先天性酶缺乏性高铁血红蛋白血症病例中的相关红细胞酶缺乏及其意义。
Associated red cell enzyme deficiencies and their significance in a case of congenital enzymopenic methemoglobinemia.
作者信息
Das Gupta A, Vaidya M S, Bapat J P, Pavri R S, Baxi A J, Advani S H
出版信息
Acta Haematol. 1980;64(5):285-8. doi: 10.1159/000207286.
Examination of the red cell enzyme profile in a case of congenital methemoglobinemia has shown associated deficiencies of glutathione reductase (GR) and glutathione peroxidase (GSHPx) in addition to NADH-methemoglobin reductase deficiency. Contrary to expectations, GR and GSHPx deficiencies do not seem to have contributed to the methemoglobinemia in this case. The lack of symptoms in spite of a high methemoglobin (Hi) level (35%) appears to be due to the restriction of Hi to a small percentage of red cells.
对一例先天性高铁血红蛋白血症患者的红细胞酶谱检查显示，除了NADH-高铁血红蛋白还原酶缺乏外，还伴有谷胱甘肽还原酶（GR）和谷胱甘肽过氧化物酶（GSHPx）缺乏。与预期相反，在该病例中，GR和GSHPx缺乏似乎并未导致高铁血红蛋白血症。尽管高铁血红蛋白（Hi）水平较高（35%），但患者没有症状，这似乎是由于Hi仅局限于一小部分红细胞。
Zotero 插件