Alteraçöes imunológicas em pacientes com anemia falciforme / Immunological disorders in sickle cell disease

Objetivos: Nosso objetivo foi revisar as alteraçöes imunológicas em pacientes com anemia falciforme, englobando as causas da maior susceptibilidade às infecçöes e o papel das moléculas de adesäo, citocinas e inflamaçäo sobre a aderência anormal das hemácias falciformes ao endotélio vascular. Métodos: Os artigos para revisäo foram obtidos a partir dos dados do MEDLINE, onde analisou-se a literatura, nos idiomas inglês e português, sobre anemia falciforme e moléculas de adesäo. Resultados: A freqüência de infecçöes por germes 0encapsulados é alta em pacientes com anemia falciforme, principalmente pelo Streptococcus pneumoniae. Isto se deve ao fato de que as crises vaso-oclusivas recorrentes no baço resultam na atrofia do órgäo, com conseqüente diminuiçäo de sua funçäo, principalmente a de opsonizaçäo. A adesäo anormal dos glóbulos vermelhos falciformes ao endotélio vascular mantém baixos níveis de isquemia tecidual. Conclusäo: Pacientes com anemia falciforme säo mais sensíveis a infecçöes, principalmente pneumocócicas, de modo que há necessidade de profilaxia antibiótica e vacinaçäo anti-pneumocócica. A melhor compreensäo dos defeitos imunológicos que predispöem à inflamaçäo e vasculite constantes, pode ser fundamental na escolha das drogas que devem ser utilizadas para diminuir as complicaçöes vasculares da anemia falciforme

Zinc, copper and iron and their interrelations in the growth of sickle cell patients / Zinc, cobre, hierro y su interrelación con el crecimiento de niños con anemia falciforme

In this study we evaluated the nutritional status of 34 children with sickle cell disease (SS). Results were compared to 9 siblings with sickle cell trait (AS) and 35 eutrophic children who presented normal hemoglobin and normal hemoglobin electrophoresis (AA). All then came from low socieconomic level. Analysis of the groeth velocity curves revelead in SS group, tendency to increase deficit in weight and heightt with age. There was no relation between weight/height (W/H) and height/age (H/A) percentile and hemoglobin levels. There was no significant relation between nutritional status and severity of the disease. SS group showed significant skeletal maturation delay, the same did not occur with the siblings (AS group). Plasma zinc levels were significantly lower in SS group than in As and AA groups In SS group there was some association between lower plasma zinc levels and H/A percentile lower or aqual to 10. Plasma copper levels were significantly greater in SS group than in AS and AA ones, and there was no relation between plasma copper levels and serum ferritin levels. In conclusion, our patients with sickle cell disease showed indexes of malnutrition, iron deficiency, hipercupremia and low plasma zinc levels related to low stature