Prognostic value of six minute walk test in cystic fibrosis adults.

Background: The 6 min walk test (6MWT) provides prognostic information in various respiratory diseases, but limited data exist in cystic fibrosis (CF) adults.

Methods: Consecutive CF adults who performed 6MWT at Cochin Hospital (Paris, France) over 12 years were analyzed. The cut-off 6 min walking distance (6MWD) value that best predicted a combined endpoint (death without transplant or lung transplant) was established using a receiver operating curve.

Small airways diseases, excluding asthma and COPD: an overview.

This review is the summary of a workshop on small airways disease, which took place in Porquerolles, France in November 2011. The purpose of this workshop was to review the evidence on small airways (bronchiolar) involvement under various pathophysiological circumstances, excluding asthma and chronic obstructive pulmonary disease. Histopathological patterns associated with small airways disease were reviewed, including cellular and obliterative bronchiolitis.

CFTR dysfunction induces vascular endothelial growth factor synthesis in airway epithelium.

Peribronchial angiogenesis may occur in cystic fibrosis and vascular endothelial growth factor (VEGF)-A regulates angiogenesis in airways. Peribronchial vascularity and VEGF-A expression were examined using immunocytochemistry and morphometric analysis in lung sections obtained in 10 cystic fibrosis patients at transplantation versus 10 control nonsmokers, and in two strains of Cftr-deficient mice versus wild-type littermates. Airway epithelial NCI-H292 cells and primary cultures of noncystic fibrosis human airway epithelial cells were treated with cystic fibrosis transmembrane conductance regulator (CFTR) inhibitors (CFTR-inh(172) or PPQ-102) or transfected with a CFTR small interfering (si)RNA with or without a selective epidermal growth factor receptor tyrosine kinase inhibitor.

Dysfunctional lung anatomy and small airways degeneration in COPD.

Chronic obstructive pulmonary disease (COPD) is characterized by incompletely reversible airflow obstruction. Direct measurement of airways resistance using invasive techniques has revealed that the site of obstruction is located in the small conducting airways, ie, bronchioles with a diameter < 2 mm. Anatomical changes in these airways include structural abnormalities of the conducting airways (eg, peribronchiolar fibrosis, mucus plugging) and loss of alveolar attachments due to emphysema, which result in destabilization of these airways related to reduced elastic recoil.

Association between Staphylococcus aureus alone or combined with Pseudomonas aeruginosa and the clinical condition of patients with cystic fibrosis.

Background: The prevalence of methicillin-resistant Staphylococcus aureus (MRSA) in cystic fibrosis (CF) patients has increased and MRSA seems to be associated with a poorer prognosis. The aim of this study was to assess the prevalence and clinical consequences of MRSA and methicillin-susceptible Staphylococcus aureus (MSSA), associated or not associated with Pseudomonas aeruginosa (PA).

Methods: In a retrospective study on 419 sputum producer patients (293 adults and 126 children >7 years of age), we recorded patient characteristics, lung function, nutritional status, i.

Two distinct chronic obstructive pulmonary disease (COPD) phenotypes are associated with high risk of mortality.

Rationale: In COPD patients, mortality risk is influenced by age, severity of respiratory disease, and comorbidities. With an unbiased statistical approach we sought to identify clusters of COPD patients and to examine their mortality risk.

Methods: Stable COPD subjects (n = 527) were classified using hierarchical cluster analysis of clinical, functional and imaging data.

Targeting cytosolic proliferating cell nuclear antigen in neutrophil-dominated inflammation.

New therapeutic approaches that can accelerate neutrophil apoptosis under inflammatory conditions to enhance the resolution of inflammation are now under study. Neutrophils are deprived of proliferative capacity and have a tightly controlled lifespan to avoid their persistence at the site of injury. We have recently described that the proliferating cell nuclear antigen (PCNA), a nuclear factor involved in DNA replication and repair of proliferating cells is a key regulator of neutrophil survival.

Impact of comorbidities on COPD-specific health-related quality of life.

Background: Comorbidities are frequent in subjects with COPD, but their contribution to health-related quality of life (HRQoL) impairment is not clearly established.

