Clinical Factors and Incidence of Acute Chest Syndrome or Pneumonia Among Children With Sickle Cell Disease Presenting With a Fever: A 17-Year Review

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The objectives of this study were to determine the incidence of acute chest syndrome (ACS) in children with sickle cell disease (SCD) presenting with fever before and after the introduction of the 7-valent pneumococcal conjugate vaccine (PCV7) and to determine clinical factors associated with ACS for a febrile child with SCD.


A retrospective chart review was undertaken for children with SCD from 1993 to 2009 in a single, urban, tertiary-care pediatric center. Clinical and laboratory data for each febrile event for each child with SCD were recorded. We compared incidence of ACS for the 3 PCV7 eras: pre-PCV7, inter-PCV7, and post-PCV7. Univariate analysis and stepwise logistic regression were used to identify clinical factors most associated with ACS in the post-PCV7 era.


Of 2504 febrile events in 466 children with SCD, we found 492 diagnoses of ACS. The incidence of ACS cumulatively decreased over time from 27.0% to 17.4% among febrile children with SCD (P < 0.001), although no change was seen in children younger than 2 years (P = 0.89). Independent predictors of ACS in the post-PCV7 era include history of previous ACS, upper respiratory tract infection symptoms, noncompliance to penicillin, male sex, hypoxemia, an absolute neutrophil count greater than 9 × 109/L, and hemoglobin less than 8.6 g/dL.


The incidence of ACS has decreased over time in febrile children with SCD. No effect was seen in those 2 years or younger. Children with SCD presenting with a fever had higher odds of developing ACS when accompanied by certain clinical, demographic, and laboratory features.

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