Exercise capacity and clinical outcomes in adults followed in the Cooperative Study of Sickle Cell Disease (CSSCD).

Exercise capacity and clinical outcomes in adults followed in the Cooperative Study of Sickle Cell Disease (CSSCD).
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DOI:
10.1111/ejh.13140
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发表时间:
2018-10
影响因子:
3.1
通讯作者:
Liem RI
Liem RI
中科院分区:
医学3区
文献类型:
--
作者:
Badawy SM;Payne AB;Rodeghier MJ;Liem RI

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确定与镰状细胞病(SCD)成人运动能力相关的因素及其与住院和死亡率的关系。镰状细胞病合作研究(CSSCD)中共有223名受试者(64%为女性,70%为血红蛋白SS/Sβ0地中海贫血,平均年龄43.3 ± 7.5岁)使用跑步机方案进行了最大运动试验,平均持续时间为11.6 ± 5.2分钟。女性性(β = −3.34,95% CI [-1.80,−4.88],p < 0.001),年龄较大(β = −0.14,95% CI [-0.24,−0.04],p = 0.005),体重指数较高(β = −0.23,95% CI [-0.37,−0.10]; p = 0.001)和较低的血红蛋白(β = 0.56,95%CI [0.08,1.04],p = 0.02)与较低的适应性独立相关,而肺功能检查异常有趋势(β = −1.42,95% CI [-2.92,0.07]; p = 0.06)。1秒用力呼气量(FEV 1)预测百分比较低与健康状况较低独立相关(β = 0.08,95% CI [0.03,0.13],p = 0.001)。测试前疼痛和急性胸部综合征(ACS)的基因型和住院率与运动能力无关。在我们的队列中,基线运动能力既不能预测未来的疼痛或ACS,也不能预测生存率。成人SCD耐受最大运动试验。需要进行前瞻性研究,以进一步评估定期锻炼和改善健身对SCD患者临床结局和死亡率的影响。
To determine factors associated with exercise capacity in adults with sickle cell disease (SCD) and its relationship to hospitalizations and mortality. A total of 223 participants in the Cooperative Study of Sickle Cell Disease (CSSCD) (64% female, 70% hemoglobin SS/Sβ0 thalassemia, mean age 43.3 ± 7.5 years) underwent maximal exercise testing using a treadmill protocol with a mean duration of 11.6 ± 5.2 minutes. Female sex (β = −3.34, 95% CI [–1.80, −4.88], p < 0.001), older age (β = −0.14, 95% CI [–0.24, −0.04], p = 0.005), higher body mass index (β = −0.23, 95% CI [–0.37, −0.10]; p = 0.001) and lower hemoglobin (β = 0.56, 95% CI [0.08, 1.04], p = 0.02) were independently associated with lower fitness, while there was a trend with abnormal pulmonary function testing (β = −1.42, 95% CI [–2.92, 0.07]; p = 0.06). Lower percent predicted forced expiratory volume in 1 second (FEV1) was independently associated with lower fitness (β = 0.08, 95% CI [0.03, 0.13], p = 0.001). Genotype and hospitalization rates for pain and acute chest syndrome (ACS) prior to testing were not associated with exercise capacity. Baseline exercise capacity predicted neither future pain or ACS nor survival in our cohort. Adults with SCD tolerated maximal exercise testing. Prospective studies are needed to further evaluate the impact of regular exercise and improved fitness on clinical outcomes and mortality in SCD.
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