Journal of Cystic Fibrosis

Article	Citations
Worldwide rates of diagnosis and effective treatment for cystic fibrosis	125
A multinational report to characterise SARS-CoV-2 infection in people with cystic fibrosis	104
Cystic Fibrosis Foundation consensus guidelines for the care of individuals with advanced cystic fibrosis lung disease	99
Effect of elexacaftor-tezacaftor-ivacaftor on body weight and metabolic parameters in adults with cystic fibrosis	73
The global impact of SARS-CoV-2 in 181 people with cystic fibrosis	72
Updated guidance on the management of children with cystic fibrosis transmembrane conductance regulator-related metabolic syndrome/cystic fibrosis screen positive, inconclusive diagnosis (CRMS/CFSPID)	69
Characterising burden of treatment in cystic fibrosis to identify priority areas for clinical trials	60
Clinical characteristics of SARS-CoV-2 infection in children with cystic fibrosis: An international observational study	59
Elexacaftor co-potentiates the activity of F508del and gating mutants of CFTR	57
Outcomes of pregnancy in women with cystic fibrosis (CF) taking CFTR modulators – an international survey	57
Changing epidemiology of the respiratory bacteriology of patients with cystic fibrosis–data from the European cystic fibrosis society patient registry	56
Functional rescue of c.3846G>A (W1282X) in patient-derived nasal cultures achieved by inhibition of nonsense mediated decay and protein modulators with complementary mechanisms of action	54
Maternal and fetal outcomes following elexacaftor-tezacaftor-ivacaftor use during pregnancy and lactation	52
Effect of highly effective modulator treatment on sinonasal symptoms in cystic fibrosis	50
Modeling pulmonary cystic fibrosis in a human lung airway-on-a-chip	49
Elexacaftor-Tezacaftor- Ivacaftor improves sinonasal outcomes in cystic fibrosis	49
Efficacy and safety of ataluren in patients with nonsense-mutation cystic fibrosis not receiving chronic inhaled aminoglycosides: The international, randomized, double-blind, placebo-controlled Atalur	48
Normal pancreatic function and false-negative CF newborn screen in a child born to a mother taking CFTR modulator therapy during pregnancy	48
Targeting G542X CFTR nonsense alleles with ELX-02 restores CFTR function in human-derived intestinal organoids	47
Effect of one-year lumacaftor–ivacaftor treatment on glucose tolerance abnormalities in cystic fibrosis patients	45
Patient and family perceptions of telehealth as part of the cystic fibrosis care model during COVID-19	42
Development of the gut microbiota in early life: The impact of cystic fibrosis and antibiotic treatment	42
Elexacafator/tezacaftor/ivacaftor resolves subfertility in females with CF: A two center case series	42
The effect of elexacaftor/tezacaftor/ivacaftor (ETI) on glycemia in adults with cystic fibrosis	42
PROMISE: Working with the CF community to understand emerging clinical and research needs for those treated with highly effective CFTR modulator therapy	41

Magnetic resonance imaging detects improvements of pulmonary and paranasal sinus abnormalities in response to elexacaftor/tezacaftor/ivacaftor therapy in adults with cystic fibrosis	41
Lumacaftor/ivacaftor therapy fails to increase insulin secretion in F508del/F508del CF patients	40
Sustained effectiveness of elexacaftor-tezacaftor-ivacaftor in lung transplant candidates with cystic fibrosis	38
Integrating the multiple breath washout test into international multicentre trials	38
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	37
Dose adjustments of Elexacaftor/Tezacaftor/Ivacaftor in response to mental health side effects in adults with cystic fibrosis	37
A case report of CFTR modulator administration via carrier mother to treat meconium ileus in a F508del homozygous fetus	36
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	36
Incidence of SARS-CoV-2 in people with cystic fibrosis in Europe between February and June 2020	36
Amplifiers co-translationally enhance CFTR biosynthesis via PCBP1-mediated regulation of CFTR mRNA	36
Forskolin-induced swelling of intestinal organoids correlates with disease severity in adults with cystic fibrosis and homozygous F508del mutations	35
Elexacaftor/tezacaftor/ivacaftor and gastrointestinal outcomes in cystic fibrosis: Report of promise-GI	35
The impact of CFTR modulator therapies on CF airway microbiology	34
Mental status changes during elexacaftor/tezacaftor / ivacaftor therapy	34
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	33
Projecting the impact of delayed access to elexacaftor/tezacaftor/ivacaftor for people with Cystic Fibrosis	33
