| Revue: | Jornal brasileiro de patologia e medicina laboratorial |
| Base de datos: | PERIÓDICA |
| Número de sistema: | 000297123 |
| ISSN: | 1676-2444 |
| Autores: | Naoum, Paulo Cesar1 Souza, Patricia Caetano de |
| Instituciones: | 1Universidade Estadual Paulista "Julio de Mesquita Filho", Sao Jose do Rio Preto, Sao Paulo. Brasil |
| Año: | 2004 |
| Periodo: | Ago |
| Volumen: | 40 |
| Número: | 4 |
| Paginación: | 249-259 |
| País: | Brasil |
| Idioma: | Portugués |
| Tipo de documento: | Artículo |
| Enfoque: | Caso clínico, analítico |
| Resumen en inglés | Sickle cell disease is a generic term used to determine a group of genetic alterations characterized by a dominance of Hb S. The main genotypes which compose the sickle cell disease group are as follows: SS, SF (S/beta0 thalassemia and S/Hereditary Persistence of Fetal Hemoglobin or HPFH), SFA (S/beta+ thalassemia), SC, SD and SH (S/alpha thalassemia). This study analyzes the products resulting from the oxidization of hemoglobin, identified by the methemoglobin concentration and by red blood cells with Heinz bodies, in two sickle cell genotypes (SS and S/beta0 thalassemia) and in the sickle cell trait (AS) compared with the normal genotype (AA). Analysis of the products resulting from hemoglobin oxidative damage, characterized by an increase in the mean levels of methemoglobin and of the number of red blood cells with Heinz bodies, which are directly related to the increase in the Hb S concentration. Thus, oxidative damage of hemoglobin diminishes among the studied genotypes in the following manner: SS>SF>AS>AA. It is important to stress that these results indicate that the simple presence of Hb S in the red blood cell, as in the AS genotype, is capable of increasing the methemoglobin concentration in 52.62% of the assessed samples and inducing the precipitation of Heinz bodies in 73.68% of cases. Elucidation of the oxidative and reductive processes of the studied hemoglobins is presented in the paper. Highlighted among the presented results is the identification, by means of alkaline agarose gel electrophoresis, of the free alpha globin fraction in all SF genotype samples originating from Hb S/beta0 thalassemia individuals. A hypothesis to explain the origin of free alpha globin, especially in the S/beta0 thalassemia genotype is proposed, as is the importance of its identification in the laboratorial diagnosis of S/beta0 thalassemia |
| Resumen en portugués | Explicações referentes aos processos oxidativos e redutores das hemoglobinas estudadas são apresentados no texto. Destaca-se, entre os resultados apresentados, a identificação por meio de eletroforese em agarose alcalina da fração de globina alfa-livre em todas as amostras do genótipo SF provenientes de pessoas com Hb S/b0 talassemia. É proposto um esquema para explicar a origem da globina alfa-livre, especialmente para o genótipo S/b0 talassemia, e a importância da sua identificação no diagnóstico laboratorial de Hb S/b0 talassemia |
| Disciplinas: | Medicina |
| Palabras clave: | Diagnóstico, Hematología, Anemia falciforme, Daño oxidativo, Hbs, Metemoglobina, Cuerpos de Heinz, Hemoglobina |
| Keyword: | Medicine, Diagnosis, Hematology, Sickle cell disease, Oxidative damage, Hbs, Methemoglobin, Heinz bodies, Hemoglobin |
| Texte intégral: | Texto completo (Ver HTML) |
