RESUMO
Sickle cell disease is enormously variable in its expression and outcome. In addition to this intrinsic variablity are the problems of symptomatic selection biasing observations towards the sever end of a wide clinical spectrum and a truly changing natural history as a result of better management. Against this background, there was a need for a description of the disease in a truly representative sample of patients and this objective has been approached in the Jamaican Cohort Study of Sickle Cell Disease. Initiated in 1973, this study is based on all cases of sickle cell disease detected among 100,000 consecutive normal deliveries in Kingston, Jamaica. All affected children as well as age matched normal controls have been followed prospectively and are currently ages 11 to 19 years. The following review is based on lessons learnt from this cohort study. It is not intended to be a comprehensive survey of knowledge of sickle cell disease and does not address major contributions from studies elsewhere. In some ways, therefore, the review may appear unbalanced because of this specific objective. However, a great deal has been learnt about the evolution of the abnormal haematology of sickle cell disease and its relationship to clinical features. The causes of early mortality in sickle cell disease in Jamaica are described and the major complications such as acute splenic sequestration, pneumococcal septicaemia, aplastic crisis, hypersplenism, and acute chest syndrome have been addressed with varying success. Overall survival to the age of 19 years has been 75 percent and it is planned that the study should continue to define the problems of late adolescence and early adult life (AU)
Assuntos
Humanos , Masculino , Feminino , Anemia Falciforme/terapia , Anemia Aplástica/etiologia , Anemia Falciforme/sangue , Anemia Falciforme/complicações , Anemia Falciforme/mortalidade , Causas de Morte , Transtornos Cerebrovasculares/etiologia , Colelitíase/epidemiologia , Colelitíase/etiologia , Estudos de Coortes , Deficiência de Ácido Fólico/etiologia , Transtornos do Crescimento/etiologia , Incidência , Infecções/complicações , Jamaica/epidemiologia , Infecções por Parvoviridae/complicações , Estudos Prospectivos , Doenças Retinianas/etiologia , Esplenopatias/etiologia , Esplenopatias/mortalidade , Taxa de Sobrevida , Doenças Torácicas/etiologia , Doenças Torácicas/mortalidadeRESUMO
The problem of single intrathoracic opacities is discussed. Intrathoracic opacities which are not diagnosed and treated early have a bad prognosis. The method of approach to diagnosis, based on anatomical position, shape and density of the opacity is advanced. The place of ancillary investigation in diagnosis is recalled. It is concluded that thoracotomy should be more frequently advocated both for confirming diagnosis and for its value in very early treatment (AU)
