MRI abnormalities of the brain in one-year-old children with sickle cell anemia

Winfred C Wang1, Steven G Pavlakis, Kathleen J Helton

  • 1Department of Hematology, St. Jude Children's Research Hospital, Memphis, Tennessee, USA. win.wang@stjude.org

Insights

Silent brain infarcts affect 13% of infants with sickle cell anemia (SCA) by age one. Early central nervous system (CNS) evaluation is crucial for timely intervention in children with SCA.

Area of Science:

  • Neurology
  • Pediatrics
  • Radiology

Background:

  • Sickle cell anemia (SCA) can cause central nervous system (CNS) damage, but the onset age is unclear.
  • Infants with SCA underwent brain MRI as part of the BABY HUG trial.

Purpose of the Study:

  • To determine the age of onset of CNS damage in infants with SCA.
  • To identify early indicators of neurological complications in SCA.

Main Methods:

  • Utilized a standardized MRI/MRA protocol across eight centers for infant participants.
  • Correlated imaging findings with clinical data, including age, HbF levels, and transcranial Doppler (TCD) velocity.

Main Results:

  • 13% of infants (23 subjects, average age 13.7 months) had silent brain infarcts on MRI.
  • Infarcts correlated with increased right-sided TCD velocity and lower HbF levels.

Conclusions:

  • Silent brain infarcts are present in infants with SCA as early as one year of age.
  • Early and thorough CNS evaluation is essential for developing timely interventions in pediatric SCA.
Abstract