Journal of Cystic Fibrosis

Article	Citations
Worldwide rates of diagnosis and effective treatment for cystic fibrosis	163
Effect of elexacaftor-tezacaftor-ivacaftor on body weight and metabolic parameters in adults with cystic fibrosis	85
Updated guidance on the management of children with cystic fibrosis transmembrane conductance regulator-related metabolic syndrome/cystic fibrosis screen positive, inconclusive diagnosis (CRMS/CFSPID)	78
The global impact of SARS-CoV-2 in 181 people with cystic fibrosis	75
Elexacaftor co-potentiates the activity of F508del and gating mutants of CFTR	65
Clinical characteristics of SARS-CoV-2 infection in children with cystic fibrosis: An international observational study	63
Maternal and fetal outcomes following elexacaftor-tezacaftor-ivacaftor use during pregnancy and lactation	62
Modeling pulmonary cystic fibrosis in a human lung airway-on-a-chip	60
Normal pancreatic function and false-negative CF newborn screen in a child born to a mother taking CFTR modulator therapy during pregnancy	58
Elexacaftor-Tezacaftor- Ivacaftor improves sinonasal outcomes in cystic fibrosis	58
Effect of highly effective modulator treatment on sinonasal symptoms in cystic fibrosis	57
Dose adjustments of Elexacaftor/Tezacaftor/Ivacaftor in response to mental health side effects in adults with cystic fibrosis	51
PROMISE: Working with the CF community to understand emerging clinical and research needs for those treated with highly effective CFTR modulator therapy	50
Targeting G542X CFTR nonsense alleles with ELX-02 restores CFTR function in human-derived intestinal organoids	50
The effect of elexacaftor/tezacaftor/ivacaftor (ETI) on glycemia in adults with cystic fibrosis	49
Magnetic resonance imaging detects improvements of pulmonary and paranasal sinus abnormalities in response to elexacaftor/tezacaftor/ivacaftor therapy in adults with cystic fibrosis	48
Patient and family perceptions of telehealth as part of the cystic fibrosis care model during COVID-19	46
Sustained effectiveness of elexacaftor-tezacaftor-ivacaftor in lung transplant candidates with cystic fibrosis	46
Elexacafator/tezacaftor/ivacaftor resolves subfertility in females with CF: A two center case series	46
Elexacaftor/tezacaftor/ivacaftor projected survival and long-term health outcomes in people with cystic fibrosis homozygous for F508del	45
Elexacaftor/tezacaftor/ivacaftor and gastrointestinal outcomes in cystic fibrosis: Report of promise-GI	44
A case report of CFTR modulator administration via carrier mother to treat meconium ileus in a F508del homozygous fetus	43
Real-world safety and effectiveness of elexacaftor/tezacaftor/ivacaftor in people with cystic fibrosis: Interim results of a long-term registry-based study	42
Lumacaftor/ivacaftor therapy fails to increase insulin secretion in F508del/F508del CF patients	42
Mental status changes during elexacaftor/tezacaftor / ivacaftor therapy	41

A phase 3, double-blind, parallel-group study to evaluate the efficacy and safety of tezacaftor in combination with ivacaftor in participants 6 through 11 years of age with cystic fibrosis homozygous	40
Incidence of SARS-CoV-2 in people with cystic fibrosis in Europe between February and June 2020	39
A survey of the prevalence, management and outcome of infants with an inconclusive diagnosis following newborn bloodspot screening for cystic fibrosis (CRMS/CFSPID) in six Italian centres	38
The impact of the COVID-19 pandemic on the emotional well-being and home treatment of Belgian patients with cystic fibrosis, including transplanted patients and paediatric patients	38
Projecting the impact of delayed access to elexacaftor/tezacaftor/ivacaftor for people with Cystic Fibrosis	37
Inhaled mRNA therapy for treatment of cystic fibrosis: Interim results of a randomized, double‐blind, placebo‐controlled phase 1/2 clinical study	37
Long term clinical effectiveness of ivacaftor in people with the G551D CFTR mutation	37
The fungal airway microbiome in cystic fibrosis and non-cystic fibrosis bronchiectasis	37
Telehealth use in cystic fibrosis during COVID-19: Association with race, ethnicity, and socioeconomic factors	35
