Published in Am J Respir Crit Care Med on March 30, 2006
Phase I/II trial of adeno-associated virus-mediated alpha-glucosidase gene therapy to the diaphragm for chronic respiratory failure in Pompe disease: initial safety and ventilatory outcomes. Hum Gene Ther (2013) 1.19
Natural history of pulmonary function in collagen VI-related myopathies. Brain (2013) 0.97
Assessment and management of respiratory function in patients with Duchenne muscular dystrophy: current and emerging options. Ther Clin Risk Manag (2015) 0.91
Respiratory assessment in centronuclear myopathies. Muscle Nerve (2014) 0.77
Prospective observational cohort study of patients with weaning failure admitted to a specialist weaning, rehabilitation and home mechanical ventilation centre. BMJ Open (2016) 0.76
The value of respiratory muscle testing in a child with congenital muscular dystrophy. Respirol Case Rep (2014) 0.75
Spontaneous Breathing Pattern as Respiratory Functional Outcome in Children with Spinal Muscular Atrophy (SMA). PLoS One (2016) 0.75
European Academy of Paediatrics, Barcelona, Spain, October 7-10, 2006. Abstracts. Eur J Pediatr (2006) 0.75
Non-invasive positive pressure ventilation to facilitate the post-operative respiratory outcome of spine surgery in neuromuscular children. Eur Spine J (2014) 0.75
Acute skeletal muscle wasting in critical illness. JAMA (2013) 6.38
Effect of bronchoscopic lung volume reduction on dynamic hyperinflation and exercise in emphysema. Am J Respir Crit Care Med (2004) 3.10
Volume targeted versus pressure support non-invasive ventilation in patients with super obesity and chronic respiratory failure: a randomised controlled trial. Thorax (2012) 2.66
Patent foramen ovale in severe obstructive sleep apnea: clinical features and effects of closure. Chest (2013) 2.65
Quadriceps strength predicts mortality in patients with moderate to severe chronic obstructive pulmonary disease. Thorax (2006) 2.55
Clinical and electrophysiological predictors of respiratory failure in Guillain-Barré syndrome: a prospective study. Lancet Neurol (2006) 2.50
Surgical approaches for lung volume reduction in emphysema. Clin Med (2014) 2.14
CCDC39 is required for assembly of inner dynein arms and the dynein regulatory complex and for normal ciliary motility in humans and dogs. Nat Genet (2010) 2.12
Is a raised bicarbonate, without hypercapnia, part of the physiologic spectrum of obesity-related hypoventilation? Chest (2015) 2.07
The 4-metre gait speed in COPD: responsiveness and minimal clinically important difference. Eur Respir J (2013) 2.07
Multicenter study of prevalence of nontuberculous mycobacteria in patients with cystic fibrosis in france. J Clin Microbiol (2009) 2.03
Health status assessment in routine clinical practice: the chronic obstructive pulmonary disease assessment test score in outpatients. Respiration (2012) 1.99
Patient-controlled positive end-expiratory pressure with neuromuscular disease: effect on speech in patients with tracheostomy and mechanical ventilation support. Chest (2013) 1.97
Angiotensin converting enzyme genotype and strength in chronic obstructive pulmonary disease. Am J Respir Crit Care Med (2004) 1.87
Phox2b controls the development of peripheral chemoreceptors and afferent visceral pathways. Development (2003) 1.84
Age-related prevalence and distribution of nontuberculous mycobacterial species among patients with cystic fibrosis. J Clin Microbiol (2005) 1.83
Noninvasive positive pressure ventilation: five years of experience in a pediatric intensive care unit. Pediatr Crit Care Med (2006) 1.80
Outpatient pulmonary rehabilitation following acute exacerbations of COPD. Thorax (2010) 1.79
Loss-of-function mutations in the human ortholog of Chlamydomonas reinhardtii ODA7 disrupt dynein arm assembly and cause primary ciliary dyskinesia. Am J Hum Genet (2009) 1.65
Bronchoscopic volume reduction with valve implants in patients with severe emphysema. Lancet (2003) 1.64
Influence of immersion in water on muscle function and breathing pattern in patients with severe diaphragm weakness. Chest (2004) 1.59
