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[Early lung disease in cystic fibrosis]

M Fayon1, Y Ladipo2, F Galodé3

  • 1Université de Bordeaux, Centre de Recherche Cardio-thoracique de Bordeaux, U1045, F-33000 Bordeaux, France; CHU de Bordeaux, Centre de référence de la mucoviscidose, Centre d'Investigation Clinique (CIC 1401), F-33076 Bordeaux, France.

Archives De Pediatrie : Organe Officiel De La Societe Francaise De Pediatrie
|February 25, 2017
PubMed

Insights

Early lung inflammation and infection in infants with Cystic Fibrosis (CF) cause lasting damage. Prompt interventions in young children are crucial for better long-term health outcomes.

Area of Science:

  • Pediatric Pulmonology
  • Genetic Diseases
  • Inflammatory Conditions

Background:

  • Infants with Cystic Fibrosis (CF) experience early lung inflammation and infection.
  • This leads to impaired lung function and structural damage, even in asymptomatic children.
  • The CF-pig model exhibits congenital airway narrowing, impaired mucus, and bacterial clearance issues from birth.

Purpose of the Study:

  • To highlight the critical need for early interventions in infants with Cystic Fibrosis.
  • To emphasize the importance of adapted clinical trials for preschool-aged CF patients.
  • To underscore the potential impact of early treatment on the CF disease course.

Main Methods:

  • Review of recent data on early lung disease in infants with Cystic Fibrosis.
  • Analysis of CF-pig model characteristics.
  • Examination of lung function and structural damage markers in young CF patients.

Main Results:

  • 25% of screened 3-month-old CF infants show decreased lung function.
  • Air trapping (68%) and bronchiectasis (28%) are prevalent by 3 months.
  • Neutrophil elastase at 3 months triples bronchiectasis risk by age 3.

Conclusions:

  • Early lung inflammation and infection significantly impact CF progression.
  • Adapted clinical trials are essential for preschool-aged children with CF.
  • Early interventions, without hindering lung growth, could transform CF natural history.

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