Elexacaftor/tezacaftor/ivacaftor projected survival and long-term health outcomes in people with cystic fibrosis homozygous for F508del	33
The fungal airway microbiome in cystic fibrosis and non-cystic fibrosis bronchiectasis	32
Long term clinical effectiveness of ivacaftor in people with the G551D CFTR mutation	32
Telehealth use in cystic fibrosis during COVID-19: Association with race, ethnicity, and socioeconomic factors	31
Antisense oligonucleotide-based drug development for Cystic Fibrosis patients carrying the 3849+10 kb C-to-T splicing mutation	31
A restructuring of microbiome niche space is associated with Elexacaftor-Tezacaftor-Ivacaftor therapy in the cystic fibrosis lung	31
Testicular pain following initiation of elexacaftor/tezacaftor/ivacaftor in males with cystic fibrosis	30
Extended-culture and culture-independent molecular analysis of the airway microbiota in cystic fibrosis following CFTR modulation with ivacaftor	30
Decreased survival in cystic fibrosis patients with a positive screen for depression	30
Emerging preclinical modulators developed for F508del-CFTR have the potential to be effective for ORKAMBI resistant processing mutants	30
Real-world safety and effectiveness of elexacaftor/tezacaftor/ivacaftor in people with cystic fibrosis: Interim results of a long-term registry-based study	29
Changes in LCI in F508del/F508del patients treated with lumacaftor/ivacaftor: Results from the prospect study	29
Recommended shielding against COVID-19 impacts physical activity levels in adults with cystic fibrosis	29
Asymptomatic case of Covid-19 in an infant with cystic fibrosis	27
Postprandial changes in gastrointestinal function and transit in cystic fibrosis assessed by Magnetic Resonance Imaging	27
Inhaled mRNA therapy for treatment of cystic fibrosis: Interim results of a randomized, double‐blind, placebo‐controlled phase 1/2 clinical study	27
Factors influencing the acquisition and eradication of early Pseudomonas aeruginosa infection in cystic fibrosis	27
Relationship between airway dysbiosis, inflammation and lung function in adults with cystic fibrosis	27
Pregnancy in cystic fibrosis: Review of the literature and expert recommendations	27
Disruption of the extracellular polymeric network of Pseudomonas aeruginosa biofilms by alginate lyase enhances pathogen eradication by antibiotics	26
Tobacco smoke exposure and socioeconomic factors are independent predictors of pulmonary decline in pediatric cystic fibrosis	26
Overweight and obesity in adults with cystic fibrosis: An Italian multicenter cohort study	26
Answering the call to address cystic fibrosis treatment burden in the era of highly effective CFTR modulator therapy	26
Significant functional differences in differentiated Conditionally Reprogrammed (CRC)- and Feeder-free Dual SMAD inhibited-expanded human nasal epithelial cells	25
Supersonic shear-wave elastography and APRI for the detection and staging of liver disease in pediatric cystic fibrosis	25
Obesity in Cystic fibrosis: prevalence, trends and associated factors data from the US cystic fibrosis foundation patient registry	25
Glucose abnormalities detected by continuous glucose monitoring are common in young children with Cystic Fibrosis	24
Functional Restoration of CFTR Nonsense Mutations in Intestinal Organoids	24
Use of elexacaftor/tezacaftor/ivacaftor among cystic fibrosis lung transplant recipients	24
Use of a mobile application for self-management of pancreatic enzyme replacement therapy is associated with improved gastro-intestinal related quality of life in children with Cystic Fibrosis	24
Biliary disease and cholecystectomy after initiation of elexacaftor/ivacaftor/tezacaftor in adults with cystic fibrosis	23
CFTR modulators increase risk of acute pancreatitis in pancreatic insufficient patients with cystic fibrosis	23
Abnormal glucose tolerance and lung function in children with cystic fibrosis. Comparing oral glucose tolerance test and continuous glucose monitoring	23
Building global development strategies for cf therapeutics during a transitional cftr modulator era	23

Tezacaftor/ivacaftor in people with cystic fibrosis heterozygous for minimal function CFTR mutations	22
Clinical response to lumacaftor-ivacaftor in patients with cystic fibrosis according to baseline lung function	22
Antimicrobial resistance in cystic fibrosis: A Delphi approach to defining best practices	22
Current prices versus minimum costs of production for CFTR modulators	22
A comparison of clinic and home spirometry as longtudinal outcomes in cystic fibrosis	22
