Luyt D K, Richards G A, Roode H, Dowdeswell R J, van Rensburg A J, Reinach S G
Department of Pediatrics, Coronation Hospital, Johannesburg, South Africa.
Pediatr Hematol Oncol. 1993 Jan-Mar;10(1):13-23. doi: 10.3109/08880019309016523.
Pulmonary function tests were performed in 15 thalassemic patients (age 5 years 8 months to 18 years 6 months), receiving both regular transfusions and desferrioxamine, to determine the presence and nature of any abnormalities in lung function. Reactive oxidant production from neutrophils was measured simultaneously to ascertain if a causal relationship existed between free radical production and tissue damage in the lungs. Mean total lung capacity, mean residual volume, and mean forced vital capacity were significantly reduced, indicating a restrictive pattern of lung function abnormality. In addition, the carbon monoxide diffusion was low, and hypoxemia was present in 6 of 13 patients tested. These pulmonary function abnormalities did not correlate with age, cumulative volume of transfusion, or serum ferritin levels. In addition, neutrophil reactive oxidant status did not correlate with these or with pulmonary function parameters. These results indicate that neutrophil-derived oxygen free radicals do not appear to be a major cause of lung function abnormalities in thalassemics.
对15名接受定期输血和去铁胺治疗的地中海贫血患者（年龄5岁8个月至18岁6个月）进行了肺功能测试，以确定肺功能是否存在异常及其性质。同时测量了中性粒细胞产生的反应性氧化剂，以确定自由基产生与肺部组织损伤之间是否存在因果关系。平均总肺容量、平均残气量和平均用力肺活量显著降低，表明存在限制性肺功能异常模式。此外，一氧化碳弥散降低，13名接受测试的患者中有6名存在低氧血症。这些肺功能异常与年龄、累计输血量或血清铁蛋白水平无关。此外，中性粒细胞反应性氧化剂状态与这些因素或肺功能参数均无相关性。这些结果表明，中性粒细胞衍生的氧自由基似乎不是地中海贫血患者肺功能异常的主要原因。
