Results of a phase IIa study of VX-809, an investigational CFTR corrector compound, in subjects with cystic fibrosis homozygous for the F508del-CFTR mutation | Thorax | 2012 | 408 |
Heterozygous STAT1 gain-of-function mutations underlie an unexpectedly broad clinical phenotype | Blood | 2016 | 314 |
Mutational analysis of the adeno-associated virus type 2 (AAV2) capsid gene and construction of AAV2 vectors with altered tropism | Journal of Virology | 2000 | 305 |
The role of inflammation in the pathophysiology of CF lung disease | Clinical Reviews in Allergy and Immunology | 2002 | 180 |
Cystic fibrosis: a review of epidemiology and pathobiology | Clinics in Chest Medicine | 2007 | 120 |
The pharmacokinetics of colistin in patients with cystic fibrosis | Journal of Clinical Pharmacology | 2001 | 110 |
Inflammation in cystic fibrosis: An update | Pediatric Pulmonology | 2018 | 106 |
Multicenter evaluation of infant lung function tests as cystic fibrosis clinical trial endpoints | American Journal of Respiratory and Critical Care Medicine | 2010 | 84 |
Safety and tolerability of denufosol tetrasodium inhalation solution, a novel P2Y2 receptor agonist: results of a phase 1/phase 2 multicenter study in mild to moderate cystic fibrosis | Pediatric Pulmonology | 2005 | 82 |
Prolonged inflammatory response to acute Pseudomonas challenge in interleukin-10 knockout mice | American Journal of Respiratory and Critical Care Medicine | 2002 | 65 |
Treatment of paediatric narcolepsy with sodium oxybate: a double-blind, placebo-controlled, randomised-withdrawal multicentre study and open-label investigation | The Lancet Child and Adolescent Health | 2018 | 49 |
The role of inhaled corticosteroids in the management of cystic fibrosis | Paediatric Drugs | 2009 | 31 |
Positive expiratory pressure and oscillatory positive expiratory pressure therapies | Respiratory Care | 2007 | 31 |
Concise Review: Mesenchymal Stem Cell Therapy for Pediatric Disease: Perspectives on Success and Potential Improvements | Stem Cells Translational Medicine | 2017 | 29 |
Augmentation of CFTR maturation by S-nitrosoglutathione reductase | American Journal of Physiology - Lung Cellular and Molecular Physiology | 2016 | 28 |
Respiratory controversies in the critical care setting. Does airway pressure release ventilation offer important new advantages in mechanical ventilator support? | Respiratory Care | 2007 | 25 |
Sleep and respiratory physiology in children | Clinics in Chest Medicine | 2014 | 19 |
S-Nitrosoglutathione Attenuates Airway Hyperresponsiveness in Murine Bronchopulmonary Dysplasia | Molecular Pharmacology | 2016 | 18 |
Pathological Hyaluronan Matrices in Cystic Fibrosis Airways and Secretions | American Journal of Respiratory Cell and Molecular Biology | 2016 | 16 |
Sleep-disordered breathing and childhood asthma: clinical implications | Current Opinion in Pulmonary Medicine | 2013 | 14 |
Chronic asthma and Mesenchymal stem cells: Hyaluronan and airway remodeling | Journal of Inflammation | 2017 | 10 |
Blood eosinophilia is associated with unfavorable hospitalization outcomes in children with bronchiolitis | Pediatric Pulmonology | 2016 | 7 |
Adjunctive Pharmacotherapies in Children With Asthma Exacerbations Requiring Continuous Albuterol Therapy: Findings From The Ohio Pediatric Asthma Repository | Hospital Pediatrics | 2018 | 6 |
S-Nitrosoglutathione formation at gastric pH is augmented by ascorbic acid and by the antioxidant vitamin complex, Resiston | Pharmaceutical Biology | 2018 | 6 |
The value of care algorithms | Pharmacotherapy | 2006 | 6 |