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Published on: February 19, 2017
[Sickle cell anemia: study of the pediatric mortality in Ile de France from 1985 to 1992]
C Thomas1, S Lemerle, F Bernaudin
1Service de pédiatrie, centre hospitalier intercommunal, Créteil, France.
Insights
Infections are the primary cause of death in children with sickle cell anemia in France, even with preventative measures. New treatments focus on patient comfort and reducing disability risks.
Area of Science:
- Hematology
- Pediatric Medicine
- Public Health
Context:
- Sickle cell anemia (SCA) presents a growing health concern in France.
- Acute episodes significantly increase mortality risk, particularly in infants.
- Retrospective analysis of pediatric deaths in the Ile-de-France region.
Purpose:
- To investigate the causes of mortality in children diagnosed with sickle cell disease.
- To analyze the demographic and clinical characteristics of deceased pediatric SCA patients.
- To determine the mortality rate in a defined pediatric SCA cohort.
Summary:
- Infection, particularly Streptococcus pneumoniae, was the leading cause of death in 26 pediatric sickle cell disease patients (1985-1992).
- Other significant causes included splenic sequestration crisis and vaso-occlusive crisis leading to cerebral infarction.
- The calculated mortality rate was 0.29% person/year for patients followed since 1987.
Impact:
- Highlights the persistent threat of infection in pediatric sickle cell disease management.
- Informs the development of new therapeutic strategies prioritizing quality of life and minimizing long-term complications.
- Emphasizes the need for continued vigilance and research in managing sickle cell anemia in pediatric populations.
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 death 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.
