Abnormal lung structure in mid-childhood resulting from preterm birth in the contemporary era has important functional consequences.

BackgroundIn school-aged children with cystic fibrosis (CF) structural lung damage assessed using chest CT is associated with abnormal ventilation distribution. The primary objective of this analysis was to determine the relationships between ventilation distribution outcomes and the presence and extent of structural damage as assessed by chest CT in infants and young children with CF.MethodsData of infants and young children with CF diagnosed following newborn screening consecutively reviewed between August 2005 and December 2009 were analysed. Ventilation distribution (lung clearance index and the first and second moment ratios [LCI, M1/M0 and M2/M0, respectively]), chest CT and airway pathology from bronchoalveolar lavage were determined at diagnosis and then annually. The chest CT scans were evaluated for the presence or absence of bronchiectasis and air trapping.ResultsMatched lung function, chest CT and pathology outcomes were available in 49 infants (31 male) with bronchiectasis and air trapping present in 13 (27%) and 24 (49%) infants, respectively. The presence of bronchiectasis or air trapping was associated with increased M2/M0 but not LCI or M1/M0. There was a weak, but statistically significant association between the extent of air trapping and all ventilation distribution outcomes.ConclusionThese findings suggest that in early CF lung disease there are weak associations between ventilation distribution and lung damage from chest CT. These finding are in contrast to those reported in older children. These findings suggest that assessments of LCI could not be used to replace a chest CT scan for the assessment of structural lung disease in the first two years of life. Further research in which both MBW and chest CT outcomes are obtained is required to assess the role of ventilation distribution in tracking the progression of lung damage in infants with CF.

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None of the currently described scoring systems are appropriately validated or superior over another. Future studies are needed to generate a validated and universal chest CT quantitative scoring method for patients with BPD. Pediatr Pulmonol. 2016; 51:975-986. © 2016 Wiley Periodicals, Inc.

Young people who are overweight/obese are more likely to display expFL during submaximal exercise compared with children of healthy weight . Use of compensatory breathing strategies appeared to enable overweight children to avoid the experience of breathlessness at this intensity of exercise.

BackgroundPompe disease is a progressive metabolic myopathy. Involvement of respiratory muscles leads to progressive pulmonary dysfunction, particularly in supine position. Diaphragmatic weakness is considered to be the most important component. Standard spirometry is to some extent indicative but provides too little insight into diaphragmatic dynamics. We used lung MRI to study diaphragmatic and chest-wall movements in Pompe disease.MethodsIn ten adult Pompe patients and six volunteers, we acquired two static spirometer-controlled MRI scans during maximum inspiration and expiration. Images were manually segmented. After normalization for lung size, changes in lung dimensions between inspiration and expiration were used for analysis; normalization was based on the cranial-caudal length ratio (representing vertical diaphragmatic displacement), and the anterior-posterior and left-right length ratios (representing chest-wall movements due to thoracic muscles).ResultsWe observed striking dysfunction of the diaphragm in Pompe patients; in some patients the diaphragm did not show any displacement. Patients had smaller cranial-caudal length ratios than volunteers (p < 0.001), indicating diaphragmatic weakness. This variable strongly correlated with forced vital capacity in supine position (r = 0.88) and postural drop (r = 0.89). While anterior-posterior length ratios also differed between patients and volunteers (p = 0.04), left-right length ratios did not (p = 0.1).ConclusionsMRI is an innovative tool to visualize diaphragmatic dynamics in Pompe patients and to study chest-walland diaphragmatic movements in more detail. Our data indicate that diaphragmatic displacement may be severely disturbed in patients with Pompe disease.Electronic supplementary materialThe online version of this article (doi:10.1186/s12890-015-0058-3) contains supplementary material, which is available to authorized users.

AimWe assessed the feasibility of telehealth spirometry assessments for children with cystic fibrosis (CF) living in a regional setting.MethodPatients with acceptable computer hardware at home were provided with a SpiroUSB (Vyaire) spirometer. Spirometry was performed during ‘home admissions’ or for ongoing home monitoring in children living outside metropolitan Melbourne. At the end of the session, the family forwarded the data to the Royal Children’s Hospital, Melbourne.ResultsTwenty-two patients aged 7 to 17 years participated, with spirometry successful in 55 of 59 (93%) attempted sessions according to American Thoracic Society/European Respiratory Society criteria. The median distance between the subject’s home and the hospital was 238 km (range 62–537 km) which equated to a travel time saving of 5 hours and 34 min per hospital visit.ConclusionHome-based telehealth spirometry is feasible in children with CF and can support the CF team during home-based admissions and for ongoing outpatient monitoring.

Exhaled breath temperature measurements are highly feasible in children with a 95% success rate in this healthy population. Room temperature and SVC significantly influence EBT variables in healthy children. Further studies are required to investigate the ability of EBT to assess airway inflammation in children with respiratory disease. Pediatr. Pulmonol. 2011; 46:1062-1068. © 2011 Wiley Periodicals, Inc.