M, Murray T, Bruscia E. CCR2+ monocytes are dispensable to resolve acute pulmonary Pseudomonas aeruginosa infections in WT and cystic fibrosis mice. Journal Of Leukocyte Biology 2024, 117: qiae218. PMID: 39365279, PMCID: PMC11953069, DOI: 10.1093/jleuko/qiae218.Peer-Reviewed Original ResearchLung tissue damageCystic fibrosisTissue damageMonocyte recruitmentImmune responsePulmonary Pseudomonas aeruginosa infectionHyper-inflammatory immune responseCystic fibrosis micePropagate tissue damagePseudomonas aeruginosaLungs of patientsChronic neutrophilic inflammationImmunological response to infectionHost immune responseMonocyte-derived macrophagesTarget monocyte recruitmentSite of injuryResponse to infectionCFTR modulatorsPA infectionChronic inflammatory disease conditionsReduced bactericidal activityAdjunctive therapyClinical outcomesEradicate infectionLocalization and function of humanized F508del-CFTR in mouse intestine following activation of serum glucocorticoid kinase 1 and Trikafta
Dastoor P, Muiler C, Garrison A, Egan M, Carlos Dos Reis D, Santos A, Ameen N. Localization and function of humanized F508del-CFTR in mouse intestine following activation of serum glucocorticoid kinase 1 and Trikafta. European Journal Of Pharmacology 2024, 978: 176771. PMID: 38925289, DOI: 10.1016/j.ejphar.2024.176771.Peer-Reviewed Original ResearchSerum glucocorticoid kinase 1Glucocorticoid kinase 1F508del-CFTRCystic fibrosisMouse modelF508del-CFTR miceCFTR-expressing epithelial cellsCF mouse modelsHumanized mouse modelTreatment of ratsIntestinal diseaseIntestinal segmentsKinase 1CFTR modulatorsCFTR mutationsCF patientsTrikaftaDex treatmentLung diseaseEfficacy of compoundsDelta F508CFTRCombined treatmentEpithelial cellsLoss of function
2023
Future therapies for cystic fibrosis
Allen L, Allen L, Carr S, Davies G, Downey D, Egan M, Forton J, Gray R, Haworth C, Horsley A, Smyth A, Southern K, Davies J. Future therapies for cystic fibrosis. Nature Communications 2023, 14: 693. PMID: 36755044, PMCID: PMC9907205, DOI: 10.1038/s41467-023-36244-2.Peer-Reviewed Original ResearchConceptsMutation-specific drugsCystic fibrosisSymptom-directed treatmentMultisystem clinical manifestationsCystic fibrosis therapyCystic fibrosis transmembrane conductance regulatorGenetic variantsClinical manifestationsFuture therapiesFibrosis therapyTranslational research collaborationsModulator drugsCFTR modulatorsSingle gene disordersHealth inequalitiesTherapyGene variantsImproved treatmentDrugsPatientsFibrosisFibrosis transmembrane conductance regulatorGene disordersTransmembrane conductance regulatorStrategy group
2022
Emerging Concepts in Defective Macrophage Phagocytosis in Cystic Fibrosis
Jaganathan D, Bruscia EM, Kopp BT. Emerging Concepts in Defective Macrophage Phagocytosis in Cystic Fibrosis. International Journal Of Molecular Sciences 2022, 23: 7750. PMID: 35887098, PMCID: PMC9319215, DOI: 10.3390/ijms23147750.Peer-Reviewed Original ResearchConceptsPhagosome formationCystic fibrosis transmembrane conductance regulator (CFTR) geneTransmembrane conductance regulator geneInnate immunityTissue homeostasisRegulator geneMutant CFTRCF macrophagesCystic fibrosisPhagocytic mechanismsPathogenic microbesAdaptive immune systemDefective macrophage phagocytosisCFTRCurrent understandingTherapeutic developmentCentral roleMacrophage phagocytosisCFTR modulatorsPhagocytic cellsPhagocytosisNew therapeutic developmentsMacrophages contributesLung functionChronic inflammation
2020
Cystic fibrosis transmembrane conductance receptor modulator therapy in cystic fibrosis, an update.
Egan ME. Cystic fibrosis transmembrane conductance receptor modulator therapy in cystic fibrosis, an update. Current Opinion In Pediatrics 2020, 32: 384-388. PMID: 32374578, DOI: 10.1097/mop.0000000000000892.Peer-Reviewed Original ResearchConceptsModulator therapyCystic fibrosisCFTR modulatorsLung functionElexacaftor/tezacaftor/ivacaftorEffective CFTR modulatorsEffective triple therapyTezacaftor/ivacaftorMonths of ageQuality of lifeCystic fibrosis patientsLong-term usePulmonary exacerbationsTriple therapyFirst therapyLong-term benefitsReceptor modulatorsFibrosisFibrosis patientsTherapyUnderlying causeWeight gainPatientsImproved healthCFTR mutations
2017
Cystic Fibrosis Transmembrane Regulator Modulators: Implications for the Management of Depression and Anxiety in Cystic Fibrosis
Talwalkar JS, Koff JL, Lee HB, Britto CJ, Mulenos AM, Georgiopoulos AM. Cystic Fibrosis Transmembrane Regulator Modulators: Implications for the Management of Depression and Anxiety in Cystic Fibrosis. Journal Of The Academy Of Consultation-Liaison Psychiatry 2017, 58: 343-354. PMID: 28576305, DOI: 10.1016/j.psym.2017.04.001.Peer-Reviewed Original ResearchConceptsCFTR modulator therapyCystic fibrosisModulator therapyNovel therapiesCF transmembrane regulatorCFTR modulatorsCentral nervous system functionNovel CF therapiesManagement of depressionWorse medical outcomesMental health specialistsDrug-drug interactionsNervous system functionMental health variablesTreatment guidelinesClinical outcomesMedication interactionsCF centersComorbid depressionCF RegistryAnxiety screeningCF teamNovel agentsHigh riskMedical outcomes
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