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.

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