Lung function in infants with cystic fibrosis (original) (raw)
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Lung function from infancy to school age in cystic fibrosis
Archives of Disease in Childhood, 1995
The aim was to investigate pulmonary mechanics in patients with cystic fibrosis during infancy and again in early childhood to see whether infant tests predicted status at school age. Plethysmographic measurements of thoracic gas volume and airways resistance were made in 29 patients at 6 months and again at 5 years 10 months. Maximum flow at functional residual capacity was measured during infancy for comparison with maximum expiratory flow rates during childhood. While many patients had normal measurements during infancy, pulmonary function had deteriorated by school age. Thoracic gas volume at school age was significantly related to the values in infancy, but other measurements made during childhood were independent of infant values. The relations between measurements in infancy and early childhood described here provide a background against which the role of external factors on pulmonary function in young children with cystic fibrosis can be investigated.
Course of lung function in children with cystic fibrosis in their first 3 years of life
Archivos Argentinos de Pediatria
Introduction. The early prevention of respiratory complications in children with cystic fibrosis is determining for a longer survival. The implementation of lung function tests in the first months of life allows to detect respiratory involvement, even in asymptomatic children. Objective. To assess the course of lung function in children with cystic fibrosis in their first 3 years of life and identify the factors affecting it. Population and methods. Observational, retrospective, analytical study. Children younger than 36 months with at least 2 lung function tests were included. Results. Between 2008 and 2016, 48 patients were included; 85 % of them had been diagnosed by newborn screening. The first lung function test was done at 5 months old. The median Z-score of maximal flow at functional residual capacity was-0.05 (interquartile range:-1.09 to 1.08). The median change in the maximal flow Z-score between tests was-0.32 (interquartile range:-1.11 to 0.25), p = 0.045. Patients with Staphylococcus aureus respiratory infections, especially methicillin-resistant SA, evidenced a greater deterioration of lung function compared to those without infection. Neither sex nor the type of genetic mutation were associated with the course of lung function. Nutritional recovery throughout the study was really good. Conclusion. Lung function in children with cystic fibrosis worsens progressively during their first 3 years of life. These findings are associated with Staphylococcus aureus respiratory infections.
Lung function from infancy to preschool in a cohort of children with cystic fibrosis
European Respiratory Journal, 2012
This study aimed to describe lung function in a cohort of children with cystic fibrosis (CF) who underwent infant pulmonary function tests (IPFTs) and preschool spirometry. Children performed up to four IPFTs (raised volume rapid thoracic compression technique) over 1 yr and five preschool spirometry tests over up to 2 yrs during participation in prospective, multicentre studies of infant and preschool lung function. All lung function data were reviewed centrally for measurement acceptability. 45 children had 252 acceptable measurements (137 IPFTs and 115 preschool spirometries) at ages 0.3-6.5 yrs. The median number of measurements per participant was 6 (range 3-9). Recent Pseudomonas aeruginosa infection was associated with 5.1% (95% CI 0.01-9.9%) lower forced expiratory volume in 0.5 s (FEV0.5) and 16.4% (95% CI 7.0-24.9%) lower forced expiratory flow at 25-25% of forced vital capacity (FEF25-75%), after adjustment for length, test type and centre. Recent cough was associated with 5.7% (95% CI 1.1-10.1%) lower FEV0.5 and 10.1% (95% CI 0.6-18.7%) lower FEF25-75%. Even after accounting for infection status, cough, sex, length, test type and centre, there was significant inter-individual variability in lung function (p,0.01 for each of FEV0.5, FEF25-75% and forced vital capacity). Recent P. aeruginosa infection and cough are associated with lower lung function in children with CF. Significant inter-individual variability in lung function remains to be explained.
