| 1 | Clinical Effectiveness of Elexacaftor/Tezacaftor/Ivacaftor in People with Cystic Fibrosis: A Clinical Trial | American Journal of Respiratory and Critical Care Medicine | 2022 | 278 |
| 2 | Variant Cystic Fibrosis Phenotypes in the Absence ofCFTRMutations | New England Journal of Medicine | 2002 | 110 |
| 3 | Use of FEV1 in cystic fibrosis epidemiologic studies and clinical trials: A statistical perspective for the clinical researcher | Journal of Cystic Fibrosis | 2017 | 99 |
| 4 | Effects of Diagnosis by Newborn Screening for Cystic Fibrosis on Weight and Length in the First Year of Life | JAMA Pediatrics | 2017 | 87 |
| 5 | Elexacaftor/tezacaftor/ivacaftor and gastrointestinal outcomes in cystic fibrosis: Report of promise-GI | Journal of Cystic Fibrosis | 2023 | 79 |
| 6 | Heterogeneity in Survival in Adult Patients With Cystic Fibrosis With FEV1 < 30% of Predicted in the United States | Chest | 2017 | 76 |
| 7 | Standardized Treatment of Pulmonary Exacerbations (STOP) study: Physician treatment practices and outcomes for individuals with cystic fibrosis with pulmonary Exacerbations | Journal of Cystic Fibrosis | 2017 | 75 |
| 8 | Risk factors for age at initial Pseudomonas acquisition in the cystic fibrosis epic observational cohort | Journal of Cystic Fibrosis | 2012 | 71 |
| 9 | Standardized Treatment of Pulmonary Exacerbations (STOP) study: Observations at the initiation of intravenous antibiotics for cystic fibrosis pulmonary exacerbations | Journal of Cystic Fibrosis | 2017 | 67 |
| 10 | Improving chronic care delivery and outcomes: the impact of the cystic fibrosis Care Center Network | BMJ Quality and Safety | 2014 | 61 |
| 11 | Short-term and long-term response to pulmonary exacerbation treatment in cystic fibrosis | Thorax | 2016 | 56 |
| 12 | Clinical outcomes after initial pseudomonas acquisition in cystic fibrosis | Pediatric Pulmonology | 2015 | 55 |
| 13 | Effect of Treatment of Cystic Fibrosis Pulmonary Exacerbations on Systemic Inflammation | Annals of the American Thoracic Society | 2015 | 53 |
| 14 | Lumacaftor/ivacaftor therapy fails to increase insulin secretion in F508del/F508del CF patients | Journal of Cystic Fibrosis | 2021 | 52 |
| 15 | Baseline Characteristics and Factors Associated With Nutritional and Pulmonary Status at Enrollment in the Cystic Fibrosis EPIC Observational Cohort | Pediatric Pulmonology | 2010 | 50 |
| 16 | Use of Phage Display To Identify Potential Pseudomonas aeruginosa Gene Products Relevant to Early Cystic Fibrosis Airway Infections | Infection and Immunity | 2005 | 49 |
| 17 | Effects of an Antioxidant-enriched Multivitamin in Cystic Fibrosis. A Randomized, Controlled, Multicenter Clinical Trial | American Journal of Respiratory and Critical Care Medicine | 2018 | 38 |
| 18 | Multicenter prospective study showing a high gastrointestinal symptom burden in cystic fibrosis | Journal of Cystic Fibrosis | 2023 | 35 |
| 19 | Clinical Effectiveness of Lumacaftor/Ivacaftor in Patients with Cystic Fibrosis Homozygous for F508del-CFTR. A Clinical Trial | Annals of the American Thoracic Society | 2021 | 34 |
| 20 | Changes in LCI in F508del/F508del patients treated with lumacaftor/ivacaftor: Results from the prospect study | Journal of Cystic Fibrosis | 2020 | 29 |
| 21 | Advancing clinical development pathways for new CFTR modulators in cystic fibrosis | Thorax | 2016 | 28 |
| 22 | Comparison of 7-day and repeated 24-hour recall of symptoms of cystic fibrosis | Journal of Cystic Fibrosis | 2010 | 23 |
| 23 | Inhaled hypertonic saline in infants and toddlers with cystic fibrosis: short‐term tolerability, adherence, and safety | Pediatric Pulmonology | 2011 | 22 |
| 24 | Cystic Fibrosis: The Dawn of a New Therapeutic Era | American Journal of Respiratory and Critical Care Medicine | 2017 | 20 |
| 25 | Evaluation of airway and circulating inflammatory biomarkers for cystic fibrosis drug development | Journal of Cystic Fibrosis | 2021 | 20 |
