RESUMO
135 children with homozygous sickle-cell (SS) disease diagnosed at birth have been followed for 1-5 years. Severe bacterial infections were confined to those in whom the spleen was first palpable at or before 1 year of age and were commonest in those in whom the spleen was first palpable at or before age 6 months. Regular follow-up of children with SS disease diagnosed at birth will identify children particularly at risk of severe infections. (Summary)
Assuntos
Humanos , Lactente , Pré-Escolar , Anemia Falciforme/genética , Infecções/etiologia , Esplenomegalia/complicações , Infecções por Escherichia coli/etiologia , Seguimentos , Gastroenterite/etiologia , Haemophilus influenzae , Infecções por Haemophilus/etiologia , Homozigoto , Meningite Pneumocócica/etiologia , Osteomielite/etiologia , Salmonella typhimurium , Infecções por Salmonella/etiologia , Sepse/etiologia , Infecções Estreptocócicas/etiologia , Streptococcus pneumoniaeRESUMO
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)