RESUMO
We analyzed the records of 153 Guadeloupean children with sickle cell anemia (SCA), for whom clinical and laboratory data were prospectively collected (mean follow-up duration 8.4 +/- 4.6 yr). Prevalence and age-specific frequencies of acute clinical events were determined and correlations between complications, hematological parameters and potential modulating factors investigated. Painful crisis and acute chest syndrome (ACS) were the two most common complications, affecting 65.4% and 58.8% of the patients, respectively. The frequency of acute anemia was 49.7% (acute splenic sequestration 24.8%; acute aplastic anemia 15.0%). Prevalences of septicemia-meningitis and osteomyelitis were 15.7% and 16.3%, respectively. A higher incidence of infections, painful crises and acute anemia was detected in patients who developed ACS. The well-documented protective effect of HbF level on the overall disease expression was observed with higher HbF level in asymptomatic than in symptomatic patients (17.5% +/- 8% vs. 9.9% +/- 6.4%, P = 0.01) with similar ages and sex ratio. It was also confirmed on ACS and, for the first time, further extended to acute anemic events and septicemia. Besides its effect on hematological parameters, alpha-thalassemia seems to have little impact on the prevalence of complications, as do beta(S)-globin haplotypes. Comparison with other series suggests that the natural history of SCA in Guadeloupe is more similar to that in Jamaica with regard to those reported in Europe and the United States, suggesting a potential impact of environmental factors on the clinical course of the disease.
Assuntos
Anemia Falciforme/complicações , Anemia Falciforme/epidemiologia , Doença Aguda , Fatores Etários , Dor no Peito/etiologia , Pré-Escolar , Feminino , Hemoglobina Fetal/análise , Globinas/genética , Guadalupe/epidemiologia , Humanos , Lactente , Infecções/etiologia , Masculino , Dor/etiologia , Prevalência , Estudos ProspectivosRESUMO
OBJECTIVE: To study the incidence of Kawasaki disease in the population of the French West Indies. METHODS: Fifty-six children where enrolled between January 1, 1995 and December 31, 2000), in this retrospective study in Guadeloupe (French West Indies), according to the diagnostic criteria of the American Heart Association. RESULTS: There were 31 boys and 25 girls. Their mean age at the time of diagnosis was 26.5 +/- 22 months. The mean incidence was 25.4/100 000 children aged under 5, per year. Cardiac involvement was noted in 17.8% of the cases during the first 3 months, with good outcome in all the children followed-up. CONCLUSION: The incidence of Kawasaki disease in Guadeloupe is high. The absence of epidemiological, clinical or biological predictive criteria of cardiac involvement should prompt the early use of immunoglobulins, notably in atypical presentations of the disease.