Exchange blood transfusion compared with simple transfusion for first overt stroke is associated with a lower risk of subsequent stroke: a retrospective cohort study of 137 children with sickle cell anemia.

A retrospective cohort study of children with sickle cell anemia (SCA) and strokes was used to test the hypothesis that exchange transfusion at the time of stroke presentation more effectively prevents second strokes than does simple transfusion. Children receiving simple transfusion had a 5-fold greater relative risk (95% confidence interval = 1.3 to 18.6) of second stroke than those receiving exchange transfusion.

Rapidly fatal acute bacterial myocarditis in a nonneutropenic child with acute lymphoblastic leukemia in remission.

The authors report a fatal case of acute bacterial myocarditis in a nonneutropenic child with acute lymphoblastic leukemia. She was admitted to the hospital with a urinary tract infection resulting from and remained persistently febrile despite resolution of the infection. On hospital day 4 signs of acute cardiac failure developed. Despite aggressive resuscitation measures, she died. Pathologic examination revealed the cause of death to be bacterial myocarditis. In addition, she was found to have a generalized decrease in her serum immunoglobulin levels. Acute bacterial myocarditis in patients with malignancy has been rarely reported. The rapid clinical deterioration and death in the patient in this report is particularly interesting.

Risk of recurrent stroke in children with sickle cell disease receiving blood transfusion therapy for at least five years after initial stroke.

OBJECTIVE: To test the hypothesis that children with sickle cell disease (SCD) who have an initial stroke temporally unrelated to another medical event are at higher risk for recurrent stroke than are children who had strokes temporally related to medical events. METHODS: A retrospective cohort study of children with SCD and stroke who received regularly scheduled blood transfusions for a minimum of 5 years was conducted. Medical records were examined for the documentation of antecedent or concurrent medical events (hypertension, acute chest syndrome, aplastic crisis, fever associated with infection, exchange transfusion) associated with physician contact within 14 days before the initial stroke. RESULTS: A total of 137 pediatric patients from 14 centers were studied. Mean age at first stroke was 6.3 years (1.4 to 14.0 years) with mean follow-up of 10.1 years (5 to 24 years). Thirty-one (22%) patients had a second stroke (2.2 per 100 patient years); 26 patients had an identified medical or concurrent event associated with their initial stroke. None of these patients had recurrent stroke 2 or more years after the initial event. The remaining 111 patients had an ongoing risk of recurrent stroke (1.9 per 100 patient-years) despite long-term transfusions (P =.038). CONCLUSIONS: The absence of an antecedent or concurrent medical event associated with an initial stroke is a major risk factor for subsequent stroke while receiving regular transfusions.