Document Detail

Lung function is abnormal in 3-month-old infants with cystic fibrosis diagnosed by newborn screening.
MedLine Citation:
PMID:  22752198     Owner:  NLM     Status:  MEDLINE    
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 ∼3 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 ∼3 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), p=0.02; 0.4 (0.1 to 0.7), p=0.02 and 0.9 (0.4 to 1.3), p<0.001, z-scores, respectively), while forced expiratory volume (FEV(0.5)) and flows (FEF(25-75)) were significantly lower (-0.9 (-1.3 to -0.6), p<0.001 and -0.7 (-1.1 to -0.2), p=0.004, 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 -1.96 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(25-75) (r=-0.43, 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.
Ah-Fong Hoo; Lena P Thia; The Thanh Diem Nguyen; Andrew Bush; Jane Chudleigh; Sooky Lum; Deeba Ahmed; Ian Balfour Lynn; Siobhan B Carr; Richard J Chavasse; Kate L Costeloe; John Price; Anu Shankar; Colin Wallis; Hilary A Wyatt; Angela Wade; Janet Stocks;
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't     Date:  2012-06-29
Journal Detail:
Title:  Thorax     Volume:  67     ISSN:  1468-3296     ISO Abbreviation:  Thorax     Publication Date:  2012 Oct 
Date Detail:
Created Date:  2012-09-10     Completed Date:  2012-11-29     Revised Date:  2013-01-16    
Medline Journal Info:
Nlm Unique ID:  0417353     Medline TA:  Thorax     Country:  England    
Other Details:
Languages:  eng     Pagination:  874-81     Citation Subset:  IM    
Respiratory Unit, Great Ormond Street Hospital for Children NHS Foundation Trust, London WC1N 3JH, UK.
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MeSH Terms
Case-Control Studies
Chi-Square Distribution
Cystic Fibrosis / diagnosis*,  physiopathology*
Early Diagnosis
Infant, Newborn
Linear Models
Longitudinal Studies
Neonatal Screening*
Respiratory Function Tests
Grant Support
//Department of Health
Comment In:
Thorax. 2012 Oct;67(10):849-50   [PMID:  22960983 ]

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

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