Long-term therapy with elexacaftor/tezacaftor/ivacaftor improves cystic fibrosis lung disease and monocyte function.

Συγγραφείς: Sangiorgi G; Department of Biology and Biotechnology 'C. Darwin' Sapienza University, Rome, Italy., Cavinato L; Department of Molecular Microbiology, Washington University in St Louis, Missouri, USA., Cristoferi M; Department of Biology and Biotechnology 'C. Darwin' Sapienza University, Rome, Italy., Chiappetta D; Department of Basic and Applied Sciences for Engineering, Sapienza University, Rome, Italy., Pastore V; Department of Biology and Biotechnology 'C. Darwin' Sapienza University, Rome, Italy., Cimino G; Regional Cystic Fibrosis Center, AOU Policlinico Umberto I - Sapienza University, Rome, Italy., Cimino L; Regional Cystic Fibrosis Center, AOU Policlinico Umberto I - Sapienza University, Rome, Italy., Ascenzioni F; Department of Biology and Biotechnology 'C. Darwin' Sapienza University, Rome, Italy., Del Porto P; Department of Biology and Biotechnology 'C. Darwin' Sapienza University, Rome, Italy. Electronic address: paola.delporto@uniroma1.it.

Πηγή: Respiratory medicine [Respir Med] 2026 Aug-Sep; Vol. 260, pp. 108930. Date of Electronic Publication: 2026 Jun 04.

Τύπος έκδοσης: Journal Article

Στοιχεία περιοδικού: Publisher: Elsevier Country of Publication: England NLM ID: 8908438 Publication Model: Print-Electronic Cited Medium: Internet ISSN: 1532-3064 (Electronic) Linking ISSN: 09546111 NLM ISO Abbreviation: Respir Med Subsets: MEDLINE

Ιατρικοί όροι (MeSH): Monocytes*/drug effects , Monocytes*/metabolism , Monocytes*/physiology , Cystic Fibrosis*/drug therapy , Cystic Fibrosis*/physiopathology , Cystic Fibrosis*/microbiology , Aminophenols*/therapeutic use , Aminophenols*/pharmacology , Indoles*/therapeutic use , Indoles*/pharmacology , Indoles*/administration & dosage , Benzodioxoles*/therapeutic use , Benzodioxoles*/pharmacology , Quinolones*/therapeutic use , Quinolones*/pharmacology , Quinolones*/administration & dosage , Pyridines*/therapeutic use , Pyridines*/pharmacology , Pyrazoles*/therapeutic use , Pyrrolidines*/therapeutic use , Pyrroles*/therapeutic use, Cystic Fibrosis Transmembrane Conductance Regulator/metabolism ; Cystic Fibrosis Transmembrane Conductance Regulator/genetics ; Phagocytosis/drug effects ; Pseudomonas aeruginosa/drug effects ; RNA, Messenger/metabolism ; Pseudomonas Infections/drug therapy ; Chloride Channel Agonists/therapeutic use ; Humans ; Female ; Male ; Drug Combinations ; Adult ; Young Adult ; Quinolines

People with cystic fibrosis' experience of symptom burden while taking elexacaftor/tezacaftor/ivacaftor.

Συγγραφείς: Muther EF; University of Colorado Anschutz Medical Campus, Aurora, CO, USA. Electronic address: Emily.muther@childrenscolorado.org., Gibson SL; Boston Children's Hospital, Boston, MA, USA., Ford C; Johns Hopkins University, Baltimore, MD, USA., Duncan C; Oklahoma State University, Stillwater, OK, USA., Riekert KA; Johns Hopkins University, Baltimore, MD, USA., Lyons E; University of Colorado Anschutz Medical Campus, Aurora, CO, USA., Hoppe JE; University of Colorado Anschutz Medical Campus, Aurora, CO, USA., Prickett M; Northwestern University School of Medicine, Chicago, IL, USA., Isenburg MV; Community Partner, Durham, NC, USA., Everhart RS; Virginia Commonwealth University, Richmond, VA, USA.

Πηγή: Respiratory medicine [Respir Med] 2026 Aug; Vol. 259, pp. 108908. Date of Electronic Publication: 2026 May 27.

Τύπος έκδοσης: Journal Article

Στοιχεία περιοδικού: Publisher: Elsevier Country of Publication: England NLM ID: 8908438 Publication Model: Print-Electronic Cited Medium: Internet ISSN: 1532-3064 (Electronic) Linking ISSN: 09546111 NLM ISO Abbreviation: Respir Med Subsets: MEDLINE

Ιατρικοί όροι (MeSH): Benzodioxoles*/therapeutic use , Cystic Fibrosis*/drug therapy , Cystic Fibrosis*/physiopathology , Cystic Fibrosis*/psychology , Indoles*/therapeutic use , Pyrazoles*/therapeutic use , Pyridines*/therapeutic use , Pyrroles*/therapeutic use , Quinolones*/therapeutic use , Aminophenols*/adverse effects , Aminophenols*/therapeutic use, Adolescent ; Adult ; Aged ; Child ; Female ; Humans ; Male ; Middle Aged ; Young Adult ; Drug Combinations ; Surveys and Questionnaires ; Symptom Burden ; Quinolines

Medication discontinuation in the era of elexacaftor/tezacaftor/ivacaftor: an Australian survey and qualitative interview.

Συγγραφείς: Corbett M; Department of Respiratory and Sleep Medicine, Royal Prince Alfred Hospital, Sydney, New South Wales, Australia., Saini B; Faculty of Medicine and Health, University of Sydney, Sydney, New South Wales, Australia., Dunn A; Department of Respiratory and Sleep Medicine, Royal Prince Alfred Hospital, Sydney, New South Wales, Australia., Visser S; Department of Respiratory and Sleep Medicine, Royal Prince Alfred Hospital, Sydney, New South Wales, Australia.; Faculty of Medicine and Health, University of Sydney, Sydney, New South Wales, Australia., Jo H; Department of Respiratory and Sleep Medicine, Royal Prince Alfred Hospital, Sydney, New South Wales, Australia.; Faculty of Medicine and Health, University of Sydney, Sydney, New South Wales, Australia., Yozghatlian V; Department of Respiratory and Sleep Medicine, Royal Prince Alfred Hospital, Sydney, New South Wales, Australia.; Faculty of Medicine and Health, University of Sydney, Sydney, New South Wales, Australia., Bell J; Department of Respiratory and Sleep Medicine, Royal Prince Alfred Hospital, Sydney, New South Wales, Australia., Sivam S; Department of Respiratory and Sleep Medicine, Royal Prince Alfred Hospital, Sydney, New South Wales, Australia.; Faculty of Medicine and Health, University of Sydney, Sydney, New South Wales, Australia.

Πηγή: Internal medicine journal [Intern Med J] 2026 Jul; Vol. 56 (7), pp. 1240-1244. Date of Electronic Publication: 2026 Jun 19.

Τύπος έκδοσης: Journal Article

Στοιχεία περιοδικού: Publisher: Blackwell Science Asia Country of Publication: Australia NLM ID: 101092952 Publication Model: Print-Electronic Cited Medium: Internet ISSN: 1445-5994 (Electronic) Linking ISSN: 14440903 NLM ISO Abbreviation: Intern Med J Subsets: MEDLINE

Ιατρικοί όροι (MeSH): Indoles*/administration & dosage , Indoles*/therapeutic use , Cystic Fibrosis*/drug therapy , Cystic Fibrosis*/epidemiology , Benzodioxoles*/administration & dosage , Benzodioxoles*/therapeutic use , Quinolones*/administration & dosage , Quinolones*/therapeutic use , Aminophenols*/administration & dosage , Aminophenols*/therapeutic use , Chloride Channel Agonists*/administration & dosage , Chloride Channel Agonists*/therapeutic use , Pyridines*, Australia/epidemiology ; Humans ; Prospective Studies ; Surveys and Questionnaires ; Drug Combinations ; Treatment Interruption ; Pyrrolidines ; Qualitative Research ; Interviews as Topic ; Pyrazoles ; Quinolines

Effects of Elexacaftor/Tezacaftor/Ivacaftor on cough frequency, physical activity patterns, and sleep quality in adolescents and adults with cystic fibrosis.

