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

The Thanh-Diem Nguyen1, Lena P Thia2, Ah-Fong Hoo3

  • 1Portex Unit: Respiratory Physiology and Medicine, UCL Institute of Child Health, London, UK Department of Respiratory Medicine, Centre Hospitalier Universitaire Sainte-Justine, Montreal, Quebec, Canada.

Thorax
|September 28, 2013
PubMed

Insights

Newborn screening for cystic fibrosis (CF) shows improved lung function (FEV₀.₅) in infants within the first year. Early lung function at 3 months predicts later outcomes, identifying high-risk infants for intensified treatment and clinical trials.

Area of Science:

  • Pediatric Pulmonology
  • Neonatal Screening
  • Genetics and Genomics

Background:

  • Newborn screening (NBS) for cystic fibrosis (CF) enables early intervention.
  • Uncertainty in lung function evolution hinders randomized controlled trial (RCT) design in infants with CF.

Purpose of the Study:

  • To evaluate pulmonary function changes in the first year of life for infants identified with CF through NBS.
  • To establish the predictive value of early lung function measurements for long-term outcomes.

Main Methods:

  • An observational longitudinal study involving infants with CF detected via NBS and healthy controls.
  • Measurement of Lung Clearance Index (LCI), plethysmographic lung volume (FRCpleth), and forced expired volume (FEV₀.₅) at 3 months and 1 year.
  • Comparison of pulmonary function parameters between CF infants and controls, and tracking changes over time.

Main Results:

  • CF infants showed significantly poorer lung function at 3 months compared to controls.
  • FEV₀.₅ improved significantly in CF infants from 3 months to 1 year, nearing control levels.
  • LCI and FRCpleth remained stable but elevated in CF infants throughout the first year.
  • Early lung function at 3 months strongly predicted pulmonary function at 1 year, identifying stable and high-risk groups.

Conclusions:

  • This study is the first to report FEV₀.₅ improvement over time in stable, screened CF infants on standard therapy.
  • Lung function changes by 1 year were less severe than previously documented.
  • Early pulmonary function assessment at 3 months is crucial for identifying high-risk CF infants who may benefit from intensified treatment and RCT enrollment.
Abstract

Keywords:
Cystic FibrosisLung PhysiologyPaediatric Lung Disaese

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