WS7.5 Lung function and structure in CF infants diagnosed through newborn screening (NBS)

Thia, L.P.; Calder, Alistair; Owens, CM; Hoo, Ah‐Fong; Nguyen, Thanh-Diem; Wallis, Colin; Bush, Andrew; Stocks, J

doi:10.1016/s1569-1993(12)60048-4

Journal of Cystic Fibrosis

2012

DOI: 10.1016/s1569-1993(12)60048-4

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WS7.5 Lung function and structure in CF infants diagnosed through newborn screening (NBS)

L.P. Thia¹,

Alistair Calder²,

CM Owens³

et al.

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An increasing body of evidence indicates that cystic fibrosis (CF) lung disease starts very early in life with studies identifying abnormalities on chest computed tomography (CT) scan even in infancy. 1,2 Patients develop initially transient Pseudomonas aeruginosa infections that can be eradicated, but later in life P aeruginosa usually persists and chronic infection develops. 3 Chronic infection is associated with worse lung function, nutritional status, and abnormal radiographic scores.

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Early-Life Pseudomonas aeruginosa Infection in Cystic Fibrosis and Lung Disease Progression

Petrocheilou

Papagrigoriou-Theodoridou

Michos

et al. 2017

Global Pediatric Health

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Lung disease in cystic fibrosis (CF) starts early, with studies identifying abnormalities on chest computed tomography (CT) scan even in infancy. In this retrospective study, abnormal chest CT was the main outcome; body mass index (BMI) z score and forced expiratory volume percent predicted (FEV1%) predicted at age 6 to 7 years were secondary outcomes. Pseudomonas aeruginosa infection prior to 12 months of age was the main explanatory variable. There was no association between early P aeruginosa infection and abnormal CT after adjustment for CFTR (cystic fibrosis transmembrane conductance regulator) functional mutation class, gender, and other pathogens (odds ratio = 0.30; 95% confidence interval = 0.07-1.35; P = .11). No significant associations were demonstrated for BMI z score and FEV1% predicted. Children with class I-III CFTR mutations had increased risk of abnormal CT findings (odds ratio = 11.67; 95% confidence interval = 1.11-115.06; P = .035) and lower FEV1% predicted (P = .04). In the current era, early-life P aeruginosa infection in CF might not influence the severity of lung disease in school age as much as previously. Larger studies are needed to confirm this finding.

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mentioning

confidence: 99%

Early-Life Pseudomonas aeruginosa Infection in Cystic Fibrosis and Lung Disease Progression

Petrocheilou

Papagrigoriou-Theodoridou

Michos

et al. 2017

Global Pediatric Health

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Evolution of lung function during the first year of life in newborn screened cystic fibrosis infants

Thia

Hoo

Bush

et al. 2013

Thorax

101

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RationaleNewborn screening (NBS) for cystic fibrosis (CF) allows early intervention. Design of randomised controlled trials (RCT) is currently impeded by uncertainty regarding evolution of lung function, an important trial end point in such infants.ObjectiveTo assess changes in pulmonary function during the first year of life in CF NBS infants.MethodsObservational longitudinal study. CF NBS infants and healthy controls were recruited between 2009 and 2011. Lung Clearance Index (LCI), plethysmographic lung volume (plethysmographic functional residual capacity (FRCpleth)) and forced expired volume (FEV0.5) were measured at 3 months and 1 year of age.Main resultsPaired measurements were obtained from 72 CF infants and 44 controls. At 3 months, CF infants had significantly worse lung function for all tests. FEV0.5 improved significantly (0.59 (95% CI 0.18 to 0.99) z-scores; p<0.01) in CF infants between 3 months and 1 year, and by 1 year, FEV0.5 was only 0.52 (0.89 to 0.15) z-scores less than in controls. LCI and FRCpleth remained stable throughout the first year of life, being on average 0.8 z-scores higher in infants with CF. Pulmonary function at 1 year was predicted by that at 3 months. Among the 45 CF infants with entirely normal LCI and FEV0.5 at 3 months, 80% remained so at 1 year, while 74% of those with early abnormalities remained abnormal at 1 year.ConclusionsThis is the first study reporting improvements in FEV0.5 over time in stable NBS CF infants treated with standard therapy. Milder changes in lung function occurred by 1 year than previously reported. Lung function at 3 months predicts a high-risk group, who should be considered for intensification of treatment and enrolment into RCTs.

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WS7.5 Lung function and structure in CF infants diagnosed through newborn screening (NBS)

Cited by 2 publications

References 0 publications

Early-Life Pseudomonas aeruginosa Infection in Cystic Fibrosis and Lung Disease Progression

Early-Life Pseudomonas aeruginosa Infection in Cystic Fibrosis and Lung Disease Progression

Evolution of lung function during the first year of life in newborn screened cystic fibrosis infants

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