O'Donnell Angela, Premawardhena A, Arambepola M, Allen S J, Peto T E A, Fisher C A, Rees D C, Olivieri Nancy F, Weatherall D J
Weatherall Institute of Molecular Medicine, University of Oxford, John Radcliffe Hospital, Headington, Oxford OX 3 9DS, United Kingdom.
Proc Natl Acad Sci U S A. 2007 May 29;104(22):9440-4. doi: 10.1073/pnas.0703424104. Epub 2007 May 17.
Severe forms of anemia in children in the developing countries may be characterized by different clinical manifestations at particular stages of development. Whether this reflects developmental changes in adaptation to anemia or other mechanisms is not clear. The pattern of adaptation to anemia has been assessed in 110 individuals with hemoglobin (Hb) E beta-thalassemia, one of the commonest forms of inherited anemia in Asia. It has been found that age and Hb levels are independent variables with respect to erythropoietin response and that there is a decline in the latter at a similar degree of anemia during development. To determine whether this finding is applicable to anemia due to other causes, a similar study has been carried out on 279 children with severe anemia due to Plasmodium falciparum malaria; the results were similar to those in the patients with thalassemia. These observations may have important implications both for the better understanding of the pathophysiology of profound anemia in early life and for its more logical and cost-effective management.
发展中国家儿童的严重贫血形式可能在特定发育阶段具有不同的临床表现。这是否反映了适应贫血的发育变化或其他机制尚不清楚。对110例血红蛋白Eβ地中海贫血患者进行了适应贫血模式的评估，血红蛋白Eβ地中海贫血是亚洲最常见的遗传性贫血形式之一。研究发现，年龄和血红蛋白水平是促红细胞生成素反应的独立变量，并且在发育过程中，在相似程度的贫血情况下，促红细胞生成素反应会出现类似程度的下降。为了确定这一发现是否适用于其他原因引起的贫血，对279例因恶性疟原虫疟疾导致严重贫血的儿童进行了类似研究；结果与地中海贫血患者相似。这些观察结果可能对更好地理解早期严重贫血的病理生理学及其更合理、更具成本效益的管理具有重要意义。