Standards of care for CFTR variant-specific therapy (including modulators) for people with cystic fibrosis	35
Pregnancy in cystic fibrosis: Review of the literature and expert recommendations	34
Decreased survival in cystic fibrosis patients with a positive screen for depression	34
A restructuring of microbiome niche space is associated with Elexacaftor-Tezacaftor-Ivacaftor therapy in the cystic fibrosis lung	34
Antisense oligonucleotide-based drug development for Cystic Fibrosis patients carrying the 3849+10 kb C-to-T splicing mutation	34
ECFS standards of care on CFTR-related disorders: Updated diagnostic criteria	32
Obesity in Cystic fibrosis: prevalence, trends and associated factors data from the US cystic fibrosis foundation patient registry	32
Emerging preclinical modulators developed for F508del-CFTR have the potential to be effective for ORKAMBI resistant processing mutants	31
Changes in LCI in F508del/F508del patients treated with lumacaftor/ivacaftor: Results from the prospect study	31
Extended-culture and culture-independent molecular analysis of the airway microbiota in cystic fibrosis following CFTR modulation with ivacaftor	31
Disruption of the extracellular polymeric network of Pseudomonas aeruginosa biofilms by alginate lyase enhances pathogen eradication by antibiotics	30
Factors influencing the acquisition and eradication of early Pseudomonas aeruginosa infection in cystic fibrosis	30
Recommended shielding against COVID-19 impacts physical activity levels in adults with cystic fibrosis	29
Congenital bilateral cataracts in newborns exposed to elexacaftor-tezacaftor-ivacaftor in utero and while breast feeding	29
Use of elexacaftor/tezacaftor/ivacaftor among cystic fibrosis lung transplant recipients	28
Postprandial changes in gastrointestinal function and transit in cystic fibrosis assessed by Magnetic Resonance Imaging	28
Significant functional differences in differentiated Conditionally Reprogrammed (CRC)- and Feeder-free Dual SMAD inhibited-expanded human nasal epithelial cells	28
Overweight and obesity in adults with cystic fibrosis: An Italian multicenter cohort study	28
Relationship between airway dysbiosis, inflammation and lung function in adults with cystic fibrosis	28
ECFS standards of care on CFTR-related disorders: Diagnostic criteria of CFTR dysfunction	28
Targeted exhaled breath analysis for detection of Pseudomonas aeruginosa in cystic fibrosis patients	27
CFTR modulators increase risk of acute pancreatitis in pancreatic insufficient patients with cystic fibrosis	27
Longitudinal effects of elexacaftor/tezacaftor/ivacaftor on liver tests at a large single adult cystic fibrosis centre	27
Current prices versus minimum costs of production for CFTR modulators	26
Drug exposure to infants born to mothers taking Elexacaftor, Tezacaftor, and Ivacaftor	25
Baseline Cystic fibrosis disease severity has an adverse impact on pregnancy and infant outcomes, but does not impact disease progression	25
Lumacaftor-ivacaftor effects on cystic fibrosis-related liver involvement in adolescents with homozygous F508 del-CFTR	25
A comparison of clinic and home spirometry as longtudinal outcomes in cystic fibrosis	25
Abnormal glucose tolerance and lung function in children with cystic fibrosis. Comparing oral glucose tolerance test and continuous glucose monitoring	25
Clinical response to lumacaftor-ivacaftor in patients with cystic fibrosis according to baseline lung function	24
Multicenter prospective study showing a high gastrointestinal symptom burden in cystic fibrosis	24
Clinical and functional efficacy of elexacaftor/tezacaftor/ivacaftor in people with cystic fibrosis carrying the N1303K mutation	24
Patient and family experience of telehealth care delivery as part of the CF chronic care model early in the COVID-19 pandemic	24
Biliary disease and cholecystectomy after initiation of elexacaftor/ivacaftor/tezacaftor in adults with cystic fibrosis	24
Organoids for personalized treatment of Cystic Fibrosis: Professional perspectives on the ethics and governance of organoid biobanking	23