Συγγραφείς: Van Braeckel E; Department of Respiratory Medicine, Ghent University Hospital, Ghent, Belgium; Department of Internal Medicine and Paediatrics, Faculty of Medicine and Health Sciences, Ghent University, Ghent, Belgium. Electronic address: Eva.VanBraeckel@UGent.be., Braun AT; University of Wisconsin School of Medicine and Public Health, Madison, WI, USA., Simmonds NJ; National Heart and Lung Institute, Imperial College London UK, Royal Brompton Hospital, part of Guy's and St Thomas' NHS Foundation Trust, London, UK., Barry PJ; Manchester University NHS Foundation Trust, Manchester, UK; Division of Infection, Immunity and Respiratory Medicine, University of Manchester, Manchester, UK., Davies JC; National Heart and Lung Institute, Imperial College London UK, Royal Brompton Hospital, part of Guy's and St Thomas' NHS Foundation Trust, London, UK., Egan ME; Yale University, New Haven, CT, USA., Lapey A; Massachusetts General Hospital, Boston, MA, USA., Mall MA; Department of Pediatric Respiratory Medicine, Immunology and Critical Care Medicine, Charité - Universitätsmedizin Berlin, Berlin, Germany; German Center for Lung Research (DZL), associated partner site, Berlin, Germany; German Center for Child and Adolescent Health (DZKJ), partner site Berlin, Berlin, Germany; Cluster of Excellence ImmunoPreCept, Charité - Universitätsmedizin Berlin, Berlin, Germany., McKone EF; St Vincent's University Hospital, Dublin, Ireland., Peckham D; St James's University Hospital, Leeds, UK., Proesmans M; University Hospital of Leuven, Leuven, Belgium., Ramsey B; Seattle Children's Hospital, Seattle, WA, USA., Santamaria SV; Hospital Ramón y Cajal, Madrid, Spain., Smith D; The Prince Charles Hospital, Brisbane, Queensland, Australia., Taylor-Cousar JL; National Jewish Health, Denver, CO, USA., Tullis E; St Michael's Hospital, Toronto, Ontario, Canada., De Wachter E; Universitair Ziekenhuis Brussel, Vrije Universiteit Brussel, Brussels, Belgium., Withers N; Royal Devon United Hospital NHS Foundation Trust, Exeter, UK., Ahluwalia N; Vertex Pharmaceuticals Incorporated, Boston, MA, USA., Conner S; Vertex Pharmaceuticals Incorporated, Boston, MA, USA., Jennings M; Vertex Pharmaceuticals Incorporated, Boston, MA, USA., Lou Y; Vertex Pharmaceuticals Incorporated, Boston, MA, USA., Weinstock TG; Vertex Pharmaceuticals Incorporated, Boston, MA, USA., Keating D; Alfred Health, and Monash University, Melbourne, Victoria, Australia.

Πηγή: Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society [J Cyst Fibros] 2026 Jul; Vol. 25 (4), pp. 682-688. Date of Electronic Publication: 2026 Mar 31.

Τύπος έκδοσης: Journal Article; Clinical Trial, Phase IV; Clinical Trial, Phase III

Στοιχεία περιοδικού: Publisher: Elsevier Country of Publication: Netherlands NLM ID: 101128966 Publication Model: Print-Electronic Cited Medium: Internet ISSN: 1873-5010 (Electronic) Linking ISSN: 15691993 NLM ISO Abbreviation: J Cyst Fibros Subsets: MEDLINE

Ιατρικοί όροι (MeSH): Cystic Fibrosis*/drug therapy , Cystic Fibrosis*/complications , Cystic Fibrosis*/physiopathology , Aminophenols*/therapeutic use , Aminophenols*/administration & dosage , Indoles*/therapeutic use , Indoles*/administration & dosage , Cough*/drug therapy , Cough*/etiology , Quinolones*/therapeutic use , Quinolones*/administration & dosage , Benzodioxoles*/therapeutic use , Benzodioxoles*/administration & dosage , Pyrrolidines*/therapeutic use , Pyrazoles*/therapeutic use , Pyrazoles*/administration & dosage , Pyridines*/therapeutic use , Pyridines*/administration & dosage , Exercise* , Sleep Quality*, Actigraphy/methods ; Chloride Channel Agonists/administration & dosage ; Chloride Channel Agonists/therapeutic use ; Humans ; Female ; Male ; Adolescent ; Adult ; Drug Combinations ; Pilot Projects ; Young Adult ; Feasibility Studies

Prenatal initiation of elexacaftor/tezacaftor/ivacaftor via carrier mother prevents congenital bilateral absence of vas deferens in a male infant with cystic fibrosis.

Συγγραφείς: Thee S; Department of Pediatric Respiratory Medicine, Immunology and Critical Care Medicine, Charité - Universitätsmedizin Berlin, Augustenburger Platz 1, Berlin D-13353, Germany; German Center for Child and Adolescent Health (DZKJ), Partner Site Berlin, Augustenburger Platz 1, Berlin D-13353, Germany; Berlin Institute of Health (BIH) at Charité, Anna-Louisa-Karsch-Str. 2, Berlin D-10178, Germany., Aleksander P; Department of Pediatric Respiratory Medicine, Immunology and Critical Care Medicine, Charité - Universitätsmedizin Berlin, Augustenburger Platz 1, Berlin D-13353, Germany., Lechner L; Department of Pediatric Respiratory Medicine, Immunology and Critical Care Medicine, Charité - Universitätsmedizin Berlin, Augustenburger Platz 1, Berlin D-13353, Germany; Department of Pediatric Endocrinology and Diabetology, Charité-Universitätsmedizin Berlin, Augustenburger Platz 1, Berlin D-13353, Germany., Posch H; Department of Radiology, Charité - Universitätsmedizin Berlin, Augustenburger Platz 1, Berlin D-13353, Germany., Koegel C; Department of Pediatric Radiology, Charité - Universitätsmedizin Berlin, Augustenburger Platz 1, Berlin D-13353, Germany., Mall MA; Department of Pediatric Respiratory Medicine, Immunology and Critical Care Medicine, Charité - Universitätsmedizin Berlin, Augustenburger Platz 1, Berlin D-13353, Germany; German Center for Child and Adolescent Health (DZKJ), Partner Site Berlin, Augustenburger Platz 1, Berlin D-13353, Germany; German Center for Lung Research (DZL), Associated Partner Site Berlin, Augustenburger Platz 1, Berlin D-13353, Germany., Stahl M; Department of Pediatric Respiratory Medicine, Immunology and Critical Care Medicine, Charité - Universitätsmedizin Berlin, Augustenburger Platz 1, Berlin D-13353, Germany; German Center for Child and Adolescent Health (DZKJ), Partner Site Berlin, Augustenburger Platz 1, Berlin D-13353, Germany; Berlin Institute of Health (BIH) at Charité, Anna-Louisa-Karsch-Str. 2, Berlin D-10178, Germany; German Center for Lung Research (DZL), Associated Partner Site Berlin, Augustenburger Platz 1, Berlin D-13353, Germany. Electronic address: Mirjam.stahl@charite.de.

Πηγή: Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society [J Cyst Fibros] 2026 Jul; Vol. 25 (4), pp. 609-612. Date of Electronic Publication: 2026 Feb 07.

Τύπος έκδοσης: Case Reports; Journal Article

Στοιχεία περιοδικού: Publisher: Elsevier Country of Publication: Netherlands NLM ID: 101128966 Publication Model: Print-Electronic Cited Medium: Internet ISSN: 1873-5010 (Electronic) Linking ISSN: 15691993 NLM ISO Abbreviation: J Cyst Fibros Subsets: MEDLINE

Ιατρικοί όροι (MeSH): Cystic Fibrosis*/drug therapy , Cystic Fibrosis*/genetics , Cystic Fibrosis*/diagnosis , Cystic Fibrosis*/complications , Vas Deferens*/abnormalities , Vas Deferens*/diagnostic imaging , Benzodioxoles*/therapeutic use , Benzodioxoles*/administration & dosage , Indoles*/therapeutic use , Indoles*/administration & dosage , Aminophenols*/therapeutic use , Aminophenols*/administration & dosage , Pyrrolidines*/therapeutic use , Pyrrolidines*/administration & dosage, Chloride Channel Agonists/therapeutic use ; Chloride Channel Agonists/administration & dosage ; Cystic Fibrosis Transmembrane Conductance Regulator/genetics ; Prenatal Diagnosis/methods ; Humans ; Male ; Infant, Newborn ; Pregnancy ; Female ; Drug Combinations ; Heterozygote ; Pyrazoles ; Pyridines ; Quinolines ; Male Urogenital Diseases

SCR Disease Name: Congenital bilateral aplasia of vas deferens

Clinical, functional and therapeutic evaluation of CFTR variant I507del.

