Lung function distinguishes preschool children with CF from healthy controls in a multi‐center setting

Kerby, Gwendolyn S.; Rosenfeld, Margaret; Ren, Clement L.; Mayer, Oscar H.; Brumback, Lyndia C.; Castile, Robert G.; Hart, Meeghan A.; Hiatt, Peter; Kloster, Margaret; Johnson, Robert P.; Jones, Paul; Davis, Stephanie D.

doi:10.1002/ppul.21589

Cited by 42 publications

(47 citation statements)

References 24 publications

(38 reference statements)

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“…However, there is evidence of tracking of spirometry outcomes over this period [63], and also evidence that children who are infected with Pseudomonas aeruginosa early in life have persistently lower spirometry outcomes than their uninfected peers [63]. Data from the recent US multicentre data suggest that spirometry outcomes are more discriminative than outcomes from forced oscillation or inductance plethysmography [78].…”

Section: Preschool Lung Functionmentioning

confidence: 99%

Early intervention studies in infants and preschool children with cystic fibrosis: are we ready?

et al. 2013

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Cystic fibrosis (CF) lung disease starts early in life and progresses even in the absence of clinical symptoms. Therefore, sensitive outcome measures to quantify and track these early abnormalities in infants and young children are needed; both for clinical care and interventional trials. Currently, the efficacy of most therapeutic interventions in CF has not been tested in children under the age of 6 years and drug development programmes have focused on assessing safety rather than efficacy in this age group. This article summarises the current status for outcome measures that can be utilised in clinical trials in infants and children with CF. Two methodologies are specifically highlighted in this review; chest computed tomography to assess structural damage of the lung and multiple breath washout as a technique to quantify ventilation inhomogeneity. While not all questions regarding the utility of these outcome measures in infants and young children have been resolved, significant advances have been made and it now appears feasible to design and conduct adequately powered efficacy studies in this age group. This could be a crucial step to further improve outcomes in CF patients as initiating effective treatment early is considered essential to prevent permanent lung damage. @ERSpublications Crucial to CF outcome is early effective treatment, hence intervention studies in infants and preschool children are a must

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Section: Preschool Lung Functionmentioning

confidence: 99%

Early intervention studies in infants and preschool children with cystic fibrosis: are we ready?

et al. 2013

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“…Subsequently, graduates of the IPFT study who were aged between 36 and 60 months of age were enrolled in a longitudinal study of preschool lung function [10,11] Requirement for inclusion in the current analysis was enrolment in both the infant and preschool study and at least one acceptable IPFT and one acceptable preschool spirometry test. Most participants in the current study were diagnosed following clinical presentation as universal newborn screening was available at only one of the six centres at the time of enrolment. Detailed eligibility criteria for the IPFT and preschool lung function studies have been previously published [9][10][11]. Informed consent was obtained from all parents/guardians and institutional review board approval was obtained at all centres.…”

Section: Participantsmentioning

confidence: 99%

“…DAVIS et al [9] recently reported mild but progressive obstructive lung disease in a multicentre US cohort of CF infants using the raised volume rapid thoracic compression (RVRTC) technique and whole-body plethysmography. A subset of these children subsequently enrolled in a multicentre study of preschool lung function [10,11]. Infant pulmonary function tests (IPFTs) are increasingly being employed to monitor early CF lung disease in the hopes that early detection of disease may improve outcomes.…”

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confidence: 99%

Lung function from infancy to preschool in a cohort of children with cystic fibrosis

Brumback

Davis²,

Kerby

et al. 2012

European Respiratory Journal

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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.

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“…The largest FEVt and FVC should be reported even if not taken from the same manoeuvre, and forced expiratory flows are taken from the manoeuvre with the largest sum of FEV0.5 and FVC [37]. In an experienced laboratory, acceptable spirometry can be obtained in over 75% of pre-school children, and success rates increase with age [39,40]. Between-test reproducibility is good for FEV0.5, FEV1 and FVC (intraclass correlation coefficient (ICC) 0.85-0.92) and moderate for FEF25-75 (ICC 0.60) [39].…”

Section: Pre-school Spirometrymentioning

confidence: 99%

Monitoring early lung disease in cystic fibrosis: where are we now?

Duncan

Aurora

2014

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Lung function distinguishes preschool children with CF from healthy controls in a multi‐center setting

Cited by 42 publications

References 24 publications

Early intervention studies in infants and preschool children with cystic fibrosis: are we ready?

Early intervention studies in infants and preschool children with cystic fibrosis: are we ready?

Lung function from infancy to preschool in a cohort of children with cystic fibrosis

Monitoring early lung disease in cystic fibrosis: where are we now?

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