Functional Restoration of CFTR Nonsense Mutations in Intestinal Organoids	23
Short-term effect of elexacaftor-tezacaftor-ivacaftor on lung function and transplant planning in cystic fibrosis patients with advanced lung disease	23
Tezacaftor/ivacaftor in people with cystic fibrosis heterozygous for minimal function CFTR mutations	23
Disparities in first evaluation of infants with cystic fibrosis since implementation of newborn screening	23
Tobacco smoke exposure limits the therapeutic benefit of tezacaftor/ivacaftor in pediatric patients with cystic fibrosis	22
Cardiovascular complications in cystic fibrosis: A review of the literature	22

Impact of COVID-19 on mental health: Effects on screening, care delivery, and people with cystic fibrosis	22
Ursodeoxycholic acid and liver disease associated with cystic fibrosis: A multicenter cohort study	22
Suicide attempts in adolescents with cystic fibrosis on Elexacaftor/Tezacaftor/Ivacaftor therapy	22
Phages as immunomodulators and their promising use as anti-inflammatory agents in a cftr loss-of-function zebrafish model	22
Neutrophil dysfunction in cystic fibrosis	21
Review of Gastrointestinal Motility in Cystic Fibrosis	21
Tezacaftor/ivacaftor in people with cystic fibrosis who stopped lumacaftor/ivacaftor due to respiratory adverse events	21
Drug-induced liver injury from elexacaftor/ivacaftor/tezacaftor	21
Use of elexacaftor/tezacaftor/ivacaftor in liver transplant patients with cystic fibrosis	21
Changes in fecal microbiota with CFTR modulator therapy: A pilot study	21
Antisense oligonucleotide splicing modulation as a novel Cystic Fibrosis therapeutic approach for the W1282X nonsense mutation	21
Microbial interaction: Prevotella spp. reduce P. aeruginosa induced inflammation in cystic fibrosis bronchial epithelial cells	21
Remote monitoring in telehealth care delivery across the U.S. cystic fibrosis care network	21
SARS-CoV-2 seroprevalence in a Belgian cohort of patients with cystic fibrosis	20
Cystic fibrosis prevalence in the United States and participation in the Cystic Fibrosis Foundation Patient Registry in 2020	20
Females with cystic fibrosis have a larger decrease in sweat chloride in response to lumacaftor/ivacaftor compared to males	20
Effects of elexacaftor/tezacaftor/ivacaftor therapy in children with cystic fibrosis – a comprehensive assessment using lung clearance index, spirometry, and functional and structural lung MRI	20
EFFECT OF ELEXACAFTOR/TEZACAFTOR/IVACAFTOR ON ANNUAL RATE OF LUNG FUNCTION DECLINE IN PEOPLE WITH CYSTIC FIBROSIS	20
Ebselen attenuates tobramycin-induced ototoxicity in mice	20
Average rate of lung function decline in adults with cystic fibrosis in the United Kingdom: Data from the UK CF registry	20
Barriers and facilitators to implementing telehealth services during the COVID-19 pandemic: A qualitative analysis of interviews with cystic fibrosis care team members	20
Comparison of Cas9 and Cas12a CRISPR editing methods to correct the W1282X-CFTR mutation	19
Nutritional considerations for a new era: A CF foundation position paper	19
Psychological distress in adults with and without cystic fibrosis during the COVID-19 lockdown	19
Challenges in the use of highly effective modulator treatment for cystic fibrosis	19
New concepts in antimicrobial resistance in cystic fibrosis respiratory infections	18
Safety and efficacy of lenabasum in a phase 2 randomized, placebo-controlled trial in adults with cystic fibrosis	18
Hypervitaminosis A with fulminant secondary intracranial hypertension following personalized medicine-based Elexacaftor/Tezacaftor/Ivacaftor initiation in a preadolescent with cystic fibrosis	18
Non-respiratory health-related quality of life in people with cystic fibrosis receiving elexacaftor/tezacaftor/ivacaftor	18
Rethinking physical exercise training in the modern era of cystic fibrosis: A step towards optimising short-term efficacy and long-term engagement	18
CFTR modulator therapy for cystic fibrosis caused by the rare c.3700A>G mutation	18
Intestinal function and transit associate with gut microbiota dysbiosis in cystic fibrosis	18