Συγγραφείς: Sharma N; Department of Genetic Medicine, Johns Hopkins University School of Medicine, Baltimore, MD, USA. Electronic address: nsharma5@jh.edu., Starego K; Department of Genetic Medicine, Johns Hopkins University School of Medicine, Baltimore, MD, USA., Li H; School of Physiology, Pharmacology and Neuroscience, University of Bristol, Bristol, UK., Kleist AB; Department of Pediatrics, Johns Hopkins University School of Medicine, Baltimore, MD, USA., Cox G; Department of Genetic Medicine, Johns Hopkins University School of Medicine, Baltimore, MD, USA., Havens M; Department of Genetic Medicine, Johns Hopkins University School of Medicine, Baltimore, MD, USA., Neneh JS; Department of Genetic Medicine, Johns Hopkins University School of Medicine, Baltimore, MD, USA., Parr A; Department of Genetic Medicine, Johns Hopkins University School of Medicine, Baltimore, MD, USA., Merlo NA; Department of Genetic Medicine, Johns Hopkins University School of Medicine, Baltimore, MD, USA., Ren CL; Children's Hospital of Philadelphia and University of Pennsylvania Perelman School of Medicine, Philadelphia, PA USA., Vanscoy L; Department of Pediatrics, Johns Hopkins University School of Medicine, Baltimore, MD, USA., Raraigh KS; Department of Genetic Medicine, Johns Hopkins University School of Medicine, Baltimore, MD, USA., Merlo C; Department of Critical Care Medicine, Johns Hopkins University School of Medicine, Baltimore MD, USA., Sheppard DN; School of Physiology, Pharmacology and Neuroscience, University of Bristol, Bristol, UK., Cutting GR; Department of Genetic Medicine, Johns Hopkins University School of Medicine, Baltimore, MD, USA.

Πηγή: Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society [J Cyst Fibros] 2026 Jul; Vol. 25 (4), pp. 745-752. Date of Electronic Publication: 2026 Feb 25.

Τύπος έκδοσης: Journal Article

Στοιχεία περιοδικού: Publisher: Elsevier Country of Publication: Netherlands NLM ID: 101128966 Publication Model: Print-Electronic Cited Medium: Internet ISSN: 1873-5010 (Electronic) Linking ISSN: 15691993 NLM ISO Abbreviation: J Cyst Fibros Subsets: MEDLINE

Ιατρικοί όροι (MeSH): Cystic Fibrosis Transmembrane Conductance Regulator*/genetics , Cystic Fibrosis Transmembrane Conductance Regulator*/metabolism , Cystic Fibrosis Transmembrane Conductance Regulator*/drug effects , Cystic Fibrosis*/genetics , Cystic Fibrosis*/drug therapy , Cystic Fibrosis*/metabolism , Aminophenols*/therapeutic use , Aminophenols*/pharmacology , Benzodioxoles*/therapeutic use , Benzodioxoles*/pharmacology , Quinolones*/therapeutic use , Quinolones*/pharmacology , Indoles*/therapeutic use , Indoles*/pharmacology, Oximes/therapeutic use ; Oximes/pharmacology ; Chloride Channel Agonists/therapeutic use ; Chloride Channel Agonists/pharmacology ; Pyrazoles/therapeutic use ; Pyrazoles/pharmacology ; Pyridines/therapeutic use ; Pyridines/pharmacology ; Nasal Mucosa/metabolism ; Humans ; Drug Combinations ; Cells, Cultured ; Pyrrolidines ; Quinolines

Fetal Transplacental Therapy to Mitigate Consequences of Cystic Fibrosis.

Συγγραφείς: Kam CW; Department of Pharmacy, University of North Carolina Hospitals, Chapel Hill, North Carolina., McKinzie CJ; Department of Pharmacy, University of North Carolina Hospitals, Chapel Hill, North Carolina., Goralski JL; Division of Pediatric Pulmonology, The University of North Carolina at Chapel Hill, Chapel Hill, North Carolina; Division of Pulmonary/Critical Care Medicine, The University of North Carolina at Chapel Hill, Chapel Hill, North Carolina. Electronic address: Jennifer_goralski@med.unc.edu.

Πηγή: Clinical therapeutics [Clin Ther] 2026 Jul; Vol. 48 (7), pp. 571-582. Date of Electronic Publication: 2026 Mar 13.

Τύπος έκδοσης: Journal Article; Review

Στοιχεία περιοδικού: Publisher: Excerpta Medica Country of Publication: United States NLM ID: 7706726 Publication Model: Print-Electronic Cited Medium: Internet ISSN: 1879-114X (Electronic) Linking ISSN: 01492918 NLM ISO Abbreviation: Clin Ther Subsets: MEDLINE

Ιατρικοί όροι (MeSH): Cystic Fibrosis*/therapy , Cystic Fibrosis*/drug therapy , Cystic Fibrosis*/genetics , Aminophenols*/therapeutic use , Aminophenols*/administration & dosage , Benzodioxoles*/therapeutic use , Benzodioxoles*/administration & dosage , Fetal Therapies*/methods, Cystic Fibrosis Transmembrane Conductance Regulator/genetics ; Humans ; Female ; Pregnancy ; Maternal-Fetal Exchange

Impact of CFTR modulator concentrations on clinical response in cystic fibrosis.

Συγγραφείς: Chalamalla AR; Arkansas Children's Research Institute, Little Rock, AR, USA., Baker E; University of Alabama at Birmingham, Birmingham, AL, USA., Ryan KJ; University of Alabama at Birmingham, Birmingham, AL, USA., Dowell A; University of Alabama at Birmingham, Birmingham, AL, USA., Natt JR; University of Alabama at Birmingham, Birmingham, AL, USA., Zemanick ET; University of Colorado Anschutz Medical Campus, Aurora, CO, USA., Konstan MW; Case Western Reserve University, Cleveland, OH, USA., Mayer-Hamblett N; Seattle Children's Hospital, Seattle, WA, USA.; University of Washington, Seattle, WA, USA., Acosta EP; University of Alabama at Birmingham, Birmingham, AL, USA., Guimbellot JS; Arkansas Children's Research Institute, Little Rock, AR, USA jguimbellot@uams.edu.; University of Arkansas Medical Center, Little Rock, AR, USA.

Συλλογικό Έργο: CHEC-SC investigators

Πηγή: The European respiratory journal [Eur Respir J] 2026 Jun 12; Vol. 67 (6). Date of Electronic Publication: 2026 Jun 12 (Print Publication: 2026).

Τύπος έκδοσης: Journal Article; Multicenter Study; Observational Study

Στοιχεία περιοδικού: Publisher: European Respiratory Society Country of Publication: England NLM ID: 8803460 Publication Model: Electronic-Print Cited Medium: Internet ISSN: 1399-3003 (Electronic) Linking ISSN: 09031936 NLM ISO Abbreviation: Eur Respir J Subsets: MEDLINE

Ιατρικοί όροι (MeSH): Aminophenols*/therapeutic use , Aminophenols*/blood , Aminophenols*/administration & dosage , Benzodioxoles*/therapeutic use , Benzodioxoles*/blood , Benzodioxoles*/administration & dosage , Benzodioxoles*/pharmacokinetics , Cystic Fibrosis*/drug therapy , Cystic Fibrosis*/genetics , Cystic Fibrosis*/metabolism , Cystic Fibrosis Transmembrane Conductance Regulator*/genetics , Cystic Fibrosis Transmembrane Conductance Regulator*/metabolism , Indoles*/therapeutic use , Indoles*/blood , Indoles*/administration & dosage , Indoles*/pharmacokinetics , Quinolones*/therapeutic use , Quinolones*/blood , Quinolones*/administration & dosage , Quinolones*/pharmacokinetics , Quinuclidines*/therapeutic use , Quinuclidines*/blood , Quinuclidines*/administration & dosage , Quinuclidines*/pharmacokinetics, Chlorides/metabolism ; Chlorides/analysis ; Cytochrome P-450 CYP3A/genetics ; Pyrazoles/therapeutic use ; Pyrazoles/blood ; Pyrazoles/administration & dosage ; Pyridines/therapeutic use ; Pyridines/blood ; Pyrrolidines/therapeutic use ; Pyrrolidines/blood ; Sweat/chemistry ; Adolescent ; Adult ; Child ; Female ; Humans ; Male ; Young Adult ; Genotype ; Treatment Outcome ; Drug Combinations ; Quinolines

Physiologically Based Pharmacokinetic Modeling of Elexacaftor/Tezacaftor/Ivacaftor in Infants With Cystic Fibrosis.

