Long-term effectiveness and safety of cystic fibrosis transmembrane conductance regulator modulators and their impact on the lives of people with cystic fibrosis

Cancer in cystic fibrosis: a narrative review of prevalence, risk factors, screening, and treatment challenges: adult cystic fibrosis series

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Lung transplant referral for individuals with cystic fibrosis: Cystic Fibrosis Foundation consensus guidelines

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Consensus document for the selection of lung transplant candidates: an update from the International Society for Heart and Lung Transplantation

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Risk factors for chronic kidney disease in adults with cystic fibrosis

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Impact of elexacaftor/tezacaftor/ivacaftor on glucose tolerance and abnormal glucose metabolism: a phase 3b, open-label clinical trial

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The effect of elexacaftor/tezacaftor/ivacaftor (ETI) on glycemia in adults with cystic fibrosis

10.1016/j.jcf.2021.09.001 · doi-reference

Glycemia and insulin secretion in cystic fibrosis 2 years after elexacaftor/tezacaftor/ivacaftor: PROMISE-ENDO

10.1210/clinem/dgae857 · doi-reference

Elevated rates and earlier onset of non-pulmonary comorbidities in adults with cystic fibrosis: a population-based study

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Prevalence, risk factors and outcomes of cardiac disease in cystic fibrosis: a multinational retrospective cohort study

10.1183/13993003.00174-2023 · doi-reference

Cardiometabolic risk factors in adults with cystic fibrosis undergoing elexacaftor/tezacaftor/ivacaftor therapy

10.1016/j.jcf.2024.11.009 · doi-reference

Development of metabolic syndrome in people with cystic fibrosis one year after exposure to elexacaftor-tezacaftor-ivacaftor

10.1016/j.jcf.2024.09.022 · doi-reference

Dietary intake remains unchanged while nutritional status improves in children and adults with cystic fibrosis on elexacaftor/tezacaftor/ivacaftor

10.1016/j.clnu.2025.04.027 · doi-reference

Effect of elexacaftor-tezacaftor-ivacaftor on body weight and metabolic parameters in adults with cystic fibrosis

10.1016/j.jcf.2021.11.012 · doi-reference

Baseline characteristics and self-reported use of chronic daily therapies of the home reported outcomes with CFTR modulator therapy (HERO-2) cohort

10.1002/ppul.71190 · doi-reference

Impact of discontinuing both hypertonic saline and dornase alfa after elexacaftor-tezacaftor-ivacaftor in cystic fibrosis

10.1513/annalsats.202404-366oc · doi-reference

Discontinuation versus continuation of hypertonic saline or dornase alfa in modulator treated people with cystic fibrosis (SIMPLIFY): results from two parallel, multicentre, open-label, randomised, controlled, non-inferiority trials

10.1016/s2213-2600(22)00434-9 · doi-reference

Cystic Fibrosis Foundation position paper: redefining the cystic fibrosis care team

10.1016/j.jcf.2024.09.011 · doi-reference

Adapting the cystic fibrosis care model: perspectives from people with CF, caregivers, and members of CF care teams

10.1016/j.jcf.2024.09.002 · doi-reference

First real-world study of fetal therapy with CFTR modulators in cystic fibrosis: report from the MODUL-CF study

10.1016/j.jcf.2025.03.009 · doi-reference

Maternal and fetal outcomes following elexacaftor-tezacaftor-ivacaftor use during pregnancy and lactation

10.1016/j.jcf.2021.03.006 · doi-reference

Use of elexacaftor/tezacaftor/ivacaftor combination in pregnancy

10.1007/s00404-023-06962-5 · doi-reference

Perinatal dysfunction of innate immunity in cystic fibrosis

10.1126/scitranslmed.adk9145 · doi-reference

Disruption of the CFTR gene produces a model of cystic fibrosis in newborn pigs

10.1126/science.1163600 · doi-reference

In utero and postnatal VX-770 administration rescues multiorgan disease in a ferret model of cystic fibrosis

10.1126/scitranslmed.aau7531 · doi-reference

Clinical effectiveness of elexacaftor/tezacaftor/ivacaftor in 6 to 11-year-olds with cystic fibrosis

10.1513/annalsats.202501-103oc · doi-reference

Long-term impact of lumacaftor/ivacaftor treatment on cystic fibrosis disease progression in children 2 through 5 years of age homozygous for F508del-CFTR: a phase 2, open-label clinical trial

10.1513/annalsats.202402-201oc · doi-reference

Effects of lumacaftor/ivacaftor on cystic fibrosis disease progression in children 2 through 5 years of age homozygous for F508del-CFTR: a phase 2 placebo-controlled clinical trial

10.1513/annalsats.202208-684oc · doi-reference

Safety and efficacy of ivacaftor in infants aged 1 to less than 4 months with cystic fibrosis

10.1016/j.jcf.2024.03.012 · doi-reference

Phase 3 open-label clinical trial of elexacaftor/tezacaftor/ivacaftor in children aged 2-5 years with cystic fibrosis and at least one F508del allele

10.1164/rccm.202301-0084oc · doi-reference

Magnetic resonance imaging detects progression of lung disease and impact of newborn screening in preschool children with cystic fibrosis

10.1164/rccm.202102-0278oc · doi-reference

Cystic fibrosis

10.1038/s41572-024-00538-6 · doi-reference

Lung disease at diagnosis in infants with cystic fibrosis detected by newborn screening

10.1164/rccm.200901-0069oc · doi-reference

Personalized CFTR modulator therapy for G85E and N1303K homozygous patients with cystic fibrosis

10.3390/ijms241512365 · doi-reference

Progress of personalized medicine of cystic fibrosis in the times of efficient CFTR modulators

10.1186/s40348-025-00194-0 · doi-reference

Sweat chloride reflects CFTR function and correlates with clinical outcomes following CFTR modulator treatment

10.1016/j.jcf.2024.12.006 · doi-reference

WS19.01 stabilizers of CFTR NBD1 synergize with galicaftor (SION-2222) or SION-109 to enable full correction of ΔF508-CFTR

10.1016/j.jcf.2025.03.671 · doi-reference

Effect of elexacaftor/tezacaftor/ivacaftor on airway and systemic inflammation in cystic fibrosis

10.1136/thorax-2022-219943 · doi-reference

Mutations in the cystic fibrosis transmembrane regulator gene and in vivo transepithelial potentials

10.1164/rccm.200509-1377oc · doi-reference

CFTR Cl- channel function in native human colon correlates with the genotype and phenotype in cystic fibrosis

10.1053/j.gastro.2004.s07.006 · doi-reference

The future of cystic fibrosis treatment: from disease mechanisms to novel therapeutic approaches

10.1016/s0140-6736(23)01608-2 · doi-reference