Herzog P, Feig S A
Clin Haematol. 1978 Feb;7(1):75-83.
Methaemoglobinaemia is a rare condition in which the haemoglobin iron is in the oxidized or ferric state and cannot reversibly bind oxygen. If severe, this condition leads to hypoxaemia and death. Methaemoglobinaemia may be acquired by exposure to oxidant drugs or chemicals. Alternatively, it may be inherited due either to a haemoglobinopathy, which renders the haem iron more susceptible to oxidation, or to a defect in the erythrocyte's enzymatic capacity to return methaemoglobin to the reduced state. Newborn infants have an increased risk of methaemoglobinaemia due to a normal transient deficiency of methaemoglobin reductase in neonatal erythrocytes and the increased tendency of fetal haemoglobin to assume the met-configuration. The clinical approach to the neonate with methaemoglobinaemia is no different from the approach to older patients. The need for therapy is dictated by the severity of hypoxic symptoms. Since the diagnosis is made simply and the treatment is effective, a high index of clinical suspicion may be potentially life-saving.
高铁血红蛋白血症是一种罕见病症，其中血红蛋白铁处于氧化态或三价铁状态，无法可逆地结合氧气。若病情严重，会导致低氧血症和死亡。高铁血红蛋白血症可能因接触氧化性药物或化学物质而获得。或者，它可能由于血红蛋白病（使血红素铁更易氧化）或红细胞将高铁血红蛋白还原为还原态的酶能力缺陷而遗传。由于新生儿红细胞中高铁血红蛋白还原酶正常短暂缺乏以及胎儿血红蛋白呈现高铁构型的倾向增加，新生儿患高铁血红蛋白血症的风险更高。对患有高铁血红蛋白血症的新生儿的临床处理方法与对年长患者的处理方法并无不同。治疗需求取决于缺氧症状的严重程度。由于诊断简单且治疗有效，高度的临床怀疑可能会挽救生命。