Συγγραφείς: Truong NH; Inserm, Pharmacologie et évaluations Des thérapeutiques Chez L'enfant et la Femme Enceinte, Université Paris Cité, Paris, France.; Service De Pharmacologie Périnatale, Pédiatrique et Adulte, Hôpital Cochin, Hôpital Européen Georges Pompidou, Assistance Publique Hôpitaux De Paris, Paris, France., Benaboud S; Inserm, Pharmacologie et évaluations Des thérapeutiques Chez L'enfant et la Femme Enceinte, Université Paris Cité, Paris, France.; Service De Pharmacologie Périnatale, Pédiatrique et Adulte, Hôpital Cochin, Hôpital Européen Georges Pompidou, Assistance Publique Hôpitaux De Paris, Paris, France., Bouazza N; Inserm, Pharmacologie et évaluations Des thérapeutiques Chez L'enfant et la Femme Enceinte, Université Paris Cité, Paris, France.; Unité de Recherche Clinique Necker Cochin, Paris, France.; CIC-1419 Inserm, Cochin-Necker, Paris, France., Deneuville E; Centre de Ressources et de Compétences de la Mucoviscidose, Hôpitaux Universitaires de Rennes, Rennes, France., Mornand A; Department of Women, Child and Adolescent, Pediatric Pulmonology, University Geneva Hospitals, Geneva, Switzerland., Reix P; Centre de Ressources et de Compétences Pour la Mucoviscidose, Site Pédiatrique, Hôpital Femme Mère Enfant, Hospices Civils de Lyon, Lyon, France., Ribault M; Centre de Ressources et de Compétences de la Mucoviscidose, Hôpitaux Universitaires de Rennes, Rennes, France., Burgel PR; Institut Cochin, Inserm U1016, Université Paris Cité, Paris, France.; Department of Respiratory Medicine and French National Cystic Fibrosis Reference Center, Hôpital Cochin, AP-HP, Paris, France., Froelicher-Bournaud L; Inserm, Pharmacologie et évaluations Des thérapeutiques Chez L'enfant et la Femme Enceinte, Université Paris Cité, Paris, France.; Service De Pharmacologie Périnatale, Pédiatrique et Adulte, Hôpital Cochin, Hôpital Européen Georges Pompidou, Assistance Publique Hôpitaux De Paris, Paris, France., Rouillon S; Inserm, Pharmacologie et évaluations Des thérapeutiques Chez L'enfant et la Femme Enceinte, Université Paris Cité, Paris, France., Sanoufi MR; Inserm, Pharmacologie et évaluations Des thérapeutiques Chez L'enfant et la Femme Enceinte, Université Paris Cité, Paris, France.; Service De Pharmacologie Périnatale, Pédiatrique et Adulte, Hôpital Cochin, Hôpital Européen Georges Pompidou, Assistance Publique Hôpitaux De Paris, Paris, France., Lui G; Inserm, Pharmacologie et évaluations Des thérapeutiques Chez L'enfant et la Femme Enceinte, Université Paris Cité, Paris, France., Urien S; Inserm, Pharmacologie et évaluations Des thérapeutiques Chez L'enfant et la Femme Enceinte, Université Paris Cité, Paris, France.; Unité de Recherche Clinique Necker Cochin, Paris, France.; CIC-1419 Inserm, Cochin-Necker, Paris, France., Tréluyer JM; Inserm, Pharmacologie et évaluations Des thérapeutiques Chez L'enfant et la Femme Enceinte, Université Paris Cité, Paris, France.; Service De Pharmacologie Périnatale, Pédiatrique et Adulte, Hôpital Cochin, Hôpital Européen Georges Pompidou, Assistance Publique Hôpitaux De Paris, Paris, France.; Unité de Recherche Clinique Necker Cochin, Paris, France.; CIC-1419 Inserm, Cochin-Necker, Paris, France., Sermet-Gaudelus I; Hôpital Necker Enfants Malades, Centre De Référence Maladies Rares Mucoviscidose et Maladies Apparentées, Paris, France.; Université de Paris Cité, Paris, France.; Institut Necker Enfants Malades, INSERM U1151, Paris, France., Foissac F; Inserm, Pharmacologie et évaluations Des thérapeutiques Chez L'enfant et la Femme Enceinte, Université Paris Cité, Paris, France.; Unité de Recherche Clinique Necker Cochin, Paris, France.; CIC-1419 Inserm, Cochin-Necker, Paris, France.

Συλλογικό Έργο: Modul‐CF study group

Πηγή: CPT: pharmacometrics & systems pharmacology [CPT Pharmacometrics Syst Pharmacol] 2026 Jun; Vol. 15 (6), pp. e70225.

Τύπος έκδοσης: Journal Article

Στοιχεία περιοδικού: Publisher: Wiley Country of Publication: United States NLM ID: 101580011 Publication Model: Print Cited Medium: Internet ISSN: 2163-8306 (Electronic) Linking ISSN: 21638306 NLM ISO Abbreviation: CPT Pharmacometrics Syst Pharmacol Subsets: MEDLINE

Ιατρικοί όροι (MeSH): Cystic Fibrosis*/drug therapy , Cystic Fibrosis*/metabolism , Quinolones*/pharmacokinetics , Quinolones*/administration & dosage , Aminophenols*/pharmacokinetics , Aminophenols*/administration & dosage , Indoles*/pharmacokinetics , Indoles*/administration & dosage , Pyrazoles*/pharmacokinetics , Pyrazoles*/administration & dosage , Pyridines*/pharmacokinetics , Pyridines*/administration & dosage , Benzodioxoles*/pharmacokinetics , Benzodioxoles*/administration & dosage , Chloride Channel Agonists*/pharmacokinetics , Chloride Channel Agonists*/administration & dosage , Models, Biological*, Humans ; Infant ; Drug Combinations ; Male ; Female ; Quinolines

Lumacaftor-Ivacaftor in Pediatric Patients With Cystic Fibrosis and Advanced Liver Disease: A Pilot Study.

Συγγραφείς: Lim AYL; Department of Respiratory and Sleep Medicine, Queensland Children's Hospital, Brisbane, Queensland, Australia; University of Queensland Centre for Clinical Research, Faculty of Health, Medicine and Behavioural Sciences, The University of Queensland, Brisbane, Queensland, Australia. Electronic address: adeline.lim@health.qld.gov.au., Hernández-Mitre MP; University of Queensland Centre for Clinical Research, Faculty of Health, Medicine and Behavioural Sciences, The University of Queensland, Brisbane, Queensland, Australia., Lewindon PJ; Department of Gastroenterology, Queensland Children's Hospital, Brisbane, Queensland, Australia., Roberts JA; University of Queensland Centre for Clinical Research, Faculty of Health, Medicine and Behavioural Sciences, The University of Queensland, Brisbane, Queensland, Australia; Departments of Pharmacy and Intensive Care Medicine, Royal Brisbane and Women's Hospital, Brisbane, Queensland, Australia; Herston Infectious Diseases Institute (HeIDI), Metro North Health, Brisbane, Queensland, Australia; UR UM 103, University of Montpellier, Division of Anesthesia Critical Care and Emergency and Pain Medicine, Nimes University Hospital, Nimes, France., Schneider-Futschik EK; Department of Biochemistry and Pharmacology, Bio21 Molecular Science and Biotechnology Institute, School of Biomedical Sciences, Faculty of Medicine, Dentistry and Health Sciences, The University of Melbourne, Parkville, Victoria, Australia., Wainwright CE; Department of Respiratory and Sleep Medicine, Queensland Children's Hospital, Brisbane, Queensland, Australia; University of Queensland Centre for Clinical Research, Faculty of Health, Medicine and Behavioural Sciences, The University of Queensland, Brisbane, Queensland, Australia.