Drug exposure to infants born to mothers taking Elexacaftor, Tezacaftor, and Ivacaftor	22
Short-term effect of elexacaftor-tezacaftor-ivacaftor on lung function and transplant planning in cystic fibrosis patients with advanced lung disease	22
Tezacaftor/ivacaftor in people with cystic fibrosis who stopped lumacaftor/ivacaftor due to respiratory adverse events	21
Organoids for personalized treatment of Cystic Fibrosis: Professional perspectives on the ethics and governance of organoid biobanking	21
Lumacaftor-ivacaftor effects on cystic fibrosis-related liver involvement in adolescents with homozygous F508 del-CFTR	21
Impact of COVID-19 on mental health: Effects on screening, care delivery, and people with cystic fibrosis	21
Cardiovascular complications in cystic fibrosis: A review of the literature	21
Baseline Cystic fibrosis disease severity has an adverse impact on pregnancy and infant outcomes, but does not impact disease progression	21
Standards of care for CFTR variant-specific therapy (including modulators) for people with cystic fibrosis	21
SARS-CoV-2 seroprevalence in a Belgian cohort of patients with cystic fibrosis	20
Remote monitoring in telehealth care delivery across the U.S. cystic fibrosis care network	20
Tobacco smoke exposure limits the therapeutic benefit of tezacaftor/ivacaftor in pediatric patients with cystic fibrosis	20
Phages as immunomodulators and their promising use as anti-inflammatory agents in a cftr loss-of-function zebrafish model	20
ECFS standards of care on CFTR-related disorders: Diagnostic criteria of CFTR dysfunction	20
Antisense oligonucleotide splicing modulation as a novel Cystic Fibrosis therapeutic approach for the W1282X nonsense mutation	19
Challenges in the use of highly effective modulator treatment for cystic fibrosis	19
Patient and family experience of telehealth care delivery as part of the CF chronic care model early in the COVID-19 pandemic	19
Psychological distress in adults with and without cystic fibrosis during the COVID-19 lockdown	19
Longitudinal effects of elexacaftor/tezacaftor/ivacaftor on liver tests at a large single adult cystic fibrosis centre	19
Prevalence of unmet palliative care needs in adults with cystic fibrosis	19
Ursodeoxycholic acid and liver disease associated with cystic fibrosis: A multicenter cohort study	19
Finding the relevance of antimicrobial stewardship for cystic fibrosis	18
ECFS standards of care on CFTR-related disorders: Updated diagnostic criteria	18
Targeted exhaled breath analysis for detection of Pseudomonas aeruginosa in cystic fibrosis patients	18
Average rate of lung function decline in adults with cystic fibrosis in the United Kingdom: Data from the UK CF registry	18
Comparison of Cas9 and Cas12a CRISPR editing methods to correct the W1282X-CFTR mutation	18
Changes in fecal microbiota with CFTR modulator therapy: A pilot study	18
Multicenter prospective study showing a high gastrointestinal symptom burden in cystic fibrosis	18
Review of Gastrointestinal Motility in Cystic Fibrosis	18
Disparities in first evaluation of infants with cystic fibrosis since implementation of newborn screening	18
Clinical effect of lumacaftor/ivacaftor in F508del homozygous CF patients with FEV1 ≥ 90% predicted at baseline	17
Use of elexacaftor/tezacaftor/ivacaftor in liver transplant patients with cystic fibrosis	17
Safety and efficacy of lenabasum in a phase 2 randomized, placebo-controlled trial in adults with cystic fibrosis	17
Congenital bilateral cataracts in newborns exposed to elexacaftor-tezacaftor-ivacaftor in utero and while breast feeding	17
Health-related quality-of-life in children with cystic fibrosis aged 5-years and associations with health outcomes	17
Ebselen attenuates tobramycin-induced ototoxicity in mice	17
CFTR modulator therapy for cystic fibrosis caused by the rare c.3700A>G mutation	17
Can exercise replace airway clearance techniques in cystic fibrosis? A survey of patients and healthcare professionals	17
Prospective longitudinal association between repeated multiple breath washout measurements and computed tomography scores in children with cystic fibrosis	17
Suicide attempts in adolescents with cystic fibrosis on Elexacaftor/Tezacaftor/Ivacaftor therapy	16
Drug-induced liver injury from elexacaftor/ivacaftor/tezacaftor	16
In vivo demonstration of Pseudomonas aeruginosa biofilms as independent pharmacological microcompartments	16
Microbial interaction: Prevotella spp. reduce P. aeruginosa induced inflammation in cystic fibrosis bronchial epithelial cells	16
