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High-risk age window for mortality in children with cystic fibrosis after lung transplantation
Don Hayes1, Karen S McCoy, Bryan A Whitson
1Department of Pediatrics, The Ohio State University, Columbus, OH, USA; Department of Internal Medicine, The Ohio State University, Columbus, OH, USA; Section of Pulmonary Medicine, Nationwide Children's Hospital, Columbus, OH, USA.
Insights
Lung transplantation (LTx) for cystic fibrosis (CF) in children shows the highest mortality risk between ages 16-20. Survival improves significantly after this critical age window, suggesting optimized post-transplant care timing may be beneficial.
Area of Science:
- Pediatric Pulmonology
- Transplant Surgery
- Biostatistics
Background:
- Lung transplantation (LTx) in pediatric patients with cystic fibrosis (CF) presents unique challenges and remains a topic of debate.
- Understanding post-transplant mortality risk is crucial for optimizing outcomes in this vulnerable population.
Purpose of the Study:
- To identify the age-specific hazard of mortality in children with CF following lung transplantation.
- To determine if there is a specific age window associated with the highest risk of post-transplant mortality.
Main Methods:
- Analysis of the United Network for Organ Sharing (UNOS) database from 1987 to 2013.
- Inclusion of pediatric CF patients (<18 years at transplant) in survival analysis (n=489).
- Application of the Proportional Hazards Cox Regression (PCHR) model to quantify mortality risk over attained age.
Main Results:
- The highest hazard of post-transplant mortality was observed in pediatric CF patients between 16 and 20 years of attained age.
- A statistically significant decline in the hazard of mortality was noted immediately following this high-risk age window (HR=0.394, p=0.004).
- This decline in mortality risk persisted even after adjusting for potential confounding factors.
Conclusions:
- Mortality risk following lung transplantation in children with CF is significantly elevated between 16 and 20 years of age.
- Outcomes appear to improve after this critical age period, suggesting a potential benefit from timing interventions or closer monitoring during this phase.
- These findings have implications for managing pediatric CF patients undergoing LTx and highlight the importance of age-specific risk stratification.
Abstract:
LTx in children with CF remains controversial. The UNOS database was queried from 1987 to 2013 for CF patients <18 yr of age at time of transplant. PCHR model was used to quantify hazard of mortality. 489 recipients were included in the survival analysis. The hazard function of post-transplant mortality was plotted over attained age to identify age window of highest risk, which was 16-20 yr. Unadjusted PCHR model revealed ages immediately after the high-risk window were characterized by lower hazard of mortality (HR = 0.472; 95% CI = 0.302, 0.738; p = 0.001). After adjusting for potential confounders, the decline in mortality hazard immediately after the high-risk window remained statistically significant (HR = 0.394; 95% CI: 0.211, 0.737; p = 0.004). Hazard of mortality in children with CF after LTx was highest between 16 and 20 yr of attained age and declined thereafter.
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