Predictors of Acute Chest Syndrome Following Vaso-Occlusive Crisis in Pediatric Sickle Cell Disease

Read full text
Authors: Narcisse Elenga, Noelis Thomas Boizan, Emmanuel Irakoze, Mody Diop, Gabriel Bafunyembaka
Cited by: 1
References: 18

Abstract

Background/Objectives: Acute chest syndrome (ACS) is a frequent and potentially life-threatening complication of sickle cell disease (SCD) that often develops during hospitalization for vaso-occlusive crisis (VOC). The early identification of pediatric patients at risk remains challenging, particularly in high-prevalence settings. This study aimed to identify predictors of acute chest syndrome following vaso-occlusive crisis in children with SCD. Methods: We conducted a retrospective cohort study of children and adolescents (≤18 years) with confirmed SCD admitted for VOC to Cayenne Hospital Center, French Guiana, between January 2014 and September 2024. ACS that occurred during hospitalization or within 7 days of admission was recorded. Multivariable logistic regression was used to identify independent predictors, and model performance was assessed using receiver operating characteristic (ROC) analysis. Results: Among the 825 VOC episodes in 190 patients, 239 (29%) were complicated by ACS. Independent ACS predictors were thoracic or abdominal pain at presentation (adjusted odds ratio [aOR] 14, 95% CI 6–32, p < 0.001), prior history of ACS (aOR 7.4, 95% CI 4.5–12.1), and Hb SS or Sβ0 genotype (aOR 1.8, 95% CI 1.2–2.4), age > 10 years (aOR 1.6, 95% CI 1.1–2.4), male sex (aOR 1.6, 95% CI 1.1–2.4), Hydroxyurea treatment was associated with a higher risk of acute chest syndrome (aOR 8.7, 95% CI 5.2–14.5), likely reflecting greater baseline disease severity among treated patients. The probability threshold maximizing the Youden index was 0.67, corresponding to a Youden index of 0.56. At this threshold, the model had a sensitivity of 61%, a specificity of 95%, a positive predictive value of 85%, and a negative predictive value of 86%. The apparent area under the receiver operating characteristic curve was 0.87 (95% CI, 0.83–0.89). The receiver operating characteristic analysis yielded an area under the curve of 0.87, indicating good apparent discrimination. Conclusions: These findings support targeted monitoring and early preventive strategies during pediatric VOC admissions.

Fields of Study & Subjects

Hemoglobinopathies and Related Disorders Blood groups and transfusion Folate and B Vitamins Research

Publication details

Journal / source
Diagnostics
Publisher
MDPI AG
Volume
16
Issue
12
Pages
1875–1875
Publication date
16 June 2026
Language
EN