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

Hemoglobin. 2024 Jan;48(1):39-46. doi: 10.1080/03630269.2023.2301490. Epub 2024 Jan 23.

Abstract

Sickle cell disease (SCD) is associated with a high occurrence of complications due to vaso-occlusive phenomenon such as stroke. This retrospective cohort study aimed to examine the clinical and laboratory characteristics of 120 children and adolescents with SCD and analyze the factors associated with overt stroke incidence. All relevant data were obtained from patient medical records. Survival analysis was used to compare the demographic, clinical, and laboratory characteristics between patients with and those without overt stroke. The patients were 52.5% female with a mean (SD) age of 11.2 (4.3) years. The incidence of overt stroke in this cohort was nine out of 956.7 patient-years, resulting in an incidence density of 0.94 cases/100 patient-years. Reports of greater than or equal to two previous attacks of dactylitis and greater than or equal to three episodes of acute chest syndrome (ACS)/pneumonia were associated with overt stroke and an increase in reticulocyte count and red blood cell distribution width (RDW). In conclusion, a history of a high number of dactylitis, ACS/pneumonia, increased RDW, and reticulocytosis was associated with overt stroke occurrence in children and adolescents with SCD. Future studies with a higher stroke incidence in the evaluated sample are necessary to confirm this hypothesis.

Keywords: Sickle cell disease; risk factors; stroke.

MeSH terms

  • Acute Chest Syndrome* / complications
  • Acute Chest Syndrome* / etiology
  • Adolescent
  • Anemia, Sickle Cell* / complications
  • Anemia, Sickle Cell* / epidemiology
  • Child
  • Female
  • Humans
  • Hydroxyurea
  • Male
  • Pneumonia* / complications
  • Retrospective Studies
  • Stroke* / epidemiology
  • Stroke* / etiology

Substances

  • Hydroxyurea