Πηγή: Clinical therapeutics [Clin Ther] 2026 Jun; Vol. 48 (6), pp. 534-538. Date of Electronic Publication: 2026 Apr 23.

Τύπος έκδοσης: Journal Article

Στοιχεία περιοδικού: Publisher: Excerpta Medica Country of Publication: United States NLM ID: 7706726 Publication Model: Print-Electronic Cited Medium: Internet ISSN: 1879-114X (Electronic) Linking ISSN: 01492918 NLM ISO Abbreviation: Clin Ther Subsets: MEDLINE

Ιατρικοί όροι (MeSH): Cystic Fibrosis*/drug therapy , Cystic Fibrosis*/complications , Aminophenols*/pharmacokinetics , Aminophenols*/therapeutic use , Aminophenols*/adverse effects , Aminophenols*/administration & dosage , Benzodioxoles*/pharmacokinetics , Benzodioxoles*/therapeutic use , Benzodioxoles*/adverse effects , Benzodioxoles*/administration & dosage , Quinolones*/pharmacokinetics , Quinolones*/therapeutic use , Quinolones*/adverse effects , Quinolones*/administration & dosage , Aminopyridines*/pharmacokinetics , Aminopyridines*/therapeutic use , Aminopyridines*/adverse effects , Aminopyridines*/administration & dosage , Liver Diseases*/drug therapy , Liver Diseases*/etiology, Cystic Fibrosis Transmembrane Conductance Regulator/genetics ; Humans ; Child ; Pilot Projects ; Adolescent ; Male ; Female ; Drug Combinations ; Child, Preschool ; Liver Function Tests

Impact of elexacaftor/tezacaftor/ivacaftor on the presence of bacterial and fungal pathogens in the lower respiratory tract of children with cystic fibrosis.

Συγγραφείς: Setnikar Kimovec G; Department of Paediatric Pulmonology, University Children's Hospital Ljubljana, University Medical Centre Ljubljana, Ljubljana, Slovenia., Seme K; Institute of Microbiology and Immunology, Faculty of Medicine, University of Ljubljana, Ljubljana, Slovenia., Matos T; Institute of Microbiology and Immunology, Faculty of Medicine, University of Ljubljana, Ljubljana, Slovenia., Cvitković Špik V; Institute of Microbiology and Immunology, Faculty of Medicine, University of Ljubljana, Ljubljana, Slovenia., Krivec U; Department of Paediatric Pulmonology, University Children's Hospital Ljubljana, University Medical Centre Ljubljana, Ljubljana, Slovenia; University of Ljubljana, Faculty of Medicine, Department of Paediatrics, Ljubljana, Slovenia., Praprotnik M; Department of Paediatric Pulmonology, University Children's Hospital Ljubljana, University Medical Centre Ljubljana, Ljubljana, Slovenia., Rodman Berlot J; Department of Paediatric Pulmonology, University Children's Hospital Ljubljana, University Medical Centre Ljubljana, Ljubljana, Slovenia; University of Ljubljana, Faculty of Medicine, Department of Paediatrics, Ljubljana, Slovenia. Electronic address: jasna.rodman@kclj.si.

Πηγή: Respiratory medicine [Respir Med] 2026 Jun; Vol. 257, pp. 108846. Date of Electronic Publication: 2026 Apr 17.

Τύπος έκδοσης: Journal Article; Observational Study

Στοιχεία περιοδικού: Publisher: Elsevier Country of Publication: England NLM ID: 8908438 Publication Model: Print-Electronic Cited Medium: Internet ISSN: 1532-3064 (Electronic) Linking ISSN: 09546111 NLM ISO Abbreviation: Respir Med Subsets: MEDLINE

Ιατρικοί όροι (MeSH): Cystic Fibrosis*/drug therapy , Cystic Fibrosis*/microbiology , Cystic Fibrosis*/physiopathology , Indoles*/therapeutic use , Indoles*/pharmacology , Aminophenols*/therapeutic use , Aminophenols*/pharmacology , Quinolones*/therapeutic use , Quinolones*/pharmacology , Benzodioxoles*/therapeutic use , Benzodioxoles*/pharmacology , Pyridines*/therapeutic use , Pyrrolidines*/therapeutic use , Pyrroles*/therapeutic use , Pyrazoles*/therapeutic use, Sputum/microbiology ; Respiratory Tract Infections/microbiology ; Humans ; Child ; Male ; Female ; Prospective Studies ; Drug Combinations

Elexacaftor/tezacaftor/ivacaftor is safe and effectively reduces sino-nasal symptoms in people with cystic fibrosis post lung transplantation.

Συγγραφείς: Mathiesen IHM; Adult Cystic Fibrosis Center, Dept. of Infectious Diseases, Copenhagen University Hospital, Rigshospitalet, Denmark., Rönsholt FF; Dept. of Cardiology, Section for Lung Transplantation, Copenhagen University Hospital, Rigshospitalet, Denmark. Electronic address: froe0010@regionh.dk., Aanæs K; Dept. of Otorhinolaryngology, Head and Neck Surgery and Audiology, Copenhagen University Hospital, Rigshospitalet, Denmark., Bille J; Dept. of Otorhinolaryngology, Head and Neck Surgery, Aarhus University Hospital, Denmark., von Buchwald C; Dept. of Otorhinolaryngology, Head and Neck Surgery and Audiology, Copenhagen University Hospital, Rigshospitalet, Denmark; Dept. of Clinical Medicine, University of Copenhagen, Copenhagen, Denmark., Jensen-Fangel S; Adult Cystic Fibrosis Center, Dept. of Infectious Diseases, Aarhus University Hospital, Denmark; Dept. of Clinical Medicine, Aarhus University, Aarhus, Denmark., Qvist T; Adult Cystic Fibrosis Center, Dept. of Infectious Diseases, Copenhagen University Hospital, Rigshospitalet, Denmark., Katzenstein TL; Adult Cystic Fibrosis Center, Dept. of Infectious Diseases, Copenhagen University Hospital, Rigshospitalet, Denmark., Bendstrup E; Center for Rare Lung Diseases, Aarhus University Hospital, Denmark; Dept. of Clinical Medicine, Aarhus University, Aarhus, Denmark., Perch M; Dept. of Cardiology, Section for Lung Transplantation, Copenhagen University Hospital, Rigshospitalet, Denmark; Dept. of Clinical Medicine, University of Copenhagen, Copenhagen, Denmark., Jeppesen M; Adult Cystic Fibrosis Center, Dept. of Infectious Diseases, Aarhus University Hospital, Denmark; Dept. of Clinical Medicine, Aarhus University, Aarhus, Denmark.

Πηγή: Respiratory medicine [Respir Med] 2026 Jun; Vol. 257, pp. 108807. Date of Electronic Publication: 2026 Apr 02.

