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 d
isease 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 pe
nicillin and pneumococcal vaccine in the majority of them). Splenic se
questration 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 calcul
ated from those patients followed since 1987 (total: 2,063 years) was
0.29% person/year. Conclusion.- Comfort of patients and risk of perman
ent disability become the main factors of choice for new treatments su
ch as chemotherapy or bone marrow transplantation.