Lung function is abnormal in 3-month-old infants with cystic fibrosis diagnosed by newborn screening
Thorax, 2012
Background Long-term benefits of newborn screening (NBS) for cystic fibrosis (CF) have been established with respect to nutritional status, but effects on pulmonary health remain unclear. Hypothesis With early diagnosis and commencement of standardised treatment, lung function at w3 months of age is normal in NBS infants with CF. Methods Lung clearance index (LCI) and functional residual capacity (FRC) using multiple breath washout (MBW), plethysmographic (pleth) FRC and forced expirations from raised lung volumes were measured in 71 infants with CF (participants in the London CF Collaboration) and 54 contemporaneous healthy controls age w3 months. Results Compared with controls, and after adjustment for body size and age, LCI, FRC MBW and FRC pleth were significantly higher in infants with CF (mean difference (95% CI): 0.5 (0.1 to 0.9), p¼0.02; 0.4 (0.1 to 0.7), p¼0.02 and 0.9 (0.4 to 1.3), p<0.001, z-scores, respectively), while forced expiratory volume (FEV 0.5 ) and flows (FEF 25e75 ) were significantly lower (À0.9 (À1.3 to À0.6), p<0.001 and À0.7 (À1.1 to À0.2), p¼0.004, z-scores, respectively). 21% (15/70) of infants with CF had an elevated LCI (>1.96 z-scores) and 25% (17/68) an abnormally low FEV 0.5 (below À1.96 z-scores). While only eight infants with CF had abnormalities of LCI and FEV 0.5 , using both techniques identified abnormalities in 35% (24/68). Hyperinflation (FRC pleth >1.96 z-scores) was identified in 18% (10/56) of infants with CF and was significantly correlated with diminished FEF 25e75 (r¼À0.43, p<0.001) but not with LCI or FEV 0.5 . Conclusion Despite early diagnosis of CF by NBS and protocol-driven treatment in specialist centres, abnormal lung function, with increased ventilation inhomogeneity and hyperinflation and diminished airway function, is evident in many infants with CF diagnosed through NBS by 3 months of age. , The Thanh Diem Nguyen, et al. newborn screening infants with cystic fibrosis diagnosed by Lung function is abnormal in 3-month-old http://thorax.bmj.com/content/67/10/874.full.html Updated information and services can be found at: These include: Data Supplement http://thorax.bmj.com/content/suppl/2012/06/28/thoraxjnl-2012-201747.DC1.html "Supplementary Data"
Pediatric Pulmonology, 2011
To analyze cross-sectional and longitudinal associations between lung function measures and clinical features in a cohort of preschool children with cystic fibrosis (CF). Methods: Longitudinal eight-center observational study of children with CF aged 36-60 months at enrollment, who underwent semiannual pulmonary function tests (PFTs) for up to 2 years consisting of spirometry (all 8 sites), forced oscillometry (FO, 5 sites), and measures of thoracoabdominal asynchrony using respiratory inductive plethysmography (IP, 5 sites). Results: Ninety-three subjects were enrolled; 181 acceptable spirometry measurements from 71 subjects, 128 FO from 47 subjects, and 142 IP from 50 subjects were available for analysis. Cross sectional analyses did not detect an association between any PFT parameter at enrollment and Pseudomonas aeruginosa (Pa) status, CF gene mutation class, Wisconsin cough score, Shwachman score, environmental tobacco smoke exposure, family history of asthma, or nutritional indices. In longitudinal analyses, Pa infection within 6 months preceding enrollment was associated with a significantly greater rate of decline in z-scores for forced expiratory flow between 25 and 75% of forced vital capacity (FEF 25-75) (À1.3 vs. À0.4 Z scores/year, P ¼ 0.024) and greater thoracoabdominal asynchrony measured by IP (mean phase angle difference 4.68, P ¼ 0.004). No other significant longitudinal associations were observed. Conclusions: Prior Pa infection is associated with a greater rate of decline in FEF 25-75 z-score and mild thoracoabdominal asynchrony in preschool children with CF. In this multicenter US study, significant associations between other lung function measures and clinical features were not detected. Pediatr Pulmonol.
Pediatric Pulmonology, 2001
The detection of early airway disease in infants with cystic ®brosis (CF) may lead to earlier intervention and an improved prognosis. We hypothesized that the ratio of maximal expiratory¯ows while breathing a mixture of helium and oxygen (heliox) and air, referred to as density dependence (DD), would identify early airway disease in infants with CF who have normal lung function. We also hypothesized that these infants with CF might be better differentiated from normal infants when the¯ows breathing heliox are compared instead of room air¯ows. We evaluated 10 infants with CF and 21 infants without CF and with normal lung function, de®ned as a forced vital capacity (FVC) and forced expiratory¯ows between 25±75% of expired volume (FEF 25±75 ) of greater than 70% predicted (z-score b À 2.0). Full forced expiratory maneuvers by the rapid thoracic compression technique were obtained while breathing room air and then heliox. Flow at 50% and 75% of expired volume (FEF 50 , FEF 75 ), FEF 25±75 , and FVC were calculated from the¯ow volume curve with patients and control subjects breathing each gas mixture. The ratio of heliox to air¯ow at FEF 50 and FEF 75 was calculated (DD 50 , DD 75 ), and the point where the two¯ow-volume curves crossed (V iso V H ) was also measured.