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Continuous Manual Exchange Transfusion for Patients with Sickle Cell Disease: An Efficient Method to Avoid Iron Overload
Published on: March 14, 2017
[Sickle cell disease and cerebrovascular stroke: a preventable event]
Ana Sofia Simões1, Pedro Garcia, Isabel Fernandes
1Unidade de Cuidados Intensivos Pediátricos, Hospital Dona Estefânia, Lisboa, Portugal.
Acta Medica Portuguesa
|April 24, 2012
Summary
Sickle cell disease can cause early-onset stroke in children, even presenting as the first symptom. Early screening for sickle cell disease is crucial for prevention.
Area of Science:
- Pediatric Neurology
- Hematology
- Genetics
Background:
- Cerebrovascular stroke in children often has an identifiable cause, with sickle cell disease being most common in Black children.
- This case highlights a preventable aspect of the disease's natural history.
Observation:
- A 27-month-old Black infant presented with acute focal neurological deficits.
- Brain imaging revealed extensive acute infarction and prior silent cerebral infarcts.
- Blood tests showed normocytic anemia, spontaneous red blood cell sickling, and 87% hemoglobin S.
Findings:
- The infant was diagnosed with sickle cell disease presenting as a stroke.
- Exchange transfusion was administered as a treatment.
Implications:
- Stroke can be an early presenting sign of sickle cell disease in young children.
- Widespread antenatal hemoglobinopathy screening and evaluation of neonatal screening programs for sickle cell disease are recommended.
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