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J Pediatr Hematol Oncol. 2018 Jan;40(1):51-55. doi: 10.1097/MPH.0000000000001012.

Recurrent Acute Chest Syndrome in Pediatric Sickle Cell Disease: Clinical Features and Risk Factors.

Author information

1
School of Medicine.
2
Division of Pediatric Hematology/Oncology.
3
Center of Biostatistics and Bioinformatics.
4
Division of Pediatric Pulmonology.
5
Division of Pediatric Critical Care, University of Mississippi Medical Center, Jackson, MS.

Abstract

Acute chest syndrome (ACS) is a common and serious lung complication in sickle cell disease. A retrospective medical chart review was performed over a 6-year period in all pediatric ACS patients to investigate whether factors during the initial hospitalization were associated with recurrent ACS episodes. There were 386 episodes of ACS: 149 had only 1 episode of ACS, and 76 had >1 episode of ACS; 172 (76.4%) had hemoglobin SS, and 39 (17.3%) had hemoglobin SC. The most common presenting features were fever (83%), pain (70%), and cough (61%), which changed with the number of ACS episodes. Children <4 years old were at greatest risk of recurrent ACS (P=0.018). In addition, history of asthma (adjusted incident rate ratio [IRR]=1.52; 95% confidence interval [CI], 1.22-1.98; P<0.0001), shortness of breath (IRR, 1.29; 95% CI, 1.02-1.62; P=0.033), and length of hospital stay (IRR, 1.04; 95% CI, 1.01-1.08; P=0.017) were significantly associated with prospective ACS events. Multiple episodes of ACS are common in sickle cell disease, and certain risk factors during the initial hospitalization are associated with recurrent ACS.

PMID:
29200151
DOI:
10.1097/MPH.0000000000001012
[Indexed for MEDLINE]

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