Mtvarelidze Z G, Kvezereli-Kopadze A N, Kvezereli-Kopadze M N
Georgian Med News. 2006 Jul(136):69-71.
beta-thalassemia is the most common monogenic hereditary blood disease in children. It is also considered to be the regional pathology for Georgia. The influence of iron metabolism disorder on metabolic processes taking place in erythrocyte membrane and their role in pathogenesis of beta-thalassemia, is very important until now. The aim of our research was to study the condition of oxidoreduction processes in RBC membranes on the background of iron metabolism disorder in children with beta-thalassemia. We observed 44 patients with beta-thalassemia aged 0.4-14 years. Iron, ferritin, malon-dialdehyde and catalase were evaluated. The carried out investigation revealed, that oxidoreduction processes in patients with beta-thalassemia, together with iron overload, is one of the factors in promoting further disorder of proliferation and differentiation processes in erythrone system and also in formation of ineffective erythropoiesis. The revealed changes in data of iron metabolism. malon-dialdehyde and catalase showed us the need for correction of this disorder. Pathogenetically there are good reasons to include in the combined treatment beta-thalassemia the membrano-protective preparations (vitamin E, acetylcysteine) together with the hemotransfusion and chelator therapy.
β地中海贫血是儿童中最常见的单基因遗传性血液疾病。它也被认为是格鲁吉亚的地方病。迄今为止，铁代谢紊乱对红细胞膜代谢过程的影响及其在β地中海贫血发病机制中的作用非常重要。我们研究的目的是在β地中海贫血患儿铁代谢紊乱的背景下，研究红细胞膜氧化还原过程的状况。我们观察了44例年龄在0.4至14岁之间的β地中海贫血患者。评估了铁、铁蛋白、丙二醛和过氧化氢酶。所进行的研究表明，β地中海贫血患者的氧化还原过程，连同铁过载，是促进红细胞系统增殖和分化过程进一步紊乱以及无效红细胞生成形成的因素之一。铁代谢、丙二醛和过氧化氢酶数据的变化表明我们需要纠正这种紊乱。从发病机制来看，有充分的理由在β地中海贫血的联合治疗中，将膜保护制剂（维生素E、乙酰半胱氨酸）与输血和螯合疗法一起使用。
