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Respiratory symptoms do not reflect functional impairment in early CF lung disease

Insa Korten1, Marc-Alexander Oestreich2, Urs Frey3

  • 1Division of Paediatric Respiratory Medicine and Allergology, Department of Paediatrics, Inselspital, Bern Unviersity Hospital, University of Bern, Switzerland.

Journal of Cystic Fibrosis : Official Journal of the European Cystic Fibrosis Society
|June 5, 2021
PubMed

Insights

Respiratory symptoms in infants with cystic fibrosis (CF) are not more frequent than in healthy infants during the first year of life. Early lung disease in CF may not be apparent through clinical symptoms alone.

Area of Science:

  • Pediatric Pulmonology
  • Neonatal Research
  • Genetic Disease Studies

Background:

  • Cystic Fibrosis (CF) can cause lung disease within the first year of life in infants.
  • The prevalence and intensity of infant respiratory symptoms in CF remain largely unknown.
  • Early detection of lung disease in CF infants is crucial for timely intervention.

Purpose of the Study:

  • To investigate the frequency and severity of respiratory symptoms in infants with CF compared to healthy controls.
  • To determine if early lung disease markers in CF infants correlate with clinical respiratory symptoms.

Main Methods:

  • Prospective birth cohort study involving 50 infants with CF and 50 matched healthy controls.
  • Standardized weekly interviews documented respiratory symptoms and rates over the first year.
  • Multiple Breath Washout (MBW) was performed in early infancy to assess lung function.

Main Results:

  • Analysis of 4552 data points revealed no significant difference in respiratory symptom frequency between CF and control infants (OR: 1.1; 95% CI: [0.76, 1.59]; p=0.6).
  • Elevated lung clearance index and increased respiratory rate in CF infants were not associated with the presence of respiratory symptoms.
  • Clinical presentation alone may not accurately reflect the extent of early lung disease in infants with CF.

Conclusions:

  • Infants with cystic fibrosis do not exhibit more frequent respiratory symptoms in their first year compared to healthy infants.
  • These findings suggest that subclinical lung disease may be present in early CF, undetectable by clinical observation.
  • Further research is needed to identify reliable early biomarkers for CF lung disease in infancy.
Abstract

Keywords:
Cohort studyCystic fibrosisHealthy controlsInfancyRespiratory symptoms

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