Females with cystic fibrosis have a larger decrease in sweat chloride in response to lumacaftor/ivacaftor compared to males	16
Formative evaluation of a dashboard to support coproduction of healthcare services in cystic fibrosis	16
Alpha-1 antitrypsin for cystic fibrosis complicated by severe cytokinemic COVID-19	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
Clinical and functional efficacy of elexacaftor/tezacaftor/ivacaftor in people with cystic fibrosis carrying the N1303K mutation	16
Neutrophil dysfunction in cystic fibrosis	16
Evaluating barriers to and promoters of telehealth during the COVID-19 pandemic at U.S. cystic fibrosis programs	16
Influence of pre-diabetic and pancreatic exocrine states on pulmonary and nutritional status in adults with Cystic Fibrosis	16
A longitudinal assessment of non-invasive biomarkers to diagnose and predict cystic fibrosis-associated liver disease	16
The prevalence of aberrations in body composition in pediatric cystic fibrosis patients and relationships with pulmonary function, bone mineral density, and hospitalizations	15
Predictors of pulmonary exacerbation treatment in cystic fibrosis	15
Barriers and facilitators to implementing telehealth services during the COVID-19 pandemic: A qualitative analysis of interviews with cystic fibrosis care team members	15
Factors associated with clinical progression to severe COVID-19 in people with cystic fibrosis: A global observational study	15
Insights into the variability of nasal potential difference, a biomarker of CFTR activity	15
Rethinking physical exercise training in the modern era of cystic fibrosis: A step towards optimising short-term efficacy and long-term engagement	15
Intestinal function and transit associate with gut microbiota dysbiosis in cystic fibrosis	15
Olfactory dysfunction in cystic fibrosis: Impact of CFTR modulator therapy	15
Hypervitaminosis A with fulminant secondary intracranial hypertension following personalized medicine-based Elexacaftor/Tezacaftor/Ivacaftor initiation in a preadolescent with cystic fibrosis	15
Variable cellular ivacaftor concentrations in people with cystic fibrosis on modulator therapy	15
Non-respiratory health-related quality of life in people with cystic fibrosis receiving elexacaftor/tezacaftor/ivacaftor	15
Total bacterial load, inflammation, and structural lung disease in paediatric cystic fibrosis	15
COVID-19 in lung-transplanted and cystic fibrosis patients: Be careful	15
When CFSPID becomes CF	15
VO2max as an exercise tolerance endpoint in people with cystic fibrosis: Lessons from a lumacaftor/ivacaftor trial	14
Loss of ciliated cells and altered airway epithelial integrity in cystic fibrosis	14
Designing the GALAXY study: Partnering with the cystic fibrosis community to optimize assessment of gastrointestinal symptoms	14
Real-world use of ivacaftor in Canada: A retrospective analysis using the Canadian Cystic Fibrosis Registry	14
Antibiofilm and mucolytic action of nitric oxide delivered via gas or macromolecular donor using in vitro and ex vivo models	14
Quality of home spirometry performance amongst adults with cystic fibrosis	14
Urine lipoarabinomannan as a marker for low-risk of NTM infection in the CF airway	14
Design and methods for understanding the state of cystic fibrosis care amid the COVID-19 pandemic	14
Lumacaftor/ivacaftor improves liver cholesterol metabolism but does not influence hypocholesterolemia in patients with cystic fibrosis	14

ACT with CF: A telehealth and in-person feasibility study to address anxiety and depressive symptoms among people with cystic fibrosis	14
Impact of cross-sensitivity error correction on representative nitrogen-based multiple breath washout data from clinical trials	14
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	14
Effect of lumacaftor-ivacaftor on mucociliary clearance and clinical outcomes in cystic fibrosis: Results from the PROSPECT MCC sub-study	14
Lumacaftor/ivacaftor reduces exacerbations in adults homozygous for Phe508del mutation with severe lung disease	14
Outcomes and endpoints reported in studies of pulmonary exacerbations in people with cystic fibrosis: A systematic review	14
Clinical outcomes of a large cohort of individuals with the F508del/5T;TG12 CFTR genotype	13
The cystic fibrosis gut as a potential source of multidrug resistant pathogens	13
Entry of cystic fibrosis transmembrane conductance potentiator ivacaftor into the developing brain and lung	13
Minimal change in structural, functional and inflammatory markers of lung disease in newborn screened infants with cystic fibrosis at one year	13