Τύπος έκδοσης: Journal Article

Στοιχεία περιοδικού: Publisher: Elsevier Country of Publication: England NLM ID: 8908438 Publication Model: Print-Electronic Cited Medium: Internet ISSN: 1532-3064 (Electronic) Linking ISSN: 09546111 NLM ISO Abbreviation: Respir Med Subsets: MEDLINE

Ιατρικοί όροι (MeSH): Cystic Fibrosis*/surgery , Cystic Fibrosis*/complications , Cystic Fibrosis*/drug therapy , Lung Transplantation*/adverse effects , Aminophenols*/therapeutic use , Aminophenols*/adverse effects , Aminophenols*/administration & dosage , Indoles*/therapeutic use , Indoles*/adverse effects , Indoles*/administration & dosage , Benzodioxoles*/therapeutic use , Benzodioxoles*/adverse effects , Benzodioxoles*/administration & dosage , Quinolones*/therapeutic use , Quinolones*/adverse effects , Quinolones*/administration & dosage , Pyridines*/therapeutic use , Pyridines*/adverse effects , Pyridines*/administration & dosage , Pyrazoles*/therapeutic use , Pyrazoles*/adverse effects , Pyrrolidines*/therapeutic use, Immunosuppressive Agents/therapeutic use ; Calcineurin Inhibitors/therapeutic use ; Chloride Channel Agonists/therapeutic use ; Humans ; Female ; Male ; Adult ; Middle Aged ; Drug Combinations ; Treatment Outcome ; Young Adult ; Adolescent ; Denmark ; Quinolines

Long-term impact of Elexacaftor/Tezacaftor/ivacaftor on pulmonary, nutritional and metabolic outcomes in homozygous F508del cystic fibrosis patients: A real-world cohort study.

Συγγραφείς: Perrotta N; Department of Physiology and Pharmacology 'V. Erspamer', Sapienza University, Rome, Italy.; Policlinico Umberto I Hospital, Sapienza University, Rome, Italy., Fiorito LA; Department of Physiology and Pharmacology 'V. Erspamer', Sapienza University, Rome, Italy.; Policlinico Umberto I Hospital, Sapienza University, Rome, Italy., Vescovo R; Department of Chemistry and Technology of Drugs, Sapienza University, Rome, Italy., Virgilio A; Department of Chemistry and Technology of Drugs, Sapienza University, Rome, Italy., Amato G; Department of Chemistry and Technology of Drugs, Sapienza University, Rome, Italy., Cimino G; Regional Cystic Fibrosis Center, Policlinico Umberto I Hospital, Sapienza University, Rome, Italy.

Πηγή: British journal of clinical pharmacology [Br J Clin Pharmacol] 2026 Jun; Vol. 92 (6), pp. 1822-1832. Date of Electronic Publication: 2026 Jan 23.

Τύπος έκδοσης: Journal Article

Στοιχεία περιοδικού: Publisher: Wiley-Blackwell Country of Publication: England NLM ID: 7503323 Publication Model: Print-Electronic Cited Medium: Internet ISSN: 1365-2125 (Electronic) Linking ISSN: 03065251 NLM ISO Abbreviation: Br J Clin Pharmacol Subsets: MEDLINE

Ιατρικοί όροι (MeSH): Cystic Fibrosis*/drug therapy , Cystic Fibrosis*/physiopathology , Cystic Fibrosis*/genetics , Cystic Fibrosis*/metabolism , Quinolones*/therapeutic use , Quinolones*/administration & dosage , Indoles*/therapeutic use , Indoles*/administration & dosage , Pyrrolidines*/therapeutic use , Pyrrolidines*/administration & dosage , Aminophenols*/therapeutic use , Aminophenols*/administration & dosage , Aminophenols*/pharmacology , Pyridines*/therapeutic use , Pyridines*/administration & dosage , Pyrazoles*/therapeutic use , Pyrazoles*/administration & dosage , Benzodioxoles*/therapeutic use, Nutritional Status/drug effects ; Cystic Fibrosis Transmembrane Conductance Regulator/genetics ; Lung/drug effects ; Lung/physiopathology ; Chloride Channel Agonists/therapeutic use ; Chloride Channel Agonists/administration & dosage ; Humans ; Female ; Adult ; Male ; Retrospective Studies ; Adolescent ; Young Adult ; Child ; Treatment Outcome

Single-cell profiling of BAL in preschool cystic fibrosis reveals macrophage dysregulation and ivacaftor-modified inflammatory programs in the early life lung.

Συγγραφείς: Maksimovic J; Computational Biology Program, Peter MacCallum Cancer Centre, Parkville, VIC, Australia; Respiratory Diseases, Murdoch Children's Research Institute, Parkville, VIC, Australia; Sir Peter MacCallum Department of Oncology, University of Melbourne, Parkville, VIC, Australia., Shanthikumar S; Respiratory Diseases, Murdoch Children's Research Institute, Parkville, VIC, Australia; Department of Paediatrics, University of Melbourne, Parkville, VIC, Australia; Respiratory and Sleep Medicine, Royal Children's Hospital, Parkville, VIC, Australia., Howitt G; Computational Biology Program, Peter MacCallum Cancer Centre, Parkville, VIC, Australia; Sir Peter MacCallum Department of Oncology, University of Melbourne, Parkville, VIC, Australia., Dixit G; Computational Biology Program, Peter MacCallum Cancer Centre, Parkville, VIC, Australia; Sir Peter MacCallum Department of Oncology, University of Melbourne, Parkville, VIC, Australia., Hickey PF; Cellular Genomics Projects Team, Advanced Technology and Biology Division, WEHI, Parkville, VIC, Australia; Department of Medical Biology, University of Melbourne, Parkville, VIC, Australia., Anttila C; Cellular Genomics Projects Team, Advanced Technology and Biology Division, WEHI, Parkville, VIC, Australia., Brown DV; Cellular Genomics Projects Team, Advanced Technology and Biology Division, WEHI, Parkville, VIC, Australia., Gubbels L; Respiratory Diseases, Murdoch Children's Research Institute, Parkville, VIC, Australia., Senabouth A; Garvan-Weizmann Centre for Cellular Genomics, Garvan Institute of Medical Research, Sydney, NSW, Australia; UNSW Cellular Genomics Futures Institute, University of New South Wales, Sydney, NSW, Australia., Amann-Zalcenstein D; Cellular Genomics Projects Team, Advanced Technology and Biology Division, WEHI, Parkville, VIC, Australia., Powell JE; Garvan-Weizmann Centre for Cellular Genomics, Garvan Institute of Medical Research, Sydney, NSW, Australia; UNSW Cellular Genomics Futures Institute, University of New South Wales, Sydney, NSW, Australia., Ranganathan SC; Respiratory Diseases, Murdoch Children's Research Institute, Parkville, VIC, Australia; Department of Paediatrics, University of Melbourne, Parkville, VIC, Australia; Respiratory and Sleep Medicine, Royal Children's Hospital, Parkville, VIC, Australia., Oshlack A; Computational Biology Program, Peter MacCallum Cancer Centre, Parkville, VIC, Australia; Sir Peter MacCallum Department of Oncology, University of Melbourne, Parkville, VIC, Australia; School of Mathematics and Statistics, University of Melbourne, Parkville, VIC, Australia., Neeland MR; Respiratory Diseases, Murdoch Children's Research Institute, Parkville, VIC, Australia; Department of Paediatrics, University of Melbourne, Parkville, VIC, Australia. Electronic address: melanie.neeland@mcri.edu.au.

Πηγή: Mucosal immunology [Mucosal Immunol] 2026 Jun; Vol. 19 (3), pp. 100338. Date of Electronic Publication: 2026 Mar 30.

Τύπος έκδοσης: Journal Article

Στοιχεία περιοδικού: Publisher: Elsevier Country of Publication: United States NLM ID: 101299742 Publication Model: Print-Electronic Cited Medium: Internet ISSN: 1935-3456 (Electronic) Linking ISSN: 19330219 NLM ISO Abbreviation: Mucosal Immunol Subsets: MEDLINE

Ιατρικοί όροι (MeSH): Quinolones*/therapeutic use , Quinolones*/pharmacology , Macrophages*/immunology , Macrophages*/drug effects , Cystic Fibrosis*/drug therapy , Cystic Fibrosis*/immunology , Aminophenols*/therapeutic use , Aminophenols*/pharmacology , Lung*/immunology , Lung*/pathology , Bronchoalveolar Lavage Fluid*/cytology , Bronchoalveolar Lavage Fluid*/immunology, Cystic Fibrosis Transmembrane Conductance Regulator/genetics ; Cystic Fibrosis Transmembrane Conductance Regulator/metabolism ; Humans ; Child, Preschool ; Single-Cell Analysis ; Female ; Transcriptome ; Single-Cell Gene Expression Analysis ; Male ; Gene Expression Profiling ; Inflammation

Impact of Elexacaftor/Tezacaftor/Ivacaftor on Systemic Antibiotic Utilization in Pediatric Patients With Cystic Fibrosis.