| 26 | Recurrent and Destructive Nasal Polyposis in 2 Siblings: A Possible Case of Woakes' Syndrome | Otolaryngology - Head and Neck Surgery | 2004 | 19 |
| 27 | Utilization of electronic patient-reported outcome measures in cystic fibrosis research: Application to the GALAXY study | Journal of Cystic Fibrosis | 2021 | 16 |
| 28 | Outcomes and Treatment of Chronic Methicillin-ResistantStaphylococcus aureusDiffers by Staphylococcal Cassette Chromosomemec(SCCmec) Type in Children With Cystic Fibrosis | Journal of the Pediatric Infectious Diseases Society | 2015 | 15 |
| 29 | Update in Cystic Fibrosis 2018 | American Journal of Respiratory and Critical Care Medicine | 2019 | 13 |
| 30 | Effects of ivacaftor on systemic inflammation and the plasma proteome in people with CF and G551D | Journal of Cystic Fibrosis | 2022 | 13 |
| 31 | Testing the effects of combining azithromycin with inhaled tobramycin forP. aeruginosain cystic fibrosis: a randomised, controlled clinical trial | Thorax | 2022 | 12 |
| 32 | Common clinical features of CF (respiratory disease and exocrine pancreatic insufficiency) | Presse Medicale | 2017 | 11 |
| 33 | Pulmonary findings in infants with cystic fibrosis during the first year of life: Results from the Baby Observational and Nutrition Study (BONUS) cohort study | Pediatric Pulmonology | 2019 | 11 |
| 34 | Urinary metabolomics reveals unique metabolic signatures in infants with cystic fibrosis | Journal of Cystic Fibrosis | 2019 | 11 |
| 35 | Utilizing centralized biorepository samples for biomarkers of cystic fibrosis lung disease severity | Journal of Cystic Fibrosis | 2020 | 11 |
| 36 | Infants with cystic fibrosis have altered fecal functional capacities with potential clinical and metabolic consequences | BMC Microbiology | 2021 | 11 |
| 37 | Built environment factors predictive of early rapid lung function decline in cystic fibrosis | Pediatric Pulmonology | 2023 | 11 |
| 38 | Alterations in the fecal microbiota in patients with advanced cystic fibrosis liver disease after 6 months of elexacaftor/tezacaftor/ivacaftor | Journal of Cystic Fibrosis | 2024 | 10 |
| 39 | The impact of SARS-CoV-2 on the cystic fibrosis foundation therapeutics development network | Journal of Cystic Fibrosis | 2021 | 7 |
| 40 | Upper airway microbiota development in infants with cystic fibrosis diagnosed by newborn screen | Journal of Cystic Fibrosis | 2023 | 5 |
| 41 | Rates of adverse and serious adverse events in children with cystic fibrosis | Journal of Cystic Fibrosis | 2021 | 4 |
| 42 | Prospective evaluation of nontuberculous mycobacteria disease in cystic fibrosis: The design of the PREDICT study | Journal of Cystic Fibrosis | 2024 | 4 |
| 43 | Estimating minimal clinically important difference (MCID) for gastrointestinal symptoms in cystic fibrosis | Journal of Cystic Fibrosis | 2024 | 3 |
| 44 | Clinical Effectiveness of Elexacaftor/Tezacaftor/Ivacaftor in 6- to 11-Year-Olds with Cystic Fibrosis: An Observational Study | Annals of the American Thoracic Society | 0 | 3 |
| 45 | Pseudomonas infections persisting after CFTR modulators are widespread throughout the lungs and drive lung inflammation | Cell Host and Microbe | 2025 | 3 |
| 46 | Role of hyperglycemia in cystic fibrosis pulmonary exacerbations | Journal of Cystic Fibrosis | 2023 | 2 |
| 47 | Clinical outcomes at 9–10 years of age in children born with cystic fibrosis transmembrane conductance regulator related metabolic syndrome | Pediatric Pulmonology | 2024 | 2 |
| 48 | Assessment of patients' baseline cystic fibrosis knowledge levels following translation and adaptation of the CF R.I.S.E translation program into Turkish | Pediatric Pulmonology | 2024 | 2 |
| 49 | Potential for Therapeutic Benefit among Cystic Fibrosis Populations Excluded from Clinical Trials or Labeling of Marketed Therapies | Annals of the American Thoracic Society | 2016 | 1 |
| 50 | Trial design of bacteriophage therapy for nontuberculous mycobacteria pulmonary disease in cystic fibrosis: The POSTSTAMP study | Journal of Cystic Fibrosis | 2025 | 1 |