Central nervous system events in children with sickle cell disease presenting acutely with headache

Patrick C Hines1, Therese P McKnight, Wendy Seto

  • 1Division of Hematology, Children's Hospital of Philadelphia, University of Pennsylvania School of Medicine, Philadelphia, PA 19104, USA.

Insights

Acute headaches are common in children with sickle cell disease (SCD-SS), with a higher risk of central nervous system (CNS) events. Specific symptoms and elevated platelets may indicate serious events, warranting imaging.

Area of Science:

  • Pediatric Neurology
  • Hematology
  • Sickle Cell Disease Research

Background:

  • Acute headache is a frequent reason for emergency care in children.
  • Sickle cell disease (SCD-SS) is associated with various neurological complications.
  • Identifying risk factors for central nervous system (CNS) events in pediatric SCD-SS is crucial.

Purpose of the Study:

  • To determine the incidence of acute care visits for headache in children with SCD-SS.
  • To identify risk factors associated with acute CNS events in this population.
  • To evaluate the diagnostic yield of neuroimaging for CNS events in pediatric SCD-SS.

Main Methods:

  • Retrospective cohort study analyzing acute care visits for headache in children with SCD-SS.
  • Calculation of prevalence for headache visits, neuroimaging, and acute CNS events.
  • Assessment of clinical and laboratory variables to identify risk factors.

Main Results:

  • Headache accounted for 3.8% of acute care visits in children with SCD-SS.
  • Acute CNS events occurred in 6.9% of headache visits.
  • Neuroimaging revealed acute CNS events in 16.3% of studies, with older age, prior neurological events, symptoms, focal findings, and elevated platelets being significant risk factors.

Conclusions:

  • Acute headaches in pediatric SCD-SS are more commonly linked to acute CNS events than in the general pediatric population.
  • Children with a history of stroke, TIA, seizures, neurological symptoms, focal exam findings, or elevated platelets require confirmatory imaging.
  • Further prospective studies are needed to determine appropriate workup strategies for other children presenting with headache.
Abstract

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