Συγγραφείς: Fava J; Department of Pharmacy, Nationwide Children's Hospital, Columbus, Ohio, USA., Stephan E; Department of Pharmacy, Nationwide Children's Hospital, Columbus, Ohio, USA., Tansmore J; Department of Pharmacy, Nationwide Children's Hospital, Columbus, Ohio, USA., Sheikh S; Section of Pulmonary Medicine, Nationwide Children's Hospital, Columbus, Ohio, USA., Eisner M; Center for Biostatistics, College of Medicine, The Ohio State University Wexner Medical Center, Columbus, Ohio, USA.; Biostatistics Resource at Nationwide Children's Hospital, Abigail Wexner Research Institute and Nationwide Children's Hospital, Columbus, OH, USA., Novak KJ; Department of Pharmacy, Nationwide Children's Hospital, Columbus, Ohio, USA.

Πηγή: Pediatric pulmonology [Pediatr Pulmonol] 2026 Aug; Vol. 61 (8), pp. e71749.

Τύπος έκδοσης: Journal Article

Στοιχεία περιοδικού: Publisher: Wiley-Liss Country of Publication: United States NLM ID: 8510590 Publication Model: Print Cited Medium: Internet ISSN: 1099-0496 (Electronic) Linking ISSN: 10990496 NLM ISO Abbreviation: Pediatr Pulmonol Subsets: MEDLINE

Ιατρικοί όροι (MeSH): Cystic Fibrosis*/drug therapy , Cystic Fibrosis*/complications , Anti-Bacterial Agents*/therapeutic use , Indoles*/therapeutic use , Aminophenols*/therapeutic use , Benzodioxoles*/therapeutic use , Quinolones*/therapeutic use , Pyrrolidines*/therapeutic use , Respiratory Tract Infections*/drug therapy , Respiratory Tract Infections*/etiology , Pyridines*/therapeutic use , Pyrazoles*/therapeutic use, Tetrazoles/therapeutic use ; Humans ; Child ; Retrospective Studies ; Female ; Adolescent ; Male ; Drug Combinations ; Quinolines

Three-year outcomes of elexacaftor/tezacaftor/ivacaftor in adults with cystic fibrosis: A real-world study.

Μεταγλωττισμένος Τίτλος: Elexacaftor/tezacaftor/ivacaftor en adultos con fibrosis quística: resultados a 3 años en práctica clínica real.

Συγγραφείς: Zamarrón de Lucas E; Servicio de Neumología, Hospital Universitario La Paz. IdiPAZ. Universidad Autónoma de Madrid. CIBERER, Madrid, España. Electronic address: Ester.zamarron@gmail.com., Crespo Catalán C; Servicio de Neumología, Hospital Universitario La Paz. IdiPAZ. Universidad Autónoma de Madrid. CIBERER, Madrid, España., Romero Campos B; Servicio de Neumología, Hospital Universitario La Paz. IdiPAZ. Universidad Autónoma de Madrid. CIBERER, Madrid, España., Sanz Sanjosé B; Servicio de Neumología, Hospital Universitario La Paz. IdiPAZ. Universidad Autónoma de Madrid. CIBERER, Madrid, España., Longás Ouzzine M; Servicio de Neumología, Hospital Universitario La Paz. IdiPAZ. Universidad Autónoma de Madrid. CIBERER, Madrid, España., Aponte Guevara CS; Servicio de Neumología, Hospital Universitario La Paz. IdiPAZ. Universidad Autónoma de Madrid. CIBERER, Madrid, España., Álvarez-Sala Walther R; Servicio de Neumología, Hospital Universitario La Paz. IdiPAZ. Universidad Autónoma de Madrid. CIBERER, Madrid, España., Prados Sánchez MC; Servicio de Neumología, Hospital Universitario La Paz. IdiPAZ. Universidad Autónoma de Madrid. CIBERER, Madrid, España.

Πηγή: Medicina clinica [Med Clin (Barc)] 2026 Aug; Vol. 166 (8), pp. 107448. Date of Electronic Publication: 2026 May 27.

Τύπος έκδοσης: Journal Article; Observational Study

Στοιχεία περιοδικού: Publisher: Elsevier España Country of Publication: Spain NLM ID: 0376377 Publication Model: Print-Electronic Cited Medium: Internet ISSN: 1578-8989 (Electronic) Linking ISSN: 00257753 NLM ISO Abbreviation: Med Clin (Barc) Subsets: MEDLINE

Ιατρικοί όροι (MeSH): Cystic Fibrosis*/drug therapy , Cystic Fibrosis*/microbiology , Cystic Fibrosis*/physiopathology , Benzodioxoles*/therapeutic use , Quinolones*/therapeutic use , Indoles*/therapeutic use , Aminophenols*/therapeutic use , Pyrrolidines*/therapeutic use , Chloride Channel Agonists*/therapeutic use , Pyridines*/therapeutic use, Sputum/microbiology ; Humans ; Female ; Adult ; Longitudinal Studies ; Male ; Treatment Outcome ; Drug Combinations ; Young Adult ; Forced Expiratory Volume ; Time Factors ; Pyrazoles ; Quinolines

Stage-dependent developmental and reproductive toxicity of p-aminophenol in the freshwater snail Biomphalaria glabrata: Linking organ-level pathology to ecological hazard.

Συγγραφείς: Zhang Y; Key Laboratory of Artificial Organs and Computational Medicine of Zhejiang Province, Shulan International Medical College, Zhejiang Shuren University, Hangzhou, PR China., Wang J; Key Laboratory of Artificial Organs and Computational Medicine of Zhejiang Province, Shulan International Medical College, Zhejiang Shuren University, Hangzhou, PR China; School of Basic Medicine and Forensic Medicine, Hangzhou Medical College, Hangzhou, China., Xu J; Key Laboratory of Artificial Organs and Computational Medicine of Zhejiang Province, Shulan International Medical College, Zhejiang Shuren University, Hangzhou, PR China., Li H; Key Laboratory of Artificial Organs and Computational Medicine of Zhejiang Province, Shulan International Medical College, Zhejiang Shuren University, Hangzhou, PR China., Fei X; Key Laboratory of Artificial Organs and Computational Medicine of Zhejiang Province, Shulan International Medical College, Zhejiang Shuren University, Hangzhou, PR China., Wang R; Key Laboratory of Artificial Organs and Computational Medicine of Zhejiang Province, Shulan International Medical College, Zhejiang Shuren University, Hangzhou, PR China., Shao Y; Key Laboratory of Artificial Organs and Computational Medicine of Zhejiang Province, Shulan International Medical College, Zhejiang Shuren University, Hangzhou, PR China., Miao Y; Key Laboratory of Artificial Organs and Computational Medicine of Zhejiang Province, Shulan International Medical College, Zhejiang Shuren University, Hangzhou, PR China., Wu H; Key Laboratory of Artificial Organs and Computational Medicine of Zhejiang Province, Shulan International Medical College, Zhejiang Shuren University, Hangzhou, PR China., Zhou Y; Key Laboratory of Artificial Organs and Computational Medicine of Zhejiang Province, Shulan International Medical College, Zhejiang Shuren University, Hangzhou, PR China., Zou H; Huzhou Institute for Food and Drug Control, Huzhou, China., Du L; Key Laboratory of Artificial Organs and Computational Medicine of Zhejiang Province, Shulan International Medical College, Zhejiang Shuren University, Hangzhou, PR China., Zhang X; Key Laboratory of Artificial Organs and Computational Medicine of Zhejiang Province, Shulan International Medical College, Zhejiang Shuren University, Hangzhou, PR China., Yao X; School of Marxism, Hangzhou Medical College, Hangzhou, China., Li H; Key Laboratory of Artificial Organs and Computational Medicine of Zhejiang Province, Shulan International Medical College, Zhejiang Shuren University, Hangzhou, PR China. Electronic address: hongyu88926@zjsru.edu.cn.

Πηγή: Aquatic toxicology (Amsterdam, Netherlands) [Aquat Toxicol] 2026 Aug; Vol. 297, pp. 107866. Date of Electronic Publication: 2026 May 19.

