Sickle cell disease and cerebrovascular stroke: a preventable event.

Ana Sofia Simões, Pedro Garcia, Isabel Fernandes, Lurdes Ventura, Rita Silva, Deolinda Barata

Abstract


About 75% of the children presenting with cerebrovascular stroke have an identified cause. For black children, the most common underlying condition is sickle cell disease. This case report describes a preventable natural history, with an unusual presenting feature.27th month black infant without relevant background, presented with an acute focal neurological deficit. Brain computed tomography scan showed extensive acute infarction, and magnetic resonance also revealed previous silent cerebral infarct. Blood sample showed normocytic anemia and spontaneous sickling of the red blood cells, with 87% hemoglobin S. Exchange transfusion was made.Stroke, as a sickle cell disease complication, can occur at early ages and being the presenting sign. We consider that it would be worth spread the antenatal hemoglobinopathy screening programme, and evaluating the cost-effectiveness of a neonatal screening programme for sickle cell disease in Portugal.

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