RESUMO
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 100,000 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 (AU)
Assuntos
Adolescente , Criança , Pré-Escolar , Humanos , Lactente , Recém-Nascido , Anemia Falciforme/mortalidade , Anemia Falciforme/genética , Anemia Falciforme/terapia , Estudos de Coortes , Homozigoto , Jamaica/epidemiologia , Meningite/mortalidade , Meningite/prevenção & controle , Pais/educação , Penicilinas/uso terapêutico , Infecções Pneumocócicas/mortalidade , Infecções Pneumocócicas/prevenção & controle , Modelos de Riscos Proporcionais , Sepse/mortalidade , Sepse/prevenção & controle , Esplenopatias/diagnóstico , Esplenopatias/mortalidade , Taxa de SobrevidaRESUMO
Bacterial cultures of leg ulcers from 90 patients with SS disease demonstrated 9 with Corynebacterium diptheriae. Follow-up swabs revealed a further 4 cases. All strains were of the gravis variety and 9 of the 13 isolates were toxigenic. The affected ulcers were not remarkable in appearance and no patient was clinically toxic. A variety of other organisms were isolated from the wound, most commonly Pseudomonas aeruginosa. Carriage of C. diptheriae tended to be brief. The significance and therapy of cutaneous diptheria are reviewed (AU)
Assuntos
Humanos , Anemia Falciforme/microbiologia , Úlcera da Perna/microbiologia , Corynebacterium diphtheriae/isolamento & purificação , JamaicaRESUMO
In Jamaican children with homozygous sickle cell (SS) disease diagnosed at birth two-year survival was 87 percent, compared with 95 percent in children with sickle cell-haemoglobin C (SC) disease, and 99 percent in normal controls. Death among those with SS disease occurred most often between the ages of 6 and 12 months. Principal causes were acute splenic sequestration and pneumococcal infection. Neonatal diagnosis of haemoglobinopathies must be followed by close observation if mortality is to be reduced by early diagnosis and treatment of these complications. (AU)