Τύπος έκδοσης: Journal Article

Στοιχεία περιοδικού: Publisher: Elsevier/North Holland Biomedical Press Country of Publication: Netherlands NLM ID: 8500246 Publication Model: Print-Electronic Cited Medium: Internet ISSN: 1879-1514 (Electronic) Linking ISSN: 0166445X NLM ISO Abbreviation: Aquat Toxicol Subsets: MEDLINE

Ιατρικοί όροι (MeSH): Biomphalaria*/drug effects , Biomphalaria*/growth & development , Water Pollutants, Chemical*/toxicity , Aminophenols*/toxicity, Reproduction/drug effects ; Animals ; Female

Contributors to the heterogeneity of response to CFTR modulators.

Συγγραφείς: Downey DG; Wellcome-Wolfson Institute for Experimental Medicine, Queen's University Belfast, Belfast, Northern Ireland. Electronic address: d.downey@qub.ac.uk., Houston C; Airway Innate Immunity Research (AiiR) Group, Queen's University Belfast, Belfast, Northern Ireland; Wellcome-Wolfson Institute for Experimental Medicine, Queen's University Belfast, Belfast, Northern Ireland., Mayer-Hamblett N; Cystic Fibrosis Therapeutics Development Network Coordinating Center, Seattle Children's Hospital, Seattle, WA, USA; Department of Pediatrics and Department of Biostatistics, University of Washington, Seattle, WA, USA; Departments of Internal Medicine and Pediatrics, National Jewish Health, Denver, CO, USA., Taylor-Cousar JL; Departments of Internal Medicine and Pediatrics, National Jewish Health, Denver, CO, USA., Taggart CC; Airway Innate Immunity Research (AiiR) Group, Queen's University Belfast, Belfast, Northern Ireland; Wellcome-Wolfson Institute for Experimental Medicine, Queen's University Belfast, Belfast, Northern Ireland.

Πηγή: The Lancet. Respiratory medicine [Lancet Respir Med] 2026 Aug; Vol. 14 (8), pp. 717-729. Date of Electronic Publication: 2026 Apr 21.

Τύπος έκδοσης: Journal Article; Review

Στοιχεία περιοδικού: Publisher: Elsevier Country of Publication: England NLM ID: 101605555 Publication Model: Print-Electronic Cited Medium: Internet ISSN: 2213-2619 (Electronic) Linking ISSN: 22132600 NLM ISO Abbreviation: Lancet Respir Med Subsets: MEDLINE

Ιατρικοί όροι (MeSH): Cystic Fibrosis*/drug therapy , Cystic Fibrosis*/genetics , Cystic Fibrosis*/physiopathology , Cystic Fibrosis Transmembrane Conductance Regulator*/genetics , Aminophenols*/therapeutic use , Quinolones*/therapeutic use , Indoles*/therapeutic use , Benzodioxoles*/therapeutic use , Pyrrolidines*/therapeutic use , Pyridines*/therapeutic use , Chloride Channel Agonists*/therapeutic use, Oxadiazoles/therapeutic use ; Pyrazoles/therapeutic use ; Pyrroles/therapeutic use ; Humans ; Treatment Effect Heterogeneity ; Drug Combinations ; Genotype ; Quinolines

Automated Ultrashort Echo Time (UTE) MRI Low Signal Volume Analysis in Stable Pediatric Cystic Fibrosis and After Elexacaftor/Tezacaftor/Ivacaftor Therapy.

Συγγραφείς: Zanette B; Translational Medicine Program, The Hospital for Sick Children, Toronto, Ontario, Canada., Bierbrier J; Translational Medicine Program, The Hospital for Sick Children, Toronto, Ontario, Canada., Munidasa S; Translational Medicine Program, The Hospital for Sick Children, Toronto, Ontario, Canada.; Department of Medical Biophysics, University of Toronto, Toronto, Ontario, Canada., Genkin D; Department of Electrical, Computer, and Biomedical Engineering, Toronto Metropolitan University, Toronto, Ontario, Canada., Kirby M; Department of Physics, Toronto Metropolitan University, Toronto, Ontario, Canada., Ratjen F; Translational Medicine Program, The Hospital for Sick Children, Toronto, Ontario, Canada.; Division of Respiratory Medicine, The Hospital for Sick Children, Toronto, Ontario, Canada., Santyr G; Translational Medicine Program, The Hospital for Sick Children, Toronto, Ontario, Canada.; Department of Medical Biophysics, University of Toronto, Toronto, Ontario, Canada.

Πηγή: Journal of magnetic resonance imaging : JMRI [J Magn Reson Imaging] 2026 Aug; Vol. 64 (2), pp. 442-451. Date of Electronic Publication: 2026 May 04.

Τύπος έκδοσης: Journal Article

Στοιχεία περιοδικού: Publisher: Wiley-Liss Country of Publication: United States NLM ID: 9105850 Publication Model: Print-Electronic Cited Medium: Internet ISSN: 1522-2586 (Electronic) Linking ISSN: 10531807 NLM ISO Abbreviation: J Magn Reson Imaging Subsets: MEDLINE

Ιατρικοί όροι (MeSH): Cystic Fibrosis*/diagnostic imaging , Cystic Fibrosis*/drug therapy , Magnetic Resonance Imaging*/methods , Quinolones*/therapeutic use , Aminophenols*/therapeutic use , Indoles*/therapeutic use , Pyrrolidines*/therapeutic use , Benzodioxoles*/therapeutic use , Pyrazoles*/therapeutic use , Pyridines*/therapeutic use, Lung/diagnostic imaging ; Image Processing, Computer-Assisted/methods ; Humans ; Female ; Male ; Adolescent ; Reproducibility of Results ; Child ; Retrospective Studies ; Drug Combinations ; Longitudinal Studies

ETI corrector therapy suppresses mitochondrial respiration independently of CFTR mutational status.

Συγγραφείς: Jha A; Department of Pediatrics, Children's Hospital of Pittsburgh, University of Pittsburgh School of Medicine, Pittsburgh, PA, 15224, USA., Mishra SK; Department of Pediatrics, Children's Hospital of Pittsburgh, University of Pittsburgh School of Medicine, Pittsburgh, PA, 15224, USA., Zhang Y; Department of Pediatrics, Children's Hospital of Pittsburgh, University of Pittsburgh School of Medicine, Pittsburgh, PA, 15224, USA., Bharathi S; Department of Pediatrics, Children's Hospital of Pittsburgh, University of Pittsburgh School of Medicine, Pittsburgh, PA, 15224, USA., Goetzman ES; Department of Pediatrics, Children's Hospital of Pittsburgh, University of Pittsburgh School of Medicine, Pittsburgh, PA, 15224, USA. Electronic address: Eric.goetzman@chp.edu.

Πηγή: Biochemical and biophysical research communications [Biochem Biophys Res Commun] 2026 Jul 02; Vol. 820, pp. 153848. Date of Electronic Publication: 2026 Apr 27.

Τύπος έκδοσης: Journal Article

Στοιχεία περιοδικού: Publisher: Elsevier Country of Publication: United States NLM ID: 0372516 Publication Model: Print-Electronic Cited Medium: Internet ISSN: 1090-2104 (Electronic) Linking ISSN: 0006291X NLM ISO Abbreviation: Biochem Biophys Res Commun Subsets: MEDLINE

Ιατρικοί όροι (MeSH): Cystic Fibrosis Transmembrane Conductance Regulator*/genetics , Cystic Fibrosis Transmembrane Conductance Regulator*/metabolism , Mitochondria*/metabolism , Mitochondria*/drug effects , Aminophenols*/pharmacology , Quinolones*/pharmacology , Pyrazoles*/pharmacology , Cystic Fibrosis*/drug therapy , Cystic Fibrosis*/genetics , Cystic Fibrosis*/metabolism , Benzodioxoles*/pharmacology , Indoles*/pharmacology , Pyrrolidines*/pharmacology , Oximes*/pharmacology , Pyridines*/pharmacology, Cell Respiration/drug effects ; Epithelial Cells/drug effects ; Epithelial Cells/metabolism ; Adenosine Triphosphate/metabolism ; Animals ; Humans ; Rats ; Mutation ; Drug Combinations ; Cell Line ; Quinolines