Prospective longitudinal association between repeated multiple breath washout measurements and computed tomography scores in children with cystic fibrosis	17
Complete CFTR gene sequencing in 5,058 individuals with cystic fibrosis informs variant-specific treatment	17
Onset of systemic arterial hypertension after initiation of elexacaftor/tezacaftor/ivacaftor in adults with cystic fibrosis: A case series	17
Improved glucose tolerance after initiation of Elexacaftor / Tezacaftor / Ivacaftor in adults with cystic fibrosis	17
Olfactory dysfunction in cystic fibrosis: Impact of CFTR modulator therapy	17
Evaluating barriers to and promoters of telehealth during the COVID-19 pandemic at U.S. cystic fibrosis programs	17
Influence of pre-diabetic and pancreatic exocrine states on pulmonary and nutritional status in adults with Cystic Fibrosis	17
Factors associated with clinical progression to severe COVID-19 in people with cystic fibrosis: A global observational study	17
ACT with CF: A telehealth and in-person feasibility study to address anxiety and depressive symptoms among people with cystic fibrosis	16
Quality of home spirometry performance amongst adults with cystic fibrosis	16
Incidence and risk factors of cancer in individuals with cystic fibrosis in the UK; a case-control study	16
Telehealth and CFTR modulators: Accelerating innovative models of cystic fibrosis care	16
The feasibility of home monitoring of young people with cystic fibrosis: Results from CLIMB-CF	16
Elexacaftor-Tezacaftor-Ivacaftor improve Gastro-Oesophageal reflux and Sinonasal symptoms in advanced cystic fibrosis	16
Nutritional status and lung function in children with pancreatic-sufficient cystic fibrosis	16
Entry of cystic fibrosis transmembrane conductance potentiator ivacaftor into the developing brain and lung	16
The cystic fibrosis gut as a potential source of multidrug resistant pathogens	16
Designing the GALAXY study: Partnering with the cystic fibrosis community to optimize assessment of gastrointestinal symptoms	16
Lumacaftor/ivacaftor improves liver cholesterol metabolism but does not influence hypocholesterolemia in patients with cystic fibrosis	16
Total bacterial load, inflammation, and structural lung disease in paediatric cystic fibrosis	16
Clinical outcomes of a large cohort of individuals with the F508del/5T;TG12 CFTR genotype	16
In vivo demonstration of Pseudomonas aeruginosa biofilms as independent pharmacological microcompartments	16
Delayed glucose peak and elevated 1-hour glucose on the oral glucose tolerance test identify youth with cystic fibrosis with lower oral disposition index	16
Alpha-1 antitrypsin for cystic fibrosis complicated by severe cytokinemic COVID-19	16
Evidence of early increased sialylation of airway mucins and defective mucociliary clearance in CFTR-deficient piglets	15
The prevalence of aberrations in body composition in pediatric cystic fibrosis patients and relationships with pulmonary function, bone mineral density, and hospitalizations	15
Outcomes and endpoints reported in studies of pulmonary exacerbations in people with cystic fibrosis: A systematic review	15
Impact of cross-sensitivity error correction on representative nitrogen-based multiple breath washout data from clinical trials	15
Loss of ciliated cells and altered airway epithelial integrity in cystic fibrosis	15
Measuring the impact of CFTR modulation on sweat chloride in cystic fibrosis: Rationale and design of the CHEC-SC study	15
European survey of newborn bloodspot screening for CF: opportunity to address challenges and improve performance	15
VO2max as an exercise tolerance endpoint in people with cystic fibrosis: Lessons from a lumacaftor/ivacaftor trial	15
Airway profile of bioactive lipids predicts early progression of lung disease in cystic fibrosis	15
A case of self-limited drug induced liver injury under treatment with elexacaftor/tezacaftor/ivacaftor: When it is worth taking the risk	15
Antibiofilm and mucolytic action of nitric oxide delivered via gas or macromolecular donor using in vitro and ex vivo models	15
When CFSPID becomes CF	15
Pharmacokinetic interactions between ivacaftor and cytochrome P450 3A4 inhibitors in people with cystic fibrosis and healthy controls	14
