Lung Function from Infancy to the Preschool Years after Clinical Diagnosis of Cystic Fibrosis

Kozlowska, Wanda; Bush, Andrew; Wade, Angela; Aurora, Paul; Carr, S.B.; Castle, Rosie A.; Hoo, Ah‐Fong; Lum, Sooky; Price, John; Ranganathan, Sarath; Saunders, Clare; Stanojevic, Sanja; Stroobant, John; Wallis, Colin; Stocks, Janet

doi:10.1164/rccm.200710-1599oc

Cited by 129 publications

(102 citation statements)

References 43 publications

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“…[17][18][19][20] Additional exclusion criteria were history of apnoeic episodes, upper airway pathology, or previous physician-diagnosed lower respiratory tract infection. The potential clinical implications of alternative methods of expressing lung function was explored using data collected from infants with Cystic Fibrosis (CF), studied by the same investigators, using identical equipment and protocols.…”

Section: Study Populationmentioning

confidence: 99%

New reference equations to improve interpretation of infant lung function

Nguyen¹,

Hoo²,

Lum³

et al. 2012

Pediatric Pulmonology

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Summary. Rationale: With increasing use of infant pulmonary function tests (IPFTs) in both clinical and research studies, appropriate interpretation of results is essential. Objectives: To investigate the potential bias associated with ''normalising'' IPF by expressing results as a ratio of body size and to develop reference ranges for tidal breathing parameters, passive respiratory mechanics (compliance [C rs ] and resistance [R rs ]) and plethysmographic functional residual capacity (FRC p ) for white infants during the first 2 years of life. Methods: IPFTs were measured using the Jaeger BabyBody system and standardized protocols. Reference equations, adjusted for body size, age, and sex where appropriate, were created using multilevel modeling. Results: The ratio of lung function to body length changes markedly with growth, thereby precluding its use for any outcome. While the ratio of tidal volume and C rs to body weight remained relatively constant with growth, this was not the case for FRC p . Even in healthy infants, a strong inverse relationship was observed between lung function/body weight and weight z-score which could distort interpretation of results in growth-restricted infants with lung disease, such as cystic fibrosis. Reference equations were derived from 153 healthy white infants on 232 test occasions (median age 35.5 weeks [range: 2.6-104.7]). Crown-heel length was the strongest predictor of IPF. Conclusions: When reporting IPF, use of size-corrected ratios should be discouraged, with interpretation instead based on appropriate reference equations. The current equations are applicable to white infants and young children up to 2 years of age, studied using the same commercially available equipment. The extent to which these equations are applicable to infants and young children of other ethnic backgrounds or who are tested with different equipment needs to be established.

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Section: Study Populationmentioning

confidence: 99%

New reference equations to improve interpretation of infant lung function

Nguyen¹,

Hoo²,

Lum³

et al. 2012

Pediatric Pulmonology

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“…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

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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

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“…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. However, investigations of how lung function progresses from infancy to the preschool years have been limited [12,13]. The objectives of the current study were to describe the effect of demographic and clinical characteristics on lung function, the trends in lung function from infancy through preschool, and the individual variability in these trends in a cohort of children with CF who underwent sedated IPFT and cooperative preschool spirometry.…”

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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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Lung Function from Infancy to the Preschool Years after Clinical Diagnosis of Cystic Fibrosis

Cited by 129 publications

References 43 publications

New reference equations to improve interpretation of infant lung function

New reference equations to improve interpretation of infant lung function

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

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