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Silent infarcts in young children with sickle cell disease

Janet L Kwiatkowski1, Robert A Zimmerman, Avrum N Pollock

  • 1Division of Hematology, The Children's Hospital of Philadelphia, Philadelphia, PA, USA. kwiatkowski@email.chop.edu

British Journal of Haematology
|June 9, 2009
PubMed

Insights

Silent infarcts are common in young children with sickle cell disease (SCD-SS), affecting over 27% of asymptomatic individuals under six. Early detection is crucial as silent infarcts are linked to potential neurocognitive deficits.

Area of Science:

  • Pediatric Neurology
  • Hematology
  • Radiology

Background:

  • Silent infarcts are prevalent in school-aged children with sickle cell disease (SCD-SS) and linked to neurocognitive deficits.
  • The occurrence of silent infarcts in younger children with SCD-SS remains underdefined.

Purpose of the Study:

  • To determine the prevalence of silent infarcts in children with SCD-SS before the age of six.
  • To identify clinical and laboratory factors associated with silent infarcts in this pediatric cohort.

Main Methods:

  • Retrospective analysis of brain MRI/A studies in children with SCD-SS under six years old.
  • Calculation of silent infarct prevalence in asymptomatic children.
  • Comparison of clinical and laboratory parameters between children with and without silent infarcts.

Main Results:

  • 18 out of 65 neurologically asymptomatic children (27.7%) had silent infarcts, with a mean age of 3.7 years.
  • Silent infarcts were associated with cerebral vessel stenosis, lower rates of vaso-occlusive pain and acute chest syndrome, and lower hemoglobin levels.
  • Prevalence in young children mirrors that in older children.

Conclusions:

  • Silent infarcts are a significant finding in young children with SCD-SS, similar in prevalence to older children.
  • Anemia and severe vasculopathy may be identified as risk factors for silent infarcts in children with SCD-SS.

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