Real-world use of ivacaftor in Canada: A retrospective analysis using the Canadian Cystic Fibrosis Registry	14
The implementation of an aminoglycoside induced ototoxicity algorithm for people with cystic fibrosis	14
Outcomes following lung re-transplantation in patients with cystic fibrosis	14
Effect of lumacaftor-ivacaftor on mucociliary clearance and clinical outcomes in cystic fibrosis: Results from the PROSPECT MCC sub-study	14
Magnetic resonance imaging of the gastrointestinal tract shows reduced small bowel motility and altered chyme in cystic fibrosis compared to controls	14
Defining key outcomes to evaluate performance of newborn screening programmes for cystic fibrosis	14
Minimal change in structural, functional and inflammatory markers of lung disease in newborn screened infants with cystic fibrosis at one year	14
C-reactive protein (CRP) as a biomarker of pulmonary exacerbation presentation and treatment response	14
Design and methods for understanding the state of cystic fibrosis care amid the COVID-19 pandemic	14
Peripheral lung effect of elexacaftor/tezacaftor/ivacaftor in adult cystic fibrosis	14
Antimicrobial resistance: Concerns of healthcare providers and people with CF	14

Utilization of electronic patient-reported outcome measures in cystic fibrosis research: Application to the GALAXY study	13
FDA-approved drug screening in patient-derived organoids demonstrates potential of drug repurposing for rare cystic fibrosis genotypes	13
Safety and efficacy of the cystic fibrosis transmembrane conductance regulator potentiator icenticaftor (QBW251)	13
Mucus and mucus flake composition and abundance reflect inflammatory and infection status in cystic fibrosis	13
Gamma-glutamyl transpeptidase-to-platelet ratio as a biomarker of liver disease and hepatic fibrosis severity in paediatric Cystic Fibrosis	13
Extensive CFTR sequencing through NGS in Brazilian individuals with cystic fibrosis: unravelling regional discrepancies in the country	13
Trends in nontuberculous mycobacteria infection in children and young people with cystic fibrosis	13
Vitamin D supplementation in patients with cystic fibrosis: A systematic review and meta-analysis	13
Forskolin induced swelling (FIS) assay in intestinal organoids to guide eligibility for compassionate use treatment in a CF patient with a rare genotype	13
Rescue by elexacaftor-tezacaftor-ivacaftor of the G1244E cystic fibrosis mutation's stability and gating defects are dependent on cell background	13
Financial impacts of the COVID-19 pandemic on cystic fibrosis care: lessons for the future	13
Modulation, microbiota and inflammation in the adult CF gut: A prospective study	13
Clinical effects of the three CFTR potentiator treatments curcumin, genistein and ivacaftor in patients with the CFTR-S1251N gating mutation	13
Standards of care guidance for sweat testing; phase two of the ECFS quality improvement programme	13
Impact of antibiotic eradication therapy of Pseudomonas aeruginosa on long term lung function in cystic fibrosis	13
Weight a minute: Exploring the effect on weight and body composition after the initiation of elexacaftor/tezacaftor/ivacaftor in adults with CF	13
Different management approaches and outcome for infants with an inconclusive diagnosis following newborn screening for cystic fibrosis (CRMS/CFSPID) and Pseudomonas aeruginosa isolation	13
Parenthood impacts short-term health outcomes in people with cystic fibrosis	13
Ivacaftor-elexacaftor-tezacaftor and tacrolimus combination in cystic fibrosis	13
Pseudomonas aeruginosa infection, but not mono or dual-combination CFTR modulator therapy affects circulating regulatory T cells in an adult population with cystic fibrosis	13
Emergence and impact of oprD mutations in Pseudomonas aeruginosa strains in cystic fibrosis	13
A four week trial of hypertonic saline in children with mild cystic fibrosis lung disease: Effect on mucociliary clearance and clinical outcomes	13
Exercise intolerance in cystic fibrosis-the role of CFTR modulator therapies	12
Drug-induced acne with elexacaftor/tezacaftor/ivacaftor in people with cystic fibrosis	12
