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Sun,Yanbo; Wang,Pingyu; Li,Youjie; Jiao,Fei; Li,Zunling; Ma,Ying; Li,Wei; Xie,Shuyang. |
Methemoglobin (Hb-M) is a rare hemoglobinopathy in China. We hereby report on a family living in Yantai, East China, with congenital cyanosis due to Hb-M mutation. The proband, a 65-year-old female, presented 63% oxygen saturation. Both Hb-M concentration and arterial oxygen saturation remained unchanged, even following intravenous treatment with methylene blue. There was also no change in blood-color (chocolate-brown) after adding 0.1% KCN. A fast-moving band (Hb-X) in hemolysates was found by cellulose acetate electrophoresis, the Hb-X/Hb-A ratio exceeding 10%. GT transition at 131nt of exon 2, although present in one of the α2-globin alleles, was not found in α1-globin alleles as a whole. This mutation leads to the aspartic acid to tyrosine substitution... |
Tipo: Info:eu-repo/semantics/article |
Palavras-chave: Congenital cyanosis; Hemoglobin; Methemoglobinemia; A-globin gene; China. |
Ano: 2010 |
URL: http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1415-47572010000300009 |
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Moraes,Natália Valadares de; Bergamaschi,Mateus Machado; Bianchi,Maria de Lourdes Pires; Bragheto,Juliana Bordinassi; Malfará,Wilson Roberto; Queiroz,Regina Helena Costa. |
Dapsone use is frequently associated to hematological side effects such as methemoglobinemia and hemolytic anemia, which are related to N-hydroxylation mediated by the P450 enzyme system. The aim of the present study was to evaluate the influence of L-arginine supplementation, a precursor for the synthesis of nitric oxide, as single or multiple dose regimens on dapsone-induced methemoglobinemia. Male Wistar rats were treated with L-arginine at 5, 15, 30, 60 and 180 mg/kg doses (p.o., gavage) in single or multiple dose regimens 2 hours prior to dapsone administration (40 mg/kg, i.p.). The effect of the nitric oxide synthase inhibitor L-NAME was investigated by treatment with multiple doses of 30 mg/kg (p.o., gavage) 2 hours before dapsone administration.... |
Tipo: Info:eu-repo/semantics/article |
Palavras-chave: Dapsone; Methemoglobinemia; L-arginine; Nitric oxide; L-NAME. |
Ano: 2012 |
URL: http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1984-82502012000100010 |
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