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[Early lung disease in infants with cystic fibrosis. Diagnostic tools and possible therapeutic pathways]

P Reix1, S Matecki2, M Fayon3

  • 1UMR5558, centre de référence de la mucoviscidose, hospices civils de Lyon, 59, boulevard Pinel, 69677 Bron, France.

Revue Des Maladies Respiratoires
|February 7, 2016
PubMed

Insights

Infants with cystic fibrosis (CF) may have lung abnormalities at birth, not just later. Early detection using specialized tests can help identify issues like airway narrowing and inflammation, paving the way for timely interventions.

Area of Science:

  • Pediatric Pulmonology
  • Genetic Disorders
  • Respiratory Medicine

Background:

  • Cystic fibrosis (CF) lung disease was historically thought to begin after birth.
  • Emerging evidence from animal models and human studies challenges this notion.
  • Constitutional airway abnormalities may be present from birth in CF infants.

Purpose of the Study:

  • To investigate the presence and implications of early lung abnormalities in infants with cystic fibrosis.
  • To highlight the potential for early detection and intervention in CF lung disease.

Main Methods:

  • Review of animal CF models showing congenital airway abnormalities.
  • Analysis of longitudinal birth cohort studies tracking asymptomatic CF infants.
  • Assessment of lung function, air trapping, bronchiectasis, and neutrophil elastase presence at 3 months of age.
  • Consideration of advanced imaging and pulmonary function testing tools.

Main Results:

  • 25% of asymptomatic CF infants showed decreased lung function by 3 months.
  • Air trapping (68%) and bronchiectasis (28%) were observed in CF infants at 3 months.
  • Neutrophil elastase at 3 months increased bronchiectasis risk threefold by age 3 years.

Conclusions:

  • Cystic fibrosis lung disease may manifest with structural and functional abnormalities very early in life, potentially from birth.
  • Advanced infant pulmonary function tests and imaging are crucial for early detection and intervention trials.
  • Early therapeutic interventions hold promise for altering the natural history of CF lung disease.

Keywords:
Computed tomographyCystic fibrosisFonction respiratoireInfantLung functionMucoviscidoseNourrissonScanner

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