Circulating CRP and calprotectin to diagnose CF pulmonary exacerbations	12
SLC26A9 SNP rs7512462 is not associated with lung disease severity or lung function response to ivacaftor in cystic fibrosis patients with G551D-CFTR	12
CFTR modulator therapy alters plasma sphingolipid profiles in people with cystic fibrosis	12
Research letter: The impact of elexacaftor/tezacaftor/ivacaftor on adherence to nebulized maintenance therapies in people with cystic fibrosis	12
The impact of liver disease on mortality in cystic fibrosis–A systematic review	12
Sex differences in treatment patterns in cystic fibrosis pulmonary exacerbations	12
A phase 3, randomized, double-blind, parallel-group study to evaluate tezacaftor/ivacaftor in people with cystic fibrosis heterozygous for F508del-CFTR and a gating mutation	12
Phenotypic characteristics of incident and chronic MRSA isolates in cystic fibrosis	12
Clinical characteristics of Pseudomonas and Aspergillus co-infected cystic fibrosis patients: A UK registry study	12
Clinician variability in the diagnosis and treatment of aspergillus fumigatus-related conditions in cystic fibrosis: An international survey	11
The lung microbiota in children with cystic fibrosis captured by induced sputum sampling	11
Club cell secretory protein and lung function in children with cystic fibrosis	11
High rates of anxiety detected in mothers of children with inconclusive cystic fibrosis screening results	11
HOMA indices as screening tests for cystic fibrosis-related diabetes	11
Standards for the care of people with cystic fibrosis (CF): A timely and accurate diagnosis	11
Ototoxicity in cystic fibrosis patients receiving intravenous tobramycin for acute pulmonary exacerbation	11
Lumacaftor/ivacaftor in people with cystic fibrosis with an A455E–CFTR mutation	11
Response to elexacaftor/tezacaftor/ivacaftor in intestinal organoids derived from people with cystic fibrosis	11
Contemporary cystic fibrosis incidence rates in Canada and the United States	11
Long-term amikacin liposome inhalation suspension in cystic fibrosis patients with chronic P. aeruginosa infection	11
Reclassifying inconclusive diagnosis after newborn screening for cystic fibrosis. Moving forward	11
Sensitive structural and functional measurements and 1-year pulmonary outcomes in pediatric cystic fibrosis	11
Effects of ivacaftor on systemic inflammation and the plasma proteome in people with CF and G551D	11
Prospective cohort study of ototoxicity in persons with cystic fibrosis following a single course of intravenous tobramycin	11
Standards for the care of people with cystic fibrosis (CF); recognising and addressing CF health issues	11
Penetrance is a critical parameter for assessing the disease liability of CFTR variants	11
The U UGA C sequence provides a favorable context to ELX-02 induced CFTR readthrough	11
Eradication of Nontuberculous Mycobacteria in People with Cystic Fibrosis Treated with Elexacaftor/Tezacaftor/Ivacaftor: A Multicenter Cohort Study	11
Advent of elexacaftor/tezacaftor/ivacaftor for cystic fibrosis treatment: What consequences on Aspergillus-related diseases? Preliminary insights	10
Diversity of cystic fibrosis chronic rhinosinusitis microbiota correlates with different pathogen dominance	10
The clinical impact of the Covid-19 pandemic first wave on patients with cystic fibrosis in New York	10
Citrullination of extracellular histone H3.1 reduces antibacterial activity and exacerbates its proteolytic degradation	10
The association between body composition, leptin levels and glucose dysregulation in youth with cystic fibrosis	10
Caution advised in the use of CFTR modulator treatment for individuals harboring specific CFTR variants	10
Changes in airway inflammation with pseudomonas eradication in early cystic fibrosis	10
Association of site of treatment with clinical outcomes following intravenous antimicrobial treatment of a pulmonary exacerbation	10
Efficacy and safety of inhaled dry-powder mannitol in adults with cystic fibrosis: An international, randomized controlled study	10
Utility of lung clearance index in CF: What we know, what we don't know and musings on how to bridge the gap	10
