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Continuous Manual Exchange Transfusion for Patients with Sickle Cell Disease: An Efficient Method to Avoid Iron Overload
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Central nervous system complications of sickle cell disease in children: an overview

Winfred C Wang1

  • 1Department of Hematology/Oncology, St. Jude Children's Research Hospital. Memphis, TN38105, USA. winfred.wang@stjude.org

Child Neuropsychology : a Journal on Normal and Abnormal Development in Childhood and Adolescence
|March 17, 2007
PubMed

Insights

Sickle cell disease frequently causes central nervous system complications like stroke and silent infarcts, impacting school performance. Transcranial Doppler screening identifies high-risk patients for stroke prevention, but treatments remain limited.

Area of Science:

  • Neurology
  • Hematology
  • Pediatrics

Background:

  • Central nervous system (CNS) complications are severe in sickle cell disease (SCD).
  • Overt stroke affects 10% of children with Hemoglobin SS, while silent cerebral infarcts are more common.
  • These neurological issues lead to significant neuropsychological deficits and reduced academic achievement.

Purpose of the Study:

  • To summarize the impact of CNS complications in sickle cell disease.
  • To highlight the role of transcranial Doppler ultrasonography in identifying at-risk individuals.
  • To discuss current strategies and limitations in managing SCD-related CNS issues.

Main Methods:

  • Review of existing literature on CNS complications in sickle cell disease.
  • Analysis of the utility of transcranial Doppler (TCD) screening.
  • Discussion of stroke prevention strategies, including chronic transfusion.

Main Results:

  • Silent cerebral infarcts are more prevalent than overt strokes in SCD.
  • Both stroke types are linked to substantial neuropsychological deficits.
  • Transcranial Doppler screening effectively identifies children at high risk for stroke.
  • Chronic transfusion can prevent stroke in identified high-risk patients.

Conclusions:

  • CNS complications significantly impair cognitive function and school performance in children with SCD.
  • Transcranial Doppler ultrasonography is a valuable tool for stroke risk stratification and prevention.
  • Further research is needed to establish definitive prophylaxis and treatments for the spectrum of CNS complications in sickle cell disease.

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