title: Primary stroke in a woman with sickle cell anemia responsive to hydroxyurea therapy.
abstract: The most common cause of stroke in children with sickle cell anemia is infarction due to ischemia. In adults, however, stroke is most commonly hemorrhagic in nature. Other causes of stroke in patients with sickle cell disease are very rare. In this short communication, we describe a woman with sickle cell anemia responsive to hydroxyurea (HU) therapy who had primary stroke due to paradoxical embolization caused by a large atrial septal defect. Successful management of the stroke included surgical closure of the defect with trans-esophageal echocardiographic guidance. To the best of our knowledge, this is the first patient with sickle cell anemia and stroke due to congenital heart disease who did not require open heart surgery for successful management.
authors.affiliation: Cardeza Foundation for Hematologic Research, Department of Medicine, Jefferson Medical College, Thomas Jefferson University , Philadelphia, Pennsylvania , USA .
authors.forename: Samir K
authors.initials: SK
authors.lastname: Ballas
authors.forename: Ubaldo
authors.initials: U
authors.lastname: Martinez
authors.forename: Michael
authors.initials: M
authors.lastname: Savage
mesh_terms: D000328:Adult; D000755:Anemia, Sickle Cell / Q000150:complications / Q000188:drug therapy / Q000503:physiopathology*; D000986:Antisickling Agents / Q000627:therapeutic use; D002545:Brain Ischemia / Q000209:etiology*; D017548:Echocardiography, Transesophageal; D005260:Female; D006344:Heart Septal Defects, Atrial / Q000150:complications* / Q000000981:diagnostic imaging / Q000601:surgery; D006801:Humans; D006918:Hydroxyurea / Q000627:therapeutic use; D015143:Philadelphia; D020521:Stroke / Q000209:etiology*; D016896:Treatment Outcome
chemical_list: D000986:Antisickling Agents; D006918:Hydroxyurea
keywords: Atrial septal defect; paradoxical embolization; patent foramen ovale; primary stroke; sickle cell anemia; stroke