Data/hora: 09/06/2024 04:13:26

Provedor de dados: | Brazilian Journal of Medical and Biological Research
País: | Brazil
Título: | Effect of hydroxyurea on G gamma chain fetal hemoglobin synthesis by sickle-cell disease patients
Autores: | Teixeira,S.M.; Cortellazzi,L.C.; Grotto,H.Z.W.
Data: | 2003-10-01
Ano: | 2003
Palavras-chave: | Gamma chain; Fetal hemoglobin; Hydroxyurea; Sickle-cell disease.
Resumo: | Hydroxyurea is used for sickle-cell disease patients in order to increase fetal hemoglobin synthesis and consequently decrease the severity of pain episodes. Fetal hemoglobin, which is formed by gamma-globin chains A and G, is present in a constant composition throughout fetal development: about 75% of Ggamma and 25% of Agamma. In contrast, adult red cells contain about 40% of Ggamma and 60% of Agamma. In the present study, we analyzed the effect of hydroxyurea induction on the gamma chain composition of fetal hemoglobin in 31 sickle-cell disease patients treated with hydroxyurea. The control group was composed of 30 sickle-cell disease patients not treated with hydroxyurea in clinical steady state. The patients were older than 13 years and were not matched for age. All patients were seen at Hemocentro/UNICAMP and Boldrini Infantile Center, Campinas, SP, Brazil. The levels of total hemoglobin were significantly higher in patients treated with hydroxyurea (mean ± SD, 9.6 ± 2.16 g/dl) than in untreated patients (8.07 ± 0.91 g/dl). Fetal hemoglobin levels were also higher in treated patients (14.16 ± 8.31%) than in untreated patients (8.8 ± 4.09%), as was the Ggamma/Agamma ratio (1.45 ± 0.78 vs 0.98 ± 0.4, P &lt; 0.005). The increase in the Ggamma/Agamma ratio in patients treated with hydroxyurea suggests the prevalence of a pattern of fetal hemoglobin synthesis, whereas patients not treated with hydroxyurea maintain the adult pattern of fetal hemoglobin synthesis. Because no correlation was observed between the Ggamma/Agamma ratio and total hemoglobin or fetal hemoglobin levels, the increase in Ggamma chain synthesis may not imply a higher production of hemoglobin.
Tipo: | Info:eu-repo/semantics/other
Idioma: | Inglês
Identificador: | http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0100-879X2003001000002
Editor: | Associação Brasileira de Divulgação Científica
Relação: | 10.1590/S0100-879X2003001000002
Formato: | text/html
Fonte: | Brazilian Journal of Medical and Biological Research  v.36 n.10 2003
Fonte: | info:eu-repo/semantics/openAccess