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Published on: November 5, 2019
Predictors of Acute Chest Syndrome Following Vaso-Occlusive Crisis in Pediatric Sickle Cell Disease
Narcisse Elenga1, Noelis Thomas Boizan1, Emmanuel Irakoze1
1Sickle Cell Reference Center, University Hospital of French Guiana, 3 Avenue Alexis Blaise, Cayenne 97300, French Guiana.
Insights
Identifying acute chest syndrome (ACS) in children with sickle cell disease (SCD) during vaso-occlusive crisis (VOC) is crucial. Thoracic/abdominal pain, prior ACS history, and specific genotypes predict ACS risk, enabling targeted interventions.
Area of Science:
- Pediatric Hematology
- Sickle Cell Disease Research
- Pulmonary Complications in SCD
Background:
- Acute Chest Syndrome (ACS) is a severe complication of Sickle Cell Disease (SCD), often occurring during Vaso-Oclusive Crisis (VOC).
- Early identification of pediatric patients at risk for ACS remains a significant clinical challenge, especially in high-prevalence areas.
- Predicting ACS development post-VOC is essential for timely intervention and improved patient outcomes.
Purpose of the Study:
- To identify independent predictors of Acute Chest Syndrome (ACS) in pediatric patients with Sickle Cell Disease (SCD) admitted for Vaso-Oclusive Crisis (VOC).
- To develop a predictive model for ACS risk assessment in this vulnerable population.
Main Methods:
- Retrospective cohort study of pediatric SCD patients (≤18 years) admitted for VOC.
- Data collected on clinical presentation, history, genotype, and treatment, including Hydroxyurea.
- Multivariable logistic regression and Receiver Operating Characteristic (ROC) analysis were used to identify predictors and assess model performance.
Main Results:
- ACS complicated 29% of 825 VOC episodes.
- Key predictors identified: thoracic/abdominal pain at presentation (aOR 14), prior ACS history (aOR 7.4), Hb SS/Sβ0 genotype (aOR 1.8), age >10 years (aOR 1.6), male sex (aOR 1.6), and Hydroxyurea treatment (aOR 8.7).
- The predictive model demonstrated good discrimination (AUC 0.87) with high specificity (95%) and positive predictive value (85%).
Conclusions:
- Thoracic/abdominal pain, prior ACS, specific genotypes, older age, male sex, and Hydroxyurea use are significant predictors of ACS in pediatric SCD patients during VOC.
- These findings support the implementation of targeted monitoring and early preventive strategies for high-risk children.
- The developed predictive model can aid clinicians in risk stratification and management during VOC admissions.
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.
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