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Wheezing in infants with cystic fibrosis: clinical course, pulmonary function, and survival analysis
1Pulmonary Division, Hospital for Sick Children, Toronto, Ontario, Canada.
Insights
Infants with cystic fibrosis (CF) who wheezed showed persistent pulmonary function abnormalities later in childhood, despite wheezing resolution. This highlights the importance of monitoring lung function in these patients.
Area of Science:
- Pediatric Pulmonology
- Genetic Disorders
- Respiratory Medicine
Background:
- Wheezing is a common symptom in infants diagnosed with cystic fibrosis (CF).
- Understanding the long-term clinical outcomes associated with early-life wheezing in CF is crucial for patient management.
Purpose of the Study:
- To determine the prevalence of wheezing in infants with CF.
- To compare the clinical outcomes, including pulmonary function and survival, of CF patients who wheezed in infancy versus those who did not.
Main Methods:
- A cohort of 229 CF patients diagnosed before age 2 (born 1965-1979) was retrospectively analyzed.
- Physician-documented wheezing in the first two years of life was recorded.
- Pulmonary function tests (spirometry) and survival data were compared between wheezing and non-wheezing groups at ages 7 and 13.
Main Results:
- 25% of infants with CF experienced physician-documented wheezing in the first two years.
- Wheezing resolved by age 4 in 75% of affected patients.
- Wheezing infants showed significantly lower forced expiratory flow rates at ages 7 and 13 compared to non-wheezers, despite similar survival rates.
Conclusions:
- Infant wheezing in cystic fibrosis, while often resolving, is associated with persistent pulmonary function deficits.
- Early wheezing in CF patients may indicate a predisposition to long-term respiratory compromise.
- Monitoring pulmonary function is essential for CF patients with a history of infant wheezing.
Abstract:
Wheezing is a common finding in infants with cystic fibrosis (CF). This study was undertaken to determine the prevalence of wheezing in infants with CF and to compare the clinical outcome of those who wheezed in infancy with that of those who did not. The study cohort included 229 CF patients born between 1965 and 1979 with CF diagnosed before 2 years of age. Fifty-seven (25%) had physician-documented wheezing during the first 2 years of life. Wheezing had resolved by the age of 2 years in 50% of the patients and by the age of 4 years in 75%. Although wheezing seemed to be linked to a family history of allergy and asthma, the frequency of the delta F508 mutation was similar to that of the non-wheezers. There was no significant difference in survival at the age of 13 years between the two groups. At the age of 7 years, patients who had wheezed had significantly lower forced expiratory flow rate at mid-expiratory phase (85 +/- 34% predicted) compared with those with no wheezing history (101 +/- 34% predicted). At the age of 13 years, forced expiratory volume in 1 second values was lower in the wheezing group (69 +/- 24% predicted vs 78 +/- 21% predicted), as was forced expiratory flow rate at mid-expiratory phase (56 +/- 33% predicted vs 69 +/- 30% predicted). In conclusion, although wheezing in infants with CF seems to have diminished with age, pulmonary function abnormalities were more evident at 7 and 13 years of age in the group that wheezed than in the group that did not.