CFTR modulation with elexacaftor-tezacaftor-ivacaftor in people with cystic fibrosis assessed by the β-adrenergic sweat rate assay	10
Continuous glucose monitoring versus self-monitoring of blood glucose in the management of cystic fibrosis related diabetes: A systematic review and meta-analysis	10
A Phase 3, open-label, 96-week trial to study the safety, tolerability, and efficacy of tezacaftor/ivacaftor in children ≥ 6 years of age homozygous for F508del or heterozygous for F508del and a resid	10
The impact of elexacaftor/tezacaftor/ivacaftor on fat-soluble vitamin levels in people with cystic fibrosis	10
Temporal trends in healthcare resource use and associated costs of patients with cystic fibrosis	10
The impact of SARS-CoV-2 on the cystic fibrosis foundation therapeutics development network	10
Final results of the southwest German pilot study on cystic fibrosis newborn screening – Evaluation of an IRT/PAP protocol with IRT-dependent safety net	10
Inflammation biomarkers in sputum for clinical trials in cystic fibrosis: current understanding and gaps in knowledge	10
Body mass index and nutritional intake following Elexacaftor/Tezacaftor/Ivacaftor modulator therapy in adults with cystic fibrosis	10
Clinical evaluation of an evidence-based method based on food characteristics to adjust pancreatic enzyme supplements dose in cystic fibrosis	10
The risk of colorectal cancer in individuals with mutations of the cystic fibrosis transmembrane conductance regulator (CFTR) gene: An English population-based study	10
Rescue of chloride and bicarbonate transport by elexacaftor-ivacaftor-tezacaftor in organoid-derived CF intestinal and cholangiocyte monolayers	10
Long-term follow-up and liver outcomes in children with cystic fibrosis and nodular liver on ultrasound in a multi-center study	10
Defective BACH1/HO-1 regulatory circuits in cystic fibrosis bronchial epithelial cells	10
Treatment patterns in people with cystic fibrosis: have they changed since the introduction of ivacaftor?	10
The clinical and microbiological utility of inhaled aztreonam lysine for the treatment of acute pulmonary exacerbations of cystic fibrosis: An open-label randomised crossover study (AZTEC-CF)	10
Early acquisition and conversion of Pseudomonas aeruginosa in Hispanic youth with cystic fibrosis in the United States	10
Empire-CF study: A phase 2 clinical trial of leukotriene A4 hydrolase inhibitor acebilustat in adult subjects with cystic fibrosis	10
Withdrawal of dornase alfa increases ventilation inhomogeneity in children with cystic fibrosis	10
Pseudomonas aeruginosa antimicrobial susceptibility test (AST) results and pulmonary exacerbation treatment responses in cystic fibrosis	9
What it takes to implement regular longitudinal multiple breath washout tests in infants with cystic fibrosis	9
Men's sexual and reproductive health in cystic fibrosis in the era of highly effective modulator therapies–A qualitative study	9
Pilot RCT of a telehealth intervention to reduce symptoms of depression and anxiety in adults with cystic fibrosis	9
Optical Measurements of Sweat for in Vivo Quantification of CFTR Function in Individual Sweat Glands	9
Diagnosing constipation in patients with cystic fibrosis applying ESPGHAN criteria	9
Lung clearance index-triggered intervention in children with cystic fibrosis – A randomised pilot study	9
Newborn screening alone insufficient to improve pulmonary outcomes for cystic fibrosis	9
Impact of biofilm formation and azoles' susceptibility in Scedosporium/Lomentospora species using an in vitro model that mimics the cystic fibrosis patients' airway environment	9
Concerns regarding the safety of azithromycin in pregnancy - relevance for women with cystic fibrosis	9
In vitro modulator responsiveness of 655 CFTR variants found in people with cystic fibrosis	9
Risk factors for progression of structural lung disease in school-age children with cystic fibrosis	9
Gene, RNA, and ASO-based therapeutic approaches in Cystic Fibrosis	9
Patient-derived cell models for personalized medicine approaches in cystic fibrosis	9