Lung Function in Infants with Cystic Fibrosis Diagnosed by Newborn Screening

Linnane, Barry; Hall, Graham L.; Nolan, Gary; Brennan, Siobhain; Stick, Stephen M.; Sly, Peter D.; Robertson, Colin F.; Robinson, Philip; Franklin, Peter; Turner, Steven; Ranganathan, Sarath

doi:10.1164/rccm.200804-551oc

Cited by 167 publications

(141 citation statements)

References 36 publications

Supporting

Mentioning

126

Contrasting

Unclassified

Order By: Relevance

“…With the recent development of disease-modifying treatments for CF, there is a need for noninvasive techniques to monitor disease development and progression in young children [3,4]. While lung function tests offer an alternative to invasive surveillance, studies correlating disease outcomes and respiratory function have predominantly been limited to those using spirometry [5][6][7] or techniques that require sedation [8][9][10][11][12][13][14], and there have been relatively few studies in preschool-aged children [8,[15][16][17][18][19][20].…”

Section: Introductionmentioning

confidence: 99%

Impact of lung disease on respiratory impedance in young children with cystic fibrosis

et al. 2015

Self Cite

View full text Add to dashboard Cite

This study aimed to evaluate the ability of the forced oscillation technique (FOT) to detect underlying lung disease in preschool children with cystic fibrosis (CF) diagnosed following newborn screening.184 children (aged 3-6 years) with CF underwent lung function testing on 422 occasions using the FOT to assess respiratory resistance and reactance at the time of their annual bronchoalveolar lavage collection and chest computed tomography scan. We examined associations between FOT outcomes and the presence and progression of respiratory inflammation, infection and structural lung disease.Children with CF who had pronounced respiratory disease, including free neutrophil elastase activity, infection with pro-inflammatory pathogens and structural lung abnormalities had similar FOT outcomes to those children without detectable lung disease. In addition, the progression of lung disease over 1 year was not associated with worsening FOT outcomes.We conclude that the forced oscillation technique is relatively insensitive to detect underlying lung disease in preschool children with CF. However, FOT may still be of value in improving our understanding of the physiological changes associated with early CF lung disease. @ERSpublications Forced oscillation technique is insensitive in detecting lung disease in preschool children with cystic fibrosis http://ow.ly/R9rSU

show abstract

Section: Introductionmentioning

confidence: 99%

Impact of lung disease on respiratory impedance in young children with cystic fibrosis

et al. 2015

Self Cite

View full text Add to dashboard Cite

show abstract

“…Studies in infants using sedated pulmonary function testing, computed tomography scanning and bronchoalveolar lavage have demonstrated that CF lung disease often begins in the first several months of life [1][2][3][4][5][6][7][8][9]. 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.…”

mentioning

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

View full text Add to dashboard Cite

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.

show abstract

“…Small airways are involved early in the disease process [4], as indicated by trapped air on CT scans [5,6] and by lung function tests [7,8] of young children.…”

mentioning

confidence: 99%

Improved treatment response to dornase alfa in cystic fibrosis patients using controlled inhalation

Bakker¹,

Volpi²,

Salonini³

et al. 2011

Eur Respir J

View full text Add to dashboard Cite

Better treatment of obstructed small airways is needed in cystic fibrosis. This study investigated whether efficient deposition of dornase alfa in the small airways improves small airway obstruction.In a multicentre, double-blind, randomised controlled clinical trial, cystic fibrosis patients on maintenance treatment with 2.5 mL dornase alfa once daily were switched to a smart nebuliser and randomised to small airway deposition (n524) or large airway deposition (n525) for 4 weeks. The primary outcome parameter was forced expiratory flow at 75% of forced vital capacity (FEF75%).FEF75% increased significantly by 0.7 SD (5.2% predicted) in the large airways group and 1.2 SD (8.8% pred) in the small airways group. Intention-to-treat analysis did not show a significant difference in treatment effect between groups. Per-protocol analysis, excluding patients not completing the trial or with adherence ,70%, showed a trend (p50.06) in FEF75% Z-score and a significant difference (p50.04) between groups in absolute FEF75% (L?s -1 ) favouring small airway deposition. Improved delivery of dornase alfa using a smart nebuliser that aids patients in correct inhalation technique resulted in significant improvement of FEF75% in children with stable cystic fibrosis. Adherent children showed a larger treatment response for small airway deposition.

show abstract

Lung Function in Infants with Cystic Fibrosis Diagnosed by Newborn Screening

Cited by 167 publications

References 36 publications

Impact of lung disease on respiratory impedance in young children with cystic fibrosis

Impact of lung disease on respiratory impedance in young children with cystic fibrosis

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

Improved treatment response to dornase alfa in cystic fibrosis patients using controlled inhalation

Contact Info

Product

Resources

About