Methods: Cross-sectional analysis of data from the French COPD cohort Initiatives BPCO. Data were recorded in stable state and included spirometry, dyspnea (modified Medical Research Council - mMRC-scale), mood disorders (hospital anxiety-depression scale) and physician-diagnosed comorbidities including diabetes, hypertension, coronary artery disease, chronic heart failure, venous thromboembolism.

Sleep quality and nocturnal hypoxaemia and hypercapnia in children and young adults with cystic fibrosis.

Objectives: The aim of the study was to evaluate sleep quality and nocturnal gas exchange in patients with cystic fibrosis (CF) and to assess if sleep quality and daytime lung function could predict nocturnal hypoxaemia or hypercapnia.

Study Design: Daytime sleepiness and objective sleep quality were evaluated by the Pittsburgh Sleep Quality Index (PSQI) and actigraphy in 25 children and 55 young adults (mean age 24±10 years, forced expiratory volume in 1 s (FEV(1)) 41±11% predicted). Nocturnal gas exchange was assessed by pulse oximetry (SpO(2)) and transcutaneous carbon dioxide (PtcCO(2)) recordings.

[Prevention of COPD exacerbation: a fundamental challenge].

Introduction: Acute exacerbations of chronic obstructive pulmonary disease (AECOPD) are a cause of suffering for patients and a burden for healthcare systems and society. Their prevention represents individual and collective challenge. The present article is based on the work of a group of experts who met on 5th and 6th May 2011 and seeks to highlight the importance of AECOPD.

Pulmonary acceleration time to optimize the timing of lung transplant in cystic fibrosis.

Pulmonary hypertension (PH) may affect survival in cystic fibrosis (CF) and can be assessed on echocardiographic measurement of the pulmonary acceleration time (PAT). The study aimed at evaluating PAT as a tool to optimize timing of lung transplant in CF patients. Prospective multicenter longitudinal study of patients with forced expiratory volume in 1 second (FEV1) ≤60% predicted.

Association between occupational exposure and the clinical characteristics of COPD.

Background: The contribution of occupational exposures to COPD and their interaction with cigarette smoking on clinical pattern of COPD remain underappreciated. The aim of this study was to explore the contribution of occupational exposures on clinical pattern of COPD.

Methods: Cross-sectional data from a multicenter tertiary care cohort of 591 smokers or ex-smokers with COPD (median FEV1 49%) were analyzed.

Pneumococcal infections: association with asthma and COPD.

Pneumococci are responsible for lower respiratory tract infections (bronchitis and pneumonia) and invasive infections (bacteremia and meningitis). Pneumococcal vaccination is recommended for adults at high risk of pneumococcal infection. Asthma is not currently considered as an indication for pneumococcal vaccination and this vaccination is indicated only in case of respiratory insufficiency.

Pemetrexed, oxaliplatin and bevacizumab as first-line treatment in patients with stage IV non-small cell lung cancer.

Background: Oxaliplatin has less haematological toxicity than cisplatin and carboplatin. The combination of pemetrexed, oxaliplatin and bevacizumab appeared well tolerated and active as second- or third-line treatment in a previous phase II study. Its role as first-line therapy remains to define.

[Eosinophilic pleural effusion related to taking valproic acid].

Eosinophilic pleural effusions have multiple aetiologies. We report on the case of a 40-year-old man who experienced an eosinophilic pleural effusion with blood hypereosinophilia that occurred nine weeks after a treatment with valproic acid was introduced. Usual aetiologies of eosinophilic pleural effusion were excluded.

High prevalence of azole-resistant Aspergillus fumigatus in adults with cystic fibrosis exposed to itraconazole.

Aspergillus fumigatus is the most frequent fungus found in the sputum of cystic fibrosis (CF) subjects. Itraconazole is prescribed for allergic bronchopulmonary aspergillosis (ABPA) or Aspergillus bronchitis in CF subjects. We hypothesized that A.

Employment and work disability in adults with cystic fibrosis.

Background: As a result of prolonged survival, more patients with cystic fibrosis (CF) participate in the labour force. The aim of this study was to evaluate their education, occupation levels and risk factors for work disability.

Method: 207 patients answered a self-administered questionnaire about their educational level and work status.