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Acute chest syndrome, airway inflammation and lung function in sickle cell disease
Aliva De1, Sanford Williams2, Yujing Yao3
1Division of Pediatric Pulmonology, Vagelos College of Physicians and Surgeons, Columbia University Irving Medical Center, New York, NY, United States of America.
Insights
Children with acute chest syndrome (ACS) experience worse lung function and elevated inflammatory markers. This suggests airway inflammation contributes to impaired pulmonary function in sickle cell disease (SCD).
Area of Science:
- Pediatric Pulmonology
- Hematology
- Immunology
Background:
- Acute chest syndrome (ACS) is a serious complication of sickle cell disease (SCD), but its impact on lung function remains unclear.
- Inflammation is central to SCD pathophysiology, yet its direct link to lung function deficits is not well-established.
- This study investigates the relationship between ACS, lung function, and inflammatory markers in children with SCD.
Purpose of the Study:
- To determine if children with ACS have poorer lung function compared to those without ACS.
- To explore the association between lung function deficits and specific inflammatory cytokines in children with SCD.
- To identify factors influencing lung function in pediatric SCD patients.
Main Methods:
- An exploratory study enrolled patients from a prior 2-year randomized clinical trial.
- Patients were classified into ACS and non-ACS groups, with demographic, clinical, and serum cytokine data collected.
- Pulmonary function tests (PFTs) were assessed, alongside quantification of serum cytokines and leukotriene B4.
Main Results:
- Children with ACS exhibited lower total lung capacity (TLC) at baseline and 2 years, with significant declines in FEV1 and FEF25-75% over time.
- Elevated serum cytokines (IL-5, IL-13) were observed in the ACS group compared to the non-ACS group.
- Multivariable analysis revealed age, sex, asthma status, and ACS history as significant predictors of lung function parameters like FEV1, FVC, and TLC.
Conclusions:
- Pulmonary function abnormalities and elevated inflammatory markers are more prevalent in pediatric SCD patients with ACS.
- Findings suggest that airway inflammation in children with SCD and ACS contributes to impaired lung function.
- These insights highlight the importance of monitoring pulmonary health in children with SCD, particularly those experiencing ACS.
Background:
Acute chest syndrome (ACS) is an acute complication in SCD but its effects on lung function are not well understood. Inflammation is a key component of SCD pathophysiology but with an unclear association with lung function. We hypothesized that children with ACS had worse lung function than children without ACS and aimed to investigate the association of lung function deficits with inflammatory cytokines.
Methods:
Patients enrolled in a previous 2-year randomized clinical trial who had consented to future data use, were enrolled for the present exploratory study. Patients were categorized into ACS and non-ACS groups. Demographic and clinical information were collected. Serum samples were used for quantification of serum cytokines and leukotriene B4 levels and pulmonary function tests (PFTs) were assessed.
Results:
Children with ACS had lower total lung capacity (TLC) at baseline and at 2 years, with a significant decline in forced expiratory volume in 1 sec (FEV1) and mid-maximal expiratory flow rate (FEF25-75%) in the 2 year period (p = 0.015 and p = 0.039 respectively). For children with ACS, serum cytokines IL-5, and IL-13 were higher at baseline and at 2 years compared to children with no ACS. IP-10 and IL-6 were negatively correlated with PFT markers. In multivariable regression using generalized estimating equation approach for factors predicting lung function, age was significantly associated FEV1 (p = 0.047) and ratio of FEV1 and forced vital capacity (FVC)- FEV1/FVC ratio (p = 0.006); males had lower FEV1/FVC (p = 0.035) and higher TLC (p = 0.031). Asthma status was associated with FEV1 (p = 0.017) and FVC (p = 0.022); history of ACS was significantly associated with TLC (p = 0.027).
Conclusion:
Pulmonary function abnormalities were more common and inflammatory markers were elevated in patients with ACS, compared with those without ACS. These findings suggest airway inflammation is present in children with SCD and ACS, which could be contributing to impaired pulmonary function.
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