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Showing results for "Neuromuscular disorders "

Reference values for spirometry: The way forward for our patients

Few clinical respiratory laboratories have assessed the impact of changing reference equations on the interpretation of spirometric outcomes.

Expression of bronchodilator response using forced oscillation technique measurements: absolute versus relative

Expression of bronchodilator response using forced oscillation technique measurements: absolute versus relative

Lung function testing in preschool-aged children with cystic fibrosis in the clinical setting

This study investigated the nature and prevalence of atypical pain responses in Rett syndrome and their relationships with specific MECP2 mutations.

Lung volume and ventilation inhomogeneity in preterm infants at 15-18 months corrected age

To assess whether lung volume and ventilation inhomogeneity in preterm infants at 15-18 months corrected age

Pandemic preparedness needs for children with rare diseases and their families: A perspective of COVID-19 experiences

People living with rare diseases had a high risk of negative health outcomes due to COVID-19. Pandemic preparedness will ensure best practice procedures and optimal outcomes during future pandemic events. This paper sought to understand the needs of children with rare diseases during the COVID-19 pandemic to inform preparation for future pandemic and disaster events. First, impacts and outcomes from the COVID-19 pandemic on people living with rare disease were identified in the literature.

Lung function changes in children exposed to mine fire smoke in infancy

Chronic, low-intensity air pollution exposure has been consistently associated with reduced lung function throughout childhood. However, there is limited research regarding the implications of acute, high-intensity air pollution exposure. We aimed to determine whether there were any associations between early life exposure to such an episode and lung growth trajectories.

PREDICT CF: Assessing the role of lung clearance index in predicting disease progression in children with cystic fibrosis

There is increasing evidence that the assessment of ventilation distribution using the multiple breath washout (MBW) technique is sensitive to changes in disease status of children with cystic fibrosis.

Lung inflammation and simulated airway resistance in infants with cystic fibrosis

Cystic fibrosis (CF) is characterized by small airway disease; but central airways may also be affected. We hypothesized that airway resistance estimated from computational fluid dynamic (CFD) methodology in infants with CF was higher than controls and that early airway inflammation in infants with CF is associated with airway resistance.

Variants associated with HHIP expression have sexdifferential effects on lung function

Lung function is highly heritable and differs between the sexes throughout life. However, little is known about sexdifferential genetic effects on lung function. We aimed to conduct the first genome-wide genotype-by-sex interaction study on lung function to identify genetic effects that differ between males and females.