The impact of chest computed tomography and chest radiography on clinical management of cystic fibrosis lung disease	13
Antimicrobial resistance: Concerns of healthcare providers and people with CF	13
A four week trial of hypertonic saline in children with mild cystic fibrosis lung disease: Effect on mucociliary clearance and clinical outcomes	13
Nutritional considerations for a new era: A CF foundation position paper	13
Financial impacts of the COVID-19 pandemic on cystic fibrosis care: lessons for the future	13
Measuring the impact of CFTR modulation on sweat chloride in cystic fibrosis: Rationale and design of the CHEC-SC study	13
Cystic fibrosis in black African children in South Africa: a case control study	13
Occurrence, outcomes and predictors of portal hypertension in cystic fibrosis: A longitudinal prospective birth cohort study	13
In vitro 3D culture lung model from expanded primary cystic fibrosis human airway cells	13
Pharmacokinetic interactions between ivacaftor and cytochrome P450 3A4 inhibitors in people with cystic fibrosis and healthy controls	13
Safety and efficacy of the cystic fibrosis transmembrane conductance regulator potentiator icenticaftor (QBW251)	13
EFFECT OF ELEXACAFTOR/TEZACAFTOR/IVACAFTOR ON ANNUAL RATE OF LUNG FUNCTION DECLINE IN PEOPLE WITH CYSTIC FIBROSIS	13
Airway profile of bioactive lipids predicts early progression of lung disease in cystic fibrosis	13
Cystic fibrosis drug trial design in the era of CFTR modulators associated with substantial clinical benefit: stakeholders’ consensus view	13
Complete CFTR gene sequencing in 5,058 individuals with cystic fibrosis informs variant-specific treatment	13
Elexacaftor-Tezacaftor-Ivacaftor improve Gastro-Oesophageal reflux and Sinonasal symptoms in advanced cystic fibrosis	12
Peripheral lung effect of elexacaftor/tezacaftor/ivacaftor in adult cystic fibrosis	12
Defining key outcomes to evaluate performance of newborn screening programmes for cystic fibrosis	12
Outcomes following lung re-transplantation in patients with cystic fibrosis	12
Fibrocyte accumulation in the lungs of cystic fibrosis patients	12
Intraoperative extracorporeal membrane oxygenation for lung transplantation in cystic fibrosis patients: Predictors and impact on outcome	12
Screening practices for nontuberculous mycobacteria at US cystic fibrosis centers	12
Pseudomonas aeruginosa infection, but not mono or dual-combination CFTR modulator therapy affects circulating regulatory T cells in an adult population with cystic fibrosis	12
Trends in nontuberculous mycobacteria infection in children and young people with cystic fibrosis	12
CFTR modulator therapy alters plasma sphingolipid profiles in people with cystic fibrosis	12
Incidence and risk factors of cancer in individuals with cystic fibrosis in the UK; a case-control study	12
Improved glucose tolerance after initiation of Elexacaftor / Tezacaftor / Ivacaftor in adults with cystic fibrosis	12
Nutritional status and lung function in children with pancreatic-sufficient cystic fibrosis	12
The implementation of an aminoglycoside induced ototoxicity algorithm for people with cystic fibrosis	12
Vitamin D supplementation in patients with cystic fibrosis: A systematic review and meta-analysis	12
Onset of systemic arterial hypertension after initiation of elexacaftor/tezacaftor/ivacaftor in adults with cystic fibrosis: A case series	12
Clinical effects of the three CFTR potentiator treatments curcumin, genistein and ivacaftor in patients with the CFTR-S1251N gating mutation	12
Utilization of electronic patient-reported outcome measures in cystic fibrosis research: Application to the GALAXY study	11
Adherence to lumacaftor-ivacaftor therapy in patients with cystic fibrosis in France	11
Forskolin induced swelling (FIS) assay in intestinal organoids to guide eligibility for compassionate use treatment in a CF patient with a rare genotype	11
The feasibility of home monitoring of young people with cystic fibrosis: Results from CLIMB-CF	11
Modulation, microbiota and inflammation in the adult CF gut: A prospective study	11
Exercise intolerance in cystic fibrosis-the role of CFTR modulator therapies	11
Ototoxicity in cystic fibrosis patients receiving intravenous tobramycin for acute pulmonary exacerbation	11
New concepts in antimicrobial resistance in cystic fibrosis respiratory infections	11
Phenotypic characteristics of incident and chronic MRSA isolates in cystic fibrosis	11
Ivacaftor-elexacaftor-tezacaftor and tacrolimus combination in cystic fibrosis	11
Magnetic resonance imaging of the gastrointestinal tract shows reduced small bowel motility and altered chyme in cystic fibrosis compared to controls	11
