SICKLE-CELL-ANEMIA IN CHILDREN

Sickle cell anemia is being increasingly encountered in France. Because patients are at risk for severe complications, early diagnosis is essential. Hemoglobin electrophoresis should therefore be performed routinely in every high risk child (i.e., from Africa or the Antilles) under 5 years of age. Education of the family and preventive steps (daily oral penicillin, anti-pneumococcal immunization) have radically improved the prognosis. However, because of the frequency of painful sickle cell crises, which usually occur in an unpredictable fashion, the disease is severely disabling in every case. Bone marrow transplants and therapies capable of increasing fetal hemoglobin are promising new approaches.