Incident Stroke in Pediatric Sickle Cell Anemia Despite Overall Improved Transcranial Doppler Velocity in a Ugandan

Bill Wambaka1, Amiirah Mpungu1, Vincent Mboizi1

  • 1Global Health Uganda, Kampala, Uganda.

PubMed

Insights

Transcranial Doppler ultrasound (TCD) screening for stroke risk in children with sickle cell anemia (SCA) may be less effective in Uganda. Additional factors like anemia and low fetal hemoglobin (HbF) also contribute to stroke risk.

Area of Science:

  • Pediatric Neurology
  • Hematology
  • Global Health

Background:

  • Stroke is a major complication of sickle cell anemia (SCA) in children.
  • Transcranial Doppler (TCD) ultrasound is used for primary stroke prevention in high-resource settings.
  • Identifying stroke risk factors in low-resource regions like Uganda is crucial.

Purpose of the Study:

  • To evaluate stroke risk factors in Ugandan children with SCA.
  • To assess the effectiveness of TCD screening in this population.
  • To investigate the role of hydroxyurea (HU) therapy in brain protection.

Main Methods:

  • A 30-month open-label, single-arm trial of dose-escalated hydroxyurea in Ugandan children (3-9 years) with SCA.
  • Included clinical assessments, TCD, and laboratory tests.
  • Prospective monitoring for stroke events.

Main Results:

  • 15.7% of 264 children had abnormal TCD at enrollment.
  • Four children without abnormal TCD developed stroke within 16 months.
  • Stroke risk factors included low fetal hemoglobin (HbF ≤3.1%), hypoxemia, and severe anemia.

Conclusions:

  • TCD screening alone may not fully identify high stroke risk in Uganda.
  • Anemia, low HbF, hypoxemia, and infections are significant stroke contributors.
  • Primary stroke prevention from early childhood and continuous hydroxyurea therapy are recommended.
Abstract

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