Evidence of early increased sialylation of airway mucins and defective mucociliary clearance in CFTR-deficient piglets	11
A case of self-limited drug induced liver injury under treatment with elexacaftor/tezacaftor/ivacaftor: When it is worth taking the risk	11
FDA-approved drug screening in patient-derived organoids demonstrates potential of drug repurposing for rare cystic fibrosis genotypes	11
Circulating CRP and calprotectin to diagnose CF pulmonary exacerbations	11
Long-term amikacin liposome inhalation suspension in cystic fibrosis patients with chronic P. aeruginosa infection	11
SLC26A9 SNP rs7512462 is not associated with lung disease severity or lung function response to ivacaftor in cystic fibrosis patients with G551D-CFTR	11
Longitudinal changes in lung function following initiation of lumacaftor/ivacaftor combination	10
Reclassifying inconclusive diagnosis after newborn screening for cystic fibrosis. Moving forward	10
Structured surveillance of Achromobacter, Pandoraea and Ralstonia species from patients in England with cystic fibrosis	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
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	10
Lumacaftor/ivacaftor in people with cystic fibrosis with an A455E–CFTR mutation	10
Response to elexacaftor/tezacaftor/ivacaftor in intestinal organoids derived from people with cystic fibrosis	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
Penetrance is a critical parameter for assessing the disease liability of CFTR variants	10
CFTR modulation with elexacaftor-tezacaftor-ivacaftor in people with cystic fibrosis assessed by the β-adrenergic sweat rate assay	10
Standards of care guidance for sweat testing; phase two of the ECFS quality improvement programme	10
C-reactive protein (CRP) as a biomarker of pulmonary exacerbation presentation and treatment response	10
Rescue by elexacaftor-tezacaftor-ivacaftor of the G1244E cystic fibrosis mutation's stability and gating defects are dependent on cell background	10
Weight a minute: Exploring the effect on weight and body composition after the initiation of elexacaftor/tezacaftor/ivacaftor in adults with CF	10
The impact of liver disease on mortality in cystic fibrosis–A systematic review	10
The impact of SARS-CoV-2 on the cystic fibrosis foundation therapeutics development network	10
HOMA indices as screening tests for cystic fibrosis-related diabetes	10
Abnormal glucose tolerance and the 50-gram glucose challenge test in Cystic fibrosis	10
European survey of newborn bloodspot screening for CF: opportunity to address challenges and improve performance	10
Sex differences in treatment patterns in cystic fibrosis pulmonary exacerbations	10
Clinician variability in the diagnosis and treatment of aspergillus fumigatus-related conditions in cystic fibrosis: An international survey	10
Temporal trends in healthcare resource use and associated costs of patients with cystic fibrosis	10
The association between body composition, leptin levels and glucose dysregulation in youth with cystic fibrosis	10
Different management approaches and outcome for infants with an inconclusive diagnosis following newborn screening for cystic fibrosis (CRMS/CFSPID) and Pseudomonas aeruginosa isolation	10
Association of site of treatment with clinical outcomes following intravenous antimicrobial treatment of a pulmonary exacerbation	10
Clinical characteristics of Pseudomonas and Aspergillus co-infected cystic fibrosis patients: A UK registry study	10
Anti-inflammatory effects of lenabasum, a cannabinoid receptor type 2 agonist, on macrophages from cystic fibrosis	9
Optical Measurements of Sweat for in Vivo Quantification of CFTR Function in Individual Sweat Glands	9
Lung clearance index-triggered intervention in children with cystic fibrosis – A randomised pilot study	9
No detection of SARS-CoV-2 in cystic fibrosis patients at the Regional (Lazio) Reference Center for CF in Italy	9
Accumulation and persistence of ivacaftor in airway epithelia with prolonged treatment	9
Diagnosing constipation in patients with cystic fibrosis applying ESPGHAN criteria	9
Impact of antibiotic eradication therapy of Pseudomonas aeruginosa on long term lung function in cystic fibrosis	9
Concerns regarding the safety of azithromycin in pregnancy - relevance for women with cystic fibrosis	9
Emergence and impact of oprD mutations in Pseudomonas aeruginosa strains in cystic fibrosis	9
Evaluation of the exercise intensity generated by active video gaming in patients with cystic fibrosis and healthy individuals	9