Background: Acute chest syndrome (ACS) remains a leading cause of mortality among children with sickle cell disease (SCD); overall, nearly 50% of ACS is diagnosed during hospitalizations for other complications including painful vaso-occlusive crises (VOC).

Methods: The present study assesses epidemiological features of children hospitalized with VOC and evaluates factors potentially associated with development of ACS utilizing the large national all-payer Kid's Inpatient Database over a recent 9 year period. Records of children (age < 20 years) were reviewed from 2003, 2006, 2009, and 2012) and multivariable logistic regression was used to assess risk factors and adjust for patient and hospital characteristics.

Results: Total annual hospitalization rates with VOC were variable, ranging from 26.4 per 100,000 children in 2009 to 29.5 in 2012. Multivariable logistic regression analysis showed children age 5-9 years had 2.38 times higher odds of developing ACS than children age 15-19 (95%CI, 2.22 to 2.56). Comorbidity of asthma was a risk factor for ACS (OR, 1.44; 95%CI, 1.36 to 1.53). ACS was as common in Hb SC patients hospitalized for VOC as those with SS.

Conclusion: These data demonstrate that despite increasing use of hydroxyurea in children shown in other reports, there has been no reduction in hospitalization rate for pain, and even young children are at risk for ACS. Though de novo ACS is less common in Hb SC, vigilance and prophylactic measures are required for those hospitalized for pain. Further studies are needed to assess the efficacy of prophylactic interventions including bronchodilator therapy for asthmatics hospitalized for VOC.


Miller: Pfizer Pharmaceutical: Research Funding.

Author notes


Asterisk with author names denotes non-ASH members.

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