Neurally adjusted ventilatory assist in patients recovering spontaneous breathing after acute respiratory distress syndrome: physiological evaluation. Crit Care Med (2010) 1.58
The COPD assessment test (CAT): response to pulmonary rehabilitation. A multicentre, prospective study. Thorax (2011) 1.54
Validity of physical activity monitors during daily life in patients with COPD. Eur Respir J (2013) 1.53
Quadriceps wasting and physical inactivity in patients with COPD. Eur Respir J (2012) 1.52
Transdiaphragmatic pressure control of airway pressure support in healthy subjects. Am J Respir Crit Care Med (2003) 1.51
Sustained elevation of circulating growth and differentiation factor-15 and a dynamic imbalance in mediators of muscle homeostasis are associated with the development of acute muscle wasting following cardiac surgery. Crit Care Med (2013) 1.51
Noninvasive positive-pressure ventilation avoids recannulation and facilitates early weaning from tracheotomy in children. Pediatr Crit Care Med (2010) 1.49
An Exploratory Study of Long-Term Outcome Measures in Critical Illness Survivors: Construct Validity of Physical Activity, Frailty, and Health-Related Quality of Life Measures. Crit Care Med (2016) 1.45
Noninvasive ventilation and breathing-swallowing interplay in chronic obstructive pulmonary disease*. Crit Care Med (2014) 1.45
Methodological aspects of bronchoscopic lung volume reduction with a proprietary system. Respiration (2004) 1.43
A randomized controlled trial of angiotensin-converting enzyme inhibition for skeletal muscle dysfunction in COPD. Chest (2014) 1.43
Pompe disease: a neuromuscular disease with respiratory muscle involvement. Respir Med (2009) 1.41
The efficacy of premixed nitrous oxide and oxygen for fiberoptic bronchoscopy in pediatric patients: a randomized, double-blind, controlled study. Chest (2004) 1.40
Downregulation of the serum response factor/miR-1 axis in the quadriceps of patients with COPD. Thorax (2011) 1.35
High pressure versus high intensity noninvasive ventilation in stable hypercapnic chronic obstructive pulmonary disease: a randomized crossover trial. Int J Chron Obstruct Pulmon Dis (2012) 1.31
Predicting outcomes from 6-minute walk distance in chronic obstructive pulmonary disease. J Am Med Dir Assoc (2011) 1.28
Interstitial lung diseases in children. Orphanet J Rare Dis (2010) 1.28
Major depressive disorder, sleep EEG and agomelatine: an open-label study. Int J Neuropsychopharmacol (2007) 1.27
Value of the chlorhexidine decontamination method for recovery of nontuberculous mycobacteria from sputum samples of patients with cystic fibrosis. J Clin Microbiol (2006) 1.25
Physical activity monitoring in COPD: compliance and associations with clinical characteristics in a multicenter study. Respir Med (2011) 1.23
Lung function indices for predicting mortality in COPD. Eur Respir J (2013) 1.23
Longitudinal lung function and structural changes in children with primary ciliary dyskinesia. Pediatr Pulmonol (2012) 1.19
Exercise-induced respiratory muscle fatigue: implications for performance. J Appl Physiol (1985) (2007) 1.18
An official American Thoracic Society Clinical Practice guideline: the diagnosis of intensive care unit-acquired weakness in adults. Am J Respir Crit Care Med (2014) 1.18
Lack of association between impaired glucose tolerance and appetite regulating hormones in patients with obstructive sleep apnea. J Clin Sleep Med (2011) 1.14
The value of multiple tests of respiratory muscle strength. Thorax (2007) 1.13
A prospective study of decline in fat free mass and skeletal muscle strength in chronic obstructive pulmonary disease. Respir Res (2007) 1.10
Vitamin D receptor genotypes influence quadriceps strength in chronic obstructive pulmonary disease. Am J Clin Nutr (2008) 1.10
Therapeutic strategies for idiopathic chylothorax. J Pediatr Surg (2008) 1.09
Comparative effects of two ventilatory modes on speech in tracheostomized patients with neuromuscular disease. Am J Respir Crit Care Med (2002) 1.09
