Endurance Exercise Training in Young Adults with Barth Syndrome: A Pilot Study
Endurance Exercise Training in Young Adults with Barth Syndrome: A Pilot Study
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DOI：
10.1007/8904_2016_553
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发表时间：
2017-01-01
期刊：
影响因子：
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通讯作者：
Taylor, Carolyn L.
中科院分区：
文献类型：
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作者：
Cade, W. Todd;Reeds, Dominic N.;Taylor, Carolyn L.
背景资料：Barth综合征（BTHS）是一种罕见的X连锁疾病，其特征是线粒体异常，心脏骨骼肌病，运动不耐受和过早死亡。耐力运动训练对运动耐量，心脏骨骼功能和生活质量的影响BTHS is unknown.Methods：四个年轻的成年人（23 +/- 5岁，n = 4）BTHS参加了为期12周的，监督，个性化的耐力运动训练计划。在自行车功率计上进行运动训练，每周三次，每次30-450，中等强度水平。运动耐量进行了分级运动试验和峰值耗氧量，通过二维和M型超声心动图，骨骼肌功能近红外光谱，生活质量通过明尼苏达州生活与心脏衰竭questionnaire.Results：有没有不良事件在运动试验或训练的任何参与者的心脏功能。三到四名参与者的峰值耗氧量略有改善（类似于5%）。运动训练后，关于呼吸困难和药物副作用的平均生活质量问题显著改善。平均静息心脏功能或骨骼肌氧提取在运动过程中没有改善后，运动training.Conclusion：耐力运动训练是安全的，似乎适度提高高峰运动耐力和某些措施的生活质量在年轻的成年人BTHS。然而，与在其他非BTHS线粒体肌病和心力衰竭中观察到的耐力运动训练引起的改善相比，这些改善似乎变钝了。对BTHS最有益的运动训练方式、强度和频率的进一步研究是必要的。
Background: Barth syndrome (BTHS) is a rare X-linked disorder that is characterized by mitochondrial abnormalities, cardio-skeletal myopathy, exercise intolerance, and premature mortality. The effect on endurance exercise training on exercise tolerance, cardio-skeletal function, and quality of life in BTHS is unknown.Methods: Four young adults (23 +/- 5 years, n = 4) with BTHS participated in a 12-week, supervised, individualized endurance exercise training program. Exercise training was performed on a cycle ergometer for 30-450 three times per week at a moderate intensity level. Exercise tolerance was measured by graded exercise testing and peak oxygen consumption, heart function via two-dimensional and M-mode echocardiography, skeletal muscle function by near-infrared spectroscopy, and quality of life through the Minnesota Living with Heart Failure questionnaire.Results: There were no adverse events during exercise testing or training for any participant. Peak oxygen consumption modestly (similar to 5%) improved in three or four participants. Mean quality of life questions regarding dyspnea and side effects from medications significantly improved following exercise training. Mean resting heart function or skeletal muscle oxygen extraction during exercise did not improve after exercise training.Conclusion: Endurance exercise training is safe and appears to modestly improve peak exercise tolerance and certain measures of quality of life in young adults with BTHS. However, compared to improvements resulting from endurance exercise training seen in other non-BTHS mitochondrial myopathies and heart failure, these improvements appear blunted. Further research into the most beneficial mode, intensity and frequency of exercise training in BTHS is warranted.