Marinov Blagoy I, Terziyski Kiril V, Sapunarova Katia G, Kostianev Stefan S
Department of Pathophysiology, Medical University, Plovdiv, 15A Vassil Aprilov St., 4002 Plovdiv, Bulgaria.
Folia Med (Plovdiv). 2008 Oct-Dec;50(4):48-54.
Thalassemia major is associated with impaired exercise tolerance because of the severe anemia and cardiopulmonary dysfunction characteristic of the condition.
To assess the exercise performance in thalassemic children before and two hours after hemotransfusion.
The study included eleven children with thalassemia major (12.3 +/- 2.8 years; Hb g/dl = 8.1 +/- 1.3) and 11 matched controls. All subjects underwent comprehensive pulmonary function assessment and incremental exercise test on a treadmill.
The thalassemic children were, in general, shorter and lighter than their healthy counterparts; we also found lower absolute values of lung function parameters which did not reach statistical significances. On the other hand the most considerable differences were found in diffusion capacity (uncorrected TL(L,CO) % = 56.8 +/- 12.1 vs. 94.3 +/- 16.1 in controls; p<0.001) and blood oxygen content (ctO2 mmol.L(-1) = 4.7 +/- 1.1 vs. 8.3 +/- 0.8, p<0.05). The thalassemic children had significantly lower exercise capacity compared to controls (VO2/ kg = 27.1 +/- 5.0 vs. 37.1 +/- 3.2 mL.min(-1).kg(-1); p<0.001). In the patients' group hemoglobin was elevated significantly two hours after transfusion (Hb g/L from 80.5 +/- 12.7 to 93.6 +/- 10.6; p<0.001) leading to significant improvement in exercise duration (7.3 +/- 2.8 vs. 10.3 +/- 2.3 min; p<0.05), VO2/kg (28.5 +/- 5.0 vs. 36.2 +/- 7.1 mL.min(-1).kg(-1); p<0.05), and transfer factor (4.27 +/- 1.40 vs. 5.41 +/- 1.08 mmol.min(-1).kPa(-1); p=0.003). There were strong correlations between Hb and TL(L,CO) and VO2 (r = 0.687 and 0.750, respectively; p < 0.01 for both).
Patients with thalassemia major have a seriously reduced transfer factor and exercise impairment. The short-term changes in hemoglobin concentration are associated with significant improvement in exercise performance.
重型地中海贫血因该疾病特有的严重贫血和心肺功能障碍而与运动耐力受损有关。
评估重型地中海贫血患儿输血前及输血后两小时的运动表现。
该研究纳入了11名重型地中海贫血患儿（年龄12.3±2.8岁；血红蛋白g/dl = 8.1±1.3）以及11名匹配的对照组儿童。所有受试者均接受了全面的肺功能评估和跑步机递增运动试验。
一般而言，重型地中海贫血患儿比健康对照儿童更矮、更轻；我们还发现肺功能参数的绝对值较低，但未达到统计学意义。另一方面，在弥散功能（未校正的TL（L，CO）% = 56.8±12.1，对照组为94.3±16.1；p<0.001）和血氧含量（ctO2 mmol.L(-1) = 4.7±1.1，对照组为8.3±0.8，p<0.05）方面发现了最显著的差异。与对照组相比，重型地中海贫血患儿的运动能力显著降低（VO2/ kg = 27.1±5.0，对照组为37.1±3.2 mL.min(-1).kg(-1)；p<0.001）。在患者组中，输血后两小时血红蛋白显著升高（血红蛋白g/L从80.5±12.7升至93.6±10.6；p<0.001），导致运动持续时间（7.3±2.8对10.3±2.3分钟；p<0.05）、VO2/kg（28.5±5.0对36.2±7.1 mL.min(-1).kg(-1)；p<0.05）和转移因子（4.27±1.40对5.41±1.08 mmol.min(-1).kPa(-1)；p = 0.003）有显著改善。血红蛋白与TL（L，CO）和VO2之间存在强相关性（r分别为0.687和0.750；两者p均<0.01）。
重型地中海贫血患者的转移因子严重降低，运动功能受损。血红蛋白浓度的短期变化与运动表现的显著改善有关。