Multiple-locus variable-number tandem-repeat analysis for longitudinal survey of sources of Pseudomonas aeruginosa infection in cystic fibrosis patients. J Clin Microbiol (2007) 1.09
The COPD Assessment Test (CAT): short- and medium-term response to pulmonary rehabilitation. COPD (2012) 1.09
Calcium-dependent regulation of NF-(kappa)B activation in cystic fibrosis airway epithelial cells. Cell Signal (2005) 1.09
Cardiac and respiratory effects of continuous positive airway pressure and noninvasive ventilation in acute cardiac pulmonary edema. Crit Care Med (2002) 1.08
Symptoms and quadriceps fatigability after walking and cycling in chronic obstructive pulmonary disease. Am J Respir Crit Care Med (2003) 1.07
Chest radiography cannot predict diaphragm function. Respir Med (2005) 1.07
The five-repetition sit-to-stand test as a functional outcome measure in COPD. Thorax (2013) 1.06
Clinical predictive value of manual muscle strength testing during critical illness: an observational cohort study. Crit Care (2013) 1.05
Pulmonary function tests in preschool children with cystic fibrosis. Am J Respir Crit Care Med (2002) 1.05
Increased skeletal muscle-specific microRNA in the blood of patients with COPD. Thorax (2013) 1.05
Ultrasound for the assessment of peripheral skeletal muscle architecture in critical illness: a systematic review. Crit Care Med (2015) 1.03
Heterogeneity of quadriceps muscle phenotype in chronic obstructive pulmonary disease (Copd); implications for stratified medicine? Muscle Nerve (2013) 1.03
Temporal dynamics of interferon gamma responses in children evaluated for tuberculosis. PLoS One (2009) 1.03
Neural respiratory drive as a physiological biomarker to monitor change during acute exacerbations of COPD. Thorax (2011) 1.03
Delineation of CCDC39/CCDC40 mutation spectrum and associated phenotypes in primary ciliary dyskinesia. J Med Genet (2012) 1.02
Reliability and validity of 4-metre gait speed in COPD. Eur Respir J (2012) 1.01
Effect of severe isolated unilateral and bilateral diaphragm weakness on exercise performance. Am J Respir Crit Care Med (2002) 1.01
Neutrophils in cystic fibrosis display a distinct gene expression pattern. Mol Med (2008) 1.00
Lung disease in Niemann-Pick disease. Pediatr Pulmonol (2007) 0.99
Structure to function: muscle failure in critically ill patients. J Physiol (2010) 0.99
Genetic variations in inflammatory mediators influence lung disease progression in cystic fibrosis. Pediatr Pulmonol (2008) 0.98
Intensive care unit acquired muscle weakness: when should we consider rehabilitation? Crit Care (2009) 0.98
Adherence of airway neutrophils and inflammatory response are increased in CF airway epithelial cell-neutrophil interactions. Am J Physiol Lung Cell Mol Physiol (2005) 0.98
Optimization of power wheelchair control for patients with severe Duchenne muscular dystrophy. Neuromuscul Disord (2004) 0.97
Carcinoid and mucoepidermoid bronchial tumours in children. Eur J Pediatr (2005) 0.97
Breathlessness and skeletal muscle weakness in patients undergoing lung health screening in primary care. COPD (2012) 0.97
Qualitative Ultrasound in Acute Critical Illness Muscle Wasting. Crit Care Med (2015) 0.97
Demonstration of a second rapidly conducting cortico-diaphragmatic pathway in humans. J Physiol (2004) 0.97
Clinical review: Update on neurally adjusted ventilatory assist--report of a round-table conference. Crit Care (2012) 0.97
Cough gastric pressure and maximum expiratory mouth pressure in humans. Am J Respir Crit Care Med (2003) 0.97
Singing teaching as a therapy for chronic respiratory disease--a randomised controlled trial and qualitative evaluation. BMC Pulm Med (2010) 0.96
Recombinant human acid alpha-glucosidase (rhGAA) in adult patients with severe respiratory failure due to Pompe disease. Neuromuscul Disord (2011) 0.96
Changes in pulmonary mechanics with increasing disease severity in children and young adults with cystic fibrosis. Am J Respir Crit Care Med (2002) 0.96