Cardiopulmonary exercise testing on adaptive equipment in children and adults with Friedreich ataxia.
Cardiopulmonary exercise testing on adaptive equipment in children and adults with Friedreich ataxia.
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弗里德赖希共济失调儿童和成人适应性设备的心肺运动测试。
DOI：
10.1002/mus.28085
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发表时间：
2024
期刊：
影响因子：
3.4
通讯作者：
Lin,KimberlyY
中科院分区：
文献类型：
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作者：
Cilenti,NicoletteA;Tamaroff,JaclynG;Capiola,ChristopherJ;Faig,Walter;McBride,MichaelG;Paridon,StephenM;O'Malley,Shannon;Edelson,JonathanB;Lynch,DavidR;McCormack,ShanaE;Lin,KimberlyY
介绍/目的传统的运动往往是很难与弗里德赖希共济失调（FRDA）的个人，证据是有限的，关于如何衡量在这一人群的运动表现。我们评估的可行性，可靠性，和适应性心肺运动试验（CPET）的表现在儿童和成人FRDA.MethodsParticipants进行CPET上的手臂自行车测力计（ACE）或横卧腿自行车测力计（RLCE）在长达四个访问（基线，2周，4周，1年）的自然历史。在达到最大意志的患者中测量最大功、耗氧量（峰值VO 2）、氧（O2）脉搏和无氧阈值（AT）。重测信度采用组内系数进行评估，纵向变化采用回归分析进行评估。结果在我们的队列（N= 23）中，中位年龄为18岁（四分位距[IQR]，14-23），FRDA发作的中位年龄为8岁FXR等位基因（GAA 1）上的GAA重复长度为766（IQR，650-900）。21例（91%）完成了最大CPET（n= 8，ACE和n = 13，RLCE）。年龄、性别和GAA 1重复长度均与峰值VO 2相关。初步估计表明，访视2和访视3之间ACE和RLCE的峰值功以及RLCE的峰值VO 2、O2脉搏和AT具有合理的一致性。我们没有检测到显着的性能变化超过1 year.DiscussionAdaptive CPET是可行的FRDA，干预措施，影响运动表现，并将增加获得参与以及调查结果的普遍性的相关临床试验结果。
Introduction/AimsTraditional exercise is often difficult for individuals with Friedreich ataxia (FRDA), and evidence is limited regarding how to measure exercise performance in this population. We evaluated the feasibility, reliability, and natural history of adaptive cardiopulmonary exercise test (CPET) performance in children and adults with FRDA.MethodsParticipants underwent CPET on either an arm cycle ergometer (ACE) or recumbent leg cycle ergometer (RLCE) at up to four visits (baseline, 2 weeks, 4 weeks, and 1 year). Maximum work, oxygen consumption (peak VO2), oxygen (O2) pulse, and anaerobic threshold (AT) were measured in those who reached maximal volition. Test–retest reliability was assessed with intraclass coefficients, and longitudinal change was assessed using regression analysis.ResultsIn our cohort (N= 23), median age was 18 years (interquartile range [IQR], 14–23), median age of FRDA onset was 8 years (IQR 6–13), median Friedreich Ataxia Rating Scale score was 58 (IQR 54–62), and GAA repeat length on the shorterFXNallele (GAA1) was 766 (IQR, 650–900). Twenty‐one (91%) completed a maximal CPET (n= 8, ACE andn= 13, RLCE). Age, sex, and GAA1 repeat length were each associated with peak VO2. Preliminary estimates demonstrated reasonable agreement between visits 2 and 3 for peak work by both ACE and RLCE, and for peak VO2, O2pulse, and AT by RLCE. We did not detect significant performance changes over 1 year.DiscussionAdaptive CPET is feasible in FRDA, a relevant clinical trial outcome for interventions that impact exercise performance and will increase access to participation as well as generalizability of findings.