BACTEREMIA IN CHILDREN WITH SICKLE HEMOGLOBIN-C DISEASE AND SICKLE BETA(-THALASSEMIA - IS PROPHYLACTIC PENICILLIN NECESSARY())

Objective: To characterize the incidence of bacteremia and its potenti al for progression to septicemia in children with sickle hemoglobin C disease and sickle beta(+)-thalassemia to assess the need for penicill in prophylaxis, Study design: Retrospective chart review of the freque ncy and natural history of bloodstream infection in such patients not receiving prophylactic penicillin therapy and followed up in a single institution, Results: During more than 842 patient-years of observatio n in 242 patients with sickle hemoglobin C disease, 15 episodes of bac teremia occurred in nine patients, Septicemia was fatal in one patient . The overall incidence of bacteremia, 1.8 events per 100 patient-year s (95% confidence limits: 0.8, 2.8) in patients with sickle hemoglobin C disease, was similar to that in hematologically normal children. On e episode of bacteremia occurred in a patient with sickle beta(+)-thal assemia. Conclusions: The incidence of bacteremia is not increased in young patients with sickle hemoglobin C disease and sickle beta(+)-tha lassemia. Further, unlike its course in children with sickle cell anem ia, it rarely evolves into life-threatening septicemia, This probably results from the maintenance of relatively intact splenic function dur ing infancy and early childhood in patients with sickle hemoglobin C d isease and sickle beta(+)-thalassemia, Prophylactic penicillin therapy may not be required in these patients.