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Risk Factors and Outcomes of Pulmonary Exacerbations in Infants and Young Children With Cystic Fibrosis
Aoife Corcoran1, Laura Bennett2, Jennifer A Faerber2
1Division of Pulmonary and Sleep Medicine, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, USA.
Insights
Pulmonary exacerbation (PEx) in young cystic fibrosis (CF) patients is linked to Medicaid, pancreatic insufficiency, and specific infections. Early PEx negatively impacts lung function (FEV1) and BMI in later childhood.
Area of Science:
- Pediatric Pulmonology
- Cystic Fibrosis Research
- Epidemiology of Respiratory Diseases
Background:
- Limited data exists on risk factors for pulmonary exacerbation (PEx) in very young people with cystic fibrosis (VY-PwCF).
- Understanding early-life PEx is crucial for predicting long-term health outcomes in CF patients.
Purpose of the Study:
- To identify risk factors for PEx in the first three years of life in children with CF.
- To investigate the relationship between early-life PEx and subsequent nutritional and pulmonary outcomes.
Main Methods:
- Analysis of demographic, clinical, and PEx data from the CF Foundation Patient Registry (CFFPR) for patients born between 2003 and 2017.
- Multivariable logistic regression was used to determine PEx probability between ages 1-3.
- Negative binomial and linear regressions modeled later-life FEV1 and BMI z-scores.
Main Results:
- Factors associated with increased PEx odds included Medicaid insurance, pancreatic insufficiency, pre-age-3 asthma, pre-age-1 hypertonic saline use, and specific bacterial infections (MRSA, S. maltophilia).
- Patients experiencing early PEx had lower FEV1 at age 6 and lower BMI z-scores at age 3.
Conclusions:
- Several risk factors for PEx in the first three years of life were identified.
- Early-life PEx negatively impacts lung function (ppFEV1) at age 6 and BMI z-score at age 3.
- Findings aid clinicians in counseling caregivers and identifying high-risk infants with CF.
Rationale:
There are limited data on the risk factors associated with pulmonary exacerbation (PEx) in very young people with CF (VY-PwCF). The goal of our study was to examine the risk factors associated with PEx in the first 3 years of life and the relationship between PEx in early life and subsequent nutritional and pulmonary outcomes.
Methods:
Using the CF Foundation Patient Registry (CFFPR), we analyzed demographic, clinical and PEx data on PwCF born between 2003 and 2017. Multivariable logistic regression analyzed the probability of a PEx between ages 1 and 3, and later-life forced expiratory volume in 1 s (FEV1) and body mass index (BMI) z-scores were modelled using negative binomial and linear regressions, respectively.
Results:
A total of 7,342 patients were included in our analysis. Medicaid insurance status, pancreatic insufficiency, asthma diagnosis before age 3, hypertonic saline use before age 1 and a positive respiratory culture for methicillin-resistant Staphylococcus Aureus and Stenotrophomonas maltophilia respectively before age 1 were associated with an increased odds of PEx diagnosis. FEV1 at age 6 and BMI z-scores at age 3 were lower in those who experienced early PEx.
Conclusions:
We identified several risk factors that are associated with PEx diagnosis in the first 3 years of life and found that PEx in the first 3 years negatively impacted ppFEV1 at age 6 and BMI z-score at age 3. These data will be helpful for clinicians in counselling caregivers of VY-PwCF on PEx risk factors and identifying patients at higher risk of PEx in the first 3 years of life.
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