Factors Associated with Overt Stroke in Children and Adolescents with Sickle Cell Disease: A Retrospective Cohort

Wolney de Oliveira Taques1,2,3, Gabriele Curvo Bett2, Bárbara Lucia Barbosa de Moraes2

  • 1Júlio Müller University Hospital/EBSERH, Teaching and Research Management, Federal University of Mato Grosso - UFMT, Cuiabá, Brazil.

Hemoglobin
|January 24, 2024
PubMed

Insights

Children with sickle cell disease (SCD) experiencing frequent dactylitis or acute chest syndrome (ACS)/pneumonia may face higher stroke risks. Increased reticulocyte count and red blood cell distribution width (RDW) also correlate with stroke occurrence in pediatric SCD patients.

Area of Science:

  • Pediatric Hematology
  • Neurology
  • Vascular Medicine

Background:

  • Sickle cell disease (SCD) is a genetic blood disorder characterized by vaso-occlusive phenomena, leading to significant complications, notably stroke.
  • Overt stroke is a devastating complication in children and adolescents with SCD, necessitating identification of predictive factors for early intervention.

Purpose of the Study:

  • To investigate the clinical and laboratory characteristics of pediatric patients with SCD.
  • To identify factors associated with the incidence of overt stroke in this cohort.

Main Methods:

  • Retrospective cohort study involving 120 children and adolescents with SCD.
  • Data collected from patient medical records, including demographic, clinical, and laboratory parameters.
  • Survival analysis used to compare characteristics between patients with and without overt stroke.

Main Results:

  • The incidence density of overt stroke was 0.94 cases per 100 patient-years.
  • A history of two or more dactylitis episodes and three or more acute chest syndrome (ACS)/pneumonia episodes were associated with overt stroke.
  • Increased reticulocyte count and red blood cell distribution width (RDW) were also linked to overt stroke occurrence.

Conclusions:

  • Frequent dactylitis, ACS/pneumonia, elevated RDW, and reticulocytosis are associated with overt stroke in pediatric SCD patients.
  • These findings highlight potential biomarkers for stroke risk stratification in children and adolescents with SCD.
  • Further research with larger cohorts and higher stroke incidence is recommended to validate these associations.