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Early initiation of inhaled corticosteroids for the prevention and treatment of acute chest syndrome does not reduce its morbidity in pediatric patients with sickle cell disease

key information

source: The American Society of Pediatric Hematology/Oncology

year: 2016

authors: Alexis Leonard, Chevy Chase, Nihal Godiwala, Robert McCarter, Matthew Sharron, Emily R. Meier

summary/abstract:

Background: Acute chest syndrome (ACS) is the leading cause of mortality in patients with sickle cell disease (SCD). Treatment is multimodal and includes anti-inflammatories like corticosteroids. While systemic corticosteroids may decrease length of hospital stay for ACS, the risk of readmission for vaso-occlusive crises (VOC) has limited their use. Inhaled corticosteroids (ICS) may be a safe alternative, but efficacy is currently unknown.

Objectives: To investigate the effects of initiation of ICS at the time of hospital admission in reducing ACS morbidity.

Design/Method: A case control study compared SCD patients with a discharge diagnosis of ACS who received ICS (cases) at admission to those who did not (controls). History of asthma and severity of ACS (unilateral infiltrate vs. bilateral) were collected. Outcome measures included transfusion and oxygen requirement, rate of PICU transfer, intubation, or BiPAP initiation, and need for readmission. Statistical analyses included Pearson chi-square and student t-tests.

Results: One hundred twenty SCD patients (55 controls, 65 cases) were included in this study. A significantly higher proportion of controls had bilateral infiltrates (40% vs. 20% of cases, p=0.03), but fewer had asthma (12.7% vs. 52.3% of cases, p<0.001). Twelve cases (18.3%) and 9 controls (16.3%) were transferred to the PICU (p=0.80). While a lower proportion of cases required intubation (8.3% vs. 33.3% of controls, p=0.15), more cases required BiPAP (15.3% vs. 5.4%, p=0.02). Transfusion rates (p=0.10), oxygen requirement (p=0.09), and readmission rates (p=0.41) did not differ between groups.

Conclusion: More controls had bilateral infiltrates in this study, suggesting more severe ACS, yet initiation of ICS in cases did not significantly decrease morbidity despite having less severe disease. More cases were diagnosed with asthma which may be why they were treated with ICS on admission despite less severe ACS. Overall, initiation of ICS did not decrease ACS morbidity as measured by the need for transfusion, oxygen supplementation, or PICU intervention. While systemic steroids increase the risk of readmission for VOC, cases were not more likely to be readmitted after the use of ICS. Despite a plausible pathogenic mechanism to reduce airway hyperreactivity, our study did not demonstrate a significant benefit of ICS in the treatment of ACS.

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