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  1. Improved survival in homozygous sickle cell disease : lessons from a cohort study.

    Article - En anglais

    Objective

    To examine whether simple interventions in a sickle cell clinic improve survival in sickle cell disease.

    Design-Survival curve analysis and hazard ratios in a cohort study followed from birth.

    Setting-MRC Laboratories (Jamaica) at the University of the West Indies, and Victoria Jubilee Hospital, Kingston, Jamaica.

    Subjects-315 patients with homozygous sickle cell disease detected during the screening of 100000 consecutive non-operative deliveries between June 1973 and December 1981 at the main government maternity hospital, Kingston, Jamaica.

    Interventions-Prophylactic penicillin to prevent pneumococcal septicaemia, parental education in early diagnosis of acute splenic sequestration, close monitoring in sickle cell clinic.

    Main outcome measures-Survival

    Results

    Survival appeared to improve, the log rank test for trend comparing the first, second, and last third of the study reaching borderline significance (P=0.05).

    Combined deaths from acute splenic sequestration and pneumococcal septicaemia-meningitis declined significantly (test for trend, P=0.02).

    Conclusion-Early diagnosis and simple prophylactic measures significantly reduce deaths associated with homozygous sickle cell disease.

    Mots-clés Pascal : Prévention, Survie, Homme, Anémie hématie falciforme, Hémopathie, Hémoglobinopathie, Maladie héréditaire, Anémie hémolytique

    Mots-clés Pascal anglais : Prevention, Survival, Human, Sickle cell anemia, Hemopathy, Hemoglobinopathy, Genetic disease, Hemolytic anemia

    Logo du centre Notice produite par :
    Inist-CNRS - Institut de l'Information Scientifique et Technique

    Cote : 96-0077610

    Code Inist : 002B30A03B. Création : 199608.