Death of children with sickle cell anemia from 1985 to 1992 in Ile-de-France.

Background.- Sickle cell anemia is now relatively frequent in France. Its clinical course is punctuated by acute episodes that threaten the life, specially during the first year of life. Population and methods.- The files of 26 children (14 boys, 12 girls) dead from sickle cell disease between 1985 and 1992 were retrospectively studied. These files concerned patients from the Ile-de-France area. Results.- Their mean age at time of diagnosis was 13.7 months (0 to 3 years 4 months); mean age was 5.5 years at time of death. Infection was the cause of death in 15 patients (S pneumoniae in eight patients despite prophylactic penicillin and pneumococcal vaccine in the majority of them). Splenic sequestration crisis was the cause of deauth in three patients less than 5 years of age; vasocclusive crisis resulting in cerebral infarction was the cause in three patients aged 7.5 to 13 years. Mortality calculated from those patients followed since 1987 (total: 2,063 years) was 0.29% person/year. Conclusion.- Comfort of patients and risk of permanent disability become the main factors of choice for new treatments such as chemotherapy or bone marrow transplantation.