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

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.

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Journal
Diagnostics
Published
2026-06-16
DOI
https://doi.org/10.3390/diagnostics16121875
Primary Topic
Hemoglobinopathies and Related Disorders
Type
article
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article

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

Narcisse Elenga, Gabriel Bafunyembaka, Mody Diop, Noelis Thomas Boizan et al.
Diagnostics
Hemoglobinopathies and Related Disorders
article

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

Narcisse Elenga, Gabriel Bafunyembaka, Mody Diop, Noelis Thomas Boizan, Emmanuel Irakoze
article en

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.

DiagnosticsVol. 16(12)
University of French Guiana (GF)
Good health and well-being
Openalex Percentile: Top 10%
Hemoglobinopathies and Related Disorders
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