RESUMO
Foi feita a revisão das complicações agudas da doença falciforme, ou crises, com sugestões de condutas, baseadas na literatura
Assuntos
Humanos , Masculino , Feminino , Anemia Falciforme , Anemia Hemolítica/classificação , Anemia Hemolítica/complicações , Anemia Hemolítica/terapiaAssuntos
Anemia Hemolítica , Anemia Hemolítica/classificação , Anemia Hemolítica/diagnóstico , Anemia Hemolítica/epidemiologia , Anemia Hemolítica/etiologia , Anemia Hemolítica/terapia , Anemia Hemolítica Autoimune/classificação , Anemia Hemolítica Autoimune/etiologia , Anemia Hemolítica Autoimune/imunologia , Anemia Hemolítica Autoimune/terapia , Autoanticorpos/imunologia , Proteínas Sanguíneas/deficiência , Proteínas Sanguíneas/genética , Membrana Eritrocítica/ultraestrutura , Deficiência de Glucosefosfato Desidrogenase/complicações , Humanos , IncidênciaAssuntos
Anemia Hemolítica/sangue , Bilirrubina/sangue , Adolescente , Adulto , Idoso , Anemia Hemolítica/classificação , Anemia Hemolítica/complicações , Colestase Extra-Hepática/diagnóstico , Colestase Extra-Hepática/etiologia , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Prognóstico , Estudos RetrospectivosRESUMO
A review is made of recent developments in the study of the hereditary haemolytic syndromes. Current concepts in the pathogenesis of haemolysis are briefly discussed and the various intrinsic haemolytic disorders classified, with particular reference to anaemias due to red cell defects. The hereditary haemolytic syndromes are discussed in detail, as regards to both their genetic interrelationship and the recent demonstration of abnormal haemoglobin moieties in sickle cell trait and anaemia, and in haemoglobin C. disease. Reference is made to the recent application of paper electrophoretic analysis to the identification of these abnormal haemoglobins. Whilst hereditary sphercytosis and the thalassaemia syndromes are but rarely seen in the Caribbean area, the incidence of sickle cell trait is noted to vary between 5-12 percent of the mixed populations. Haemoglobin C. trait has been found to occur in 2 per cent of North American Negroes, so that these two anomalies alone or together may be present in a significant proportion of persons. Emphasis is placed on the potentiating effect which results from the linking of the dissimilar genes responsible for the hereditary haemolytic syndromes. The simultaneous occurrence of any one of these together with that determining the sickle cell trait is the probable cause of the so-called "mild" anaemia found to occur in those heterozygous children of whom one parent fails to show the sickling trait. It is stressed that while the hereditary haemolytic syndromes have many feature in common, the actual cause of red cell destruction varies with the shape to which the erythrocyte is ultimately changed (AU)