Department of Pathophysiology and Transplantation, University of Milan and Bronchopneumology Unit, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Via Francesco Sforza 35, Milan, Italy.
Department of Clinical Sciences and Community Health, University of Milan and Center for Rare Diseases, Internal Medicine Unit, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Via Francesco Sforza 35, Milan, Italy.
Ann Hematol. 2022 Nov;101(11):2445-2452. doi: 10.1007/s00277-022-04974-w. Epub 2022 Sep 13.
β-Thalassemia patients often have a reduced capacity of exercise and abnormal respiratory function parameters, but the reasons are unclear. In order to identify the causes of the exercise limitation, we performed a cardiopulmonary exercise testing (CPET) in a group of 54 adult β-thalassemia major (TM) patients without pulmonary arterial hypertension and in a group of healthy control subjects. All subjects underwent cardiac echocardiography and carried out pulmonary function tests. TM patients also filled an IPAQ questionnaire on usual physical activity (PA).Overall, TM patients have a diminished exercise performance in comparison to control subjects. In fact, peak oxygen uptake (V'O peak), expressing maximum exercise capacity, was decreased in 81.5% of the patients; similarly, anaerobic threshold (V'O@AT) and O pulse also resulted lowered. In multivariable regression models adjusted for gender, age, BMI, and mean haemoglobin, V'O peak and O pulse were positively associated with cardiac iron overload (T2*). No ventilatory limitation to exercise was observed. The most important causes of exercise limitation in these patients were muscular deconditioning and reduced cardiac inotropism due to iron deposition. Only 15/54 (27.8%) TM patients used to perform vigorous physical activity. These results suggest that a program of regular physical activity may be useful to increase the tolerance to effort and therefore to improve the quality of life in these patients.
β-地中海贫血患者常表现出运动能力下降和呼吸功能参数异常，但具体原因尚不清楚。为了明确运动受限的原因，我们对 54 名无肺动脉高压的成年重型β-地中海贫血（TM）患者和一组健康对照者进行了心肺运动测试（CPET）。所有受试者均接受了心脏超声心动图检查和肺功能检查。TM 患者还填写了一份 IPAQ 问卷，以了解其日常体力活动（PA）情况。总的来说，TM 患者的运动能力明显低于对照组。事实上，81.5%的患者的峰值摄氧量（V'O peak），即最大运动能力降低；同样，无氧阈（V'O@AT）和 O 脉搏也降低。在调整了性别、年龄、BMI 和平均血红蛋白后，多元回归模型显示 V'O peak 和 O 脉搏与心脏铁过载（T2*）呈正相关。未观察到运动时出现通气受限。导致这些患者运动受限的最重要原因是肌肉失健和铁沉积导致的心脏变力性降低。仅有 15/54（27.8%）的 TM 患者经常进行剧烈体力活动。这些结果表明，定期进行身体活动的方案可能有助于提高对体力活动的耐受性，从而改善这些患者的生活质量。
