Results 1 to 10 of about 4,057 (184)

Systematic Review of Nasal Endoscopy Scores in Cystic Fibrosis Patients Treated With Cystic Fibrosis Transmembrane Conductance Regulator Modulators. [PDF]

Pediatr Pulmonol
ABSTRACT Introduction Cystic fibrosis (CF) is a severe genetic disorder caused by pathogenic variants in the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) gene, leading to multisystem complications including chronic rhinosinusitis and nasal polyposis. Recent advances in CFTR modulator therapies have revolutionized systemic disease control,
Gallardo R, Silva BS, Salgado LS, Ribeiro JD, Sakano E.   +4 more
europepmc   +2 more sources

Impact of CFTR Modulator Concentrations on Clinical Response in Cystic Fibrosis. [PDF]

Eur Respir J
Chalamalla AR, Baker E, Ryan KJ, Dowell A, Natt JR, Zemanick ET, Konstan MW, Mayer-Hamblett N, Acosta EP, Guimbellot JS, CHEC-SC investigators.   +10 more
europepmc   +1 more source

Prenatal Elexacaftor/Tezacaftor/Ivacaftor for Fetal Meconium Ileus: First Italian Case and Narrative Overview of the Emerging Evidence. [PDF]

J Clin Med
Boni A, Vassallo C, Ciciriello F, Cristiani L, Mancini A, Zucaro L, Graziano S, Goffredo BM, Alghisi F, Raponi M, Fabietti I, On Behalf Of Opbg Cf Pregnancy And Fetal Therapy Multidisciplinary Group.   +11 more
europepmc   +1 more source

Correction to: Improved quality of life in cystic fibrosis patients observed up to 36 months after starting Elexacaftor/Tezacaftor/Ivacaftor treatment. [PDF]

J Patient Rep Outcomes
Buniotto F, Tridello G, De Scolari A, Meneghelli I, Pintani E, Perobelli S, Cipolli M.   +6 more
europepmc   +1 more source

Long-term impact of elexacaftor/tezacaftor/ivacaftor on small and large airways in people with cystic fibrosis aged ≥6 years: 24-month real-world evidence from the German Cystic Fibrosis Registry. [PDF]

ERJ Open Res
Dillenhöfer S, Schütz K, Burkhart M, Ellemunter H, Kappler M, Sieber S, Naehrlich L, Brinkmann F, Sutharsan S.   +8 more
europepmc   +1 more source

Effect of elexacaftor–tezacaftor–ivacaftor modulator on lung structure in cystic fibrosis

Pulmonology, 2023
V. Fainardi, K. Skenderaj, A. Ciuni, S. Esposito, N. Sverzellati, G. Pisi   +5 more
doaj   +1 more source

Comparison of Stool Microbiome in Children with Cystic Fibrosis Treated with and Without Elexacaftor-Tezacaftor-Ivacaftor-A Pilot Study. [PDF]

Int J Mol Sci
Sankararaman S, Liu R, Sun X, Retuerto M, Schindler T, Roesch E, Sferra TJ, Drumm M, Ghannoum M, Zhang L.   +9 more
europepmc   +1 more source

Comparative efficacy and safety of CFTR modulators for people with cystic fibrosis with phe508del mutation: a systematic review and bayesian network meta-analysis. [PDF]

EClinicalMedicine
Safeer V S M, Behl S, Vaidya PC, Tiwari P, Bharti SK, Nahan N, Kumar Sahu J, Bansal D.   +7 more
europepmc   +1 more source

Elexacaftor, Tezacaftor, and Ivacaftor

American Journal of Health-System Pharmacy, 2020
openaire   +1 more source

Changes in sputum viscoelastic properties and airway inflammation in primary ciliary dyskinesia are comparable to cystic fibrosis on elexacaftor/tezacaftor/ivacaftor therapy. [PDF]

Eur Respir J
Nussstein H, Urbantat RM, Fentker K, Loewe A, Duerr J, Haji M, Doellinger F, Stahl M, Graeber SY, Gradzielski M, Röhmel J, Mertins P, Schaupp L, Mall MA.   +13 more
europepmc   +1 more source