Real-world assessment of LCI following lumacaftor-ivacaftor initiation in adolescents and adults with cystic fibrosis.

Lung clearance index (LCI) is a biomarker of ventilation inhomogeneity. Data are scarce on its usefulness in daily practice for monitoring the effects of treatments in older children and adults with CF. In this French observational study of lumacaftor-ivacaftor, 63 of 845 patients (7.

Altered pulmonary gas transfer capacity and capillary blood volume in pediatric Crohn's disease.

Objectives: To describe diffusing capacity for carbon monoxide (DLCO) and its components, that is, membrane diffusing capacity (DmCO) and pulmonary capillary blood volume (Vc) in children with Crohn's disease (CD), and to investigate the correlation between these parameters and disease activity.

Working Hypothesis: The most common lung function abnormalities are a reduced pulmonary DLCO and small airways disorders which are in many instances, clinically silent. No valid explanations have been proposed regarding the modifications in gas transfer capacity in active CD.

Pulmonary outcome and its correlates in school-aged children born with a gestational age ≤ 32 weeks.

Background: There is limited data regarding factors influencing the respiratory outcome at school age of ex-preterms born since the introduction of antenatal steroids, surfactant replacement together with less aggressive ventilation.

Objectives: To establish the main antenatal, neonatal and early childhood respiratory correlates of respiratory status in school-aged children born at ≤ 32 weeks of gestation.

Methods: Ex-preterm children born at ≤ 32 weeks of gestation between 1997 and 2001 at Bordeaux University Hospital were evaluated at school age, using a respiratory questionnaire and lung function tests (spirometry, plethysmography, exercise challenge test and CO lung diffusing capacity DLCO measurements).

Efficacy and tolerance of high-dose inhaled ipratropium bromide vs. terbutaline in intubated premature human neonates.

Background: There is insufficient data to reliably assess the benefit of bronchodilators in ventilated premature neonates.

Objectives: To compare the efficacy/tolerance of inhaled ipratropium bromide (IB) vs. terbutaline (T) and to describe factors associated with their efficacy.

Montelukast does not protect against hyperoxia-induced inhibition of alveolarization in newborn rats.

Impaired lung development has been demonstrated in neonatal animals exposed to hyperoxia. High lung cys-leukotriene levels may be a contributing factor towards the increase in oxygen toxicity. We investigated the effect of cysteinyl-leukotriene inhibition using the receptor antagonist, montelukast (MK, Singulair), on hyperoxia-induced changes in lung parenchymal structure in neonatal rat pups.

Differential effect of dexamethasone and hydrocortisone on alveolar growth in rat pups.

Glucocorticoids are widely used in perinatology, since they decrease the incidence of respiratory distress syndrome and chronic lung disease. However, evidence is now increasing that their use in this age group may result in impaired alveolar lung growth and general development. The aim of this study was to determine whether a low dose of hydrocortisone (1 mg/kg/day for 11 days) was deleterious to lung growth in rat pups, as compared to an equivalent dose of dexamethasone.

Pharmacologically induced relaxation of tracheal smooth muscle is increased in hyperoxia-exposed 15-day-old rats.

Hyperoxia increases maximum airway contractility in newborn guinea pigs and immature rats. Studies examining the mechanisms of hyperoxia-induced airway hyperresponsiveness have focused on contractile mechanisms, although excessive airway narrowing could be due to impaired relaxation. Our objective was to determine the effects of hyperoxia on airway structure and relaxing properties in juvenile rats exposed to an inspired fraction of oxygen (FiO(2)) of 0.