Physical activity in patients with sickle cell disease: Effect on the vascular function, frequency of painful episodes and clinical course of the disease

迄今为止，从未相对于客观测量的体力活动（PA）水平来研究与镰状细胞病（SCD）相关的临床并发症。截至 2022 年 3 月 31 日，85 名受试者（65 名患者和 20 名健康受试者）已纳入研究方案。所有受试者均接受为期 5 周的 PA 评估。 SCD 患者已接受一组测试，旨在评估他们的临床状态和血管健康状况。他们还完成了日记来记录疼痛、潜在的血管闭塞性疼痛事件和相关药物。八十二名受试者已完成基线评估。 30 名患者被纳入为期 3 个月的纵向方案，其中包括 PA 干预和随访。其中二十二人已完成整个纵向协议。基线时，患者每日步数中位数分布在 3341 至 29643 步之间。有些患者每天定期进行超过 300 分钟的中度至剧烈 PA。血红蛋白正常的受试者表现出相似的步数范围，表明 SCD 患者可以进行与对照患者一样多的 PA。尚未发现严重临床事件与 PA 的量或强度之间存在任何关系。然而，在未报告主要临床并发症的患者中，每日步数与疼痛发生率之间存在显着负相关。此外，我们观察到 PA 体积与血液粘度之间呈负相关，这表明最活跃的患者的血液流变学特征有所改善。

To date, clinical complications associated with sickle cell diseases (SCD) have never been studied relatively to the level of objectively measured physical activity (PA). By March 31, 2022, 85 subjects (65 patients and 20 healthy subjects) have been included in the study protocol. All subjects underwent a 5-week assessment of PA. The SCD patients have been submitted to a panel of tests aiming at assessing their clinical status and vascular health. They also completed a diary to record pain, potential vaso-occlusive painful events and related medications. Eighty-two subjects have completed the baseline assessment. Thirty patients have been included in a 3-month longitudinal protocol that includes a PA intervention and follow-up visits. Twenty-two of them have completed the whole longitudinal protocol. At baseline, the median daily step-count of patients are distributed from 3341 to 29643 steps. Some patients regularly performed more than 300 min per day of moderate-to-vigorous PA. Subjects with normal hemoglobin showed a similar range for step-count indicating that SCD patients can perform as much PA as their control counterpart. No relationship has been found between severe clinical events and the volume or intensity of PA. However, a significant negative correlation was found between daily step-count and pain occurrence in patients who did not report major clinical complications. In addition, we observed a negative correlation between the volume of PA and blood viscosity, which points to an improved hemorheological profile in the most active patients.