Children and Adolescents With Marfan Syndrome: 10,000 Healthy Steps and Beyond
试验速览
- 阶段
- 不适用
- 状态
- 已完成
- 入组人数
- 24
- 试验地点
- 2
- 主要终点
- To determine if 6 months of regular physical activity improves aortic stiffness in MFS patients. exercise, a decrease in expressed biomarkers, and an increase in COPE scores of MFS patients.
研究概览
简要总结
Marfan patients are at risk of sudden death due to weakening of the wall of the large blood vessel leading from the heart (aorta). The wall of the aorta weakens and dilates which can rupture, leading to death, and sometimes during intense exercise.
There is some evidence in Marfan patients that a stiffer aorta increases risk for rupture.
For some time, clinical care has focused on what type of exercise these patients should avoid due to risk for aortic dissection. Little clinical emphasis has been placed on encouraging patients to engage in routine and safe exercise such as walking. Informed by this evidence, the investigators propose to collaboratively investigate whether regular exercise improves aortic health in adolescent Marfan patients.
详细描述
Marfan syndrome is an inherited disorder of the connective tissue, which provides material and support for the skeleton, muscles, and blood vessels. Marfan patients are at risk of sudden death due to weakening of the wall of the large blood vessel leading from the heart (aorta). The wall of the aorta weakens and dilates which can rupture, leading to death, and sometimes during intense exercise.
Typically, the weakening process starts when elastin fibers in the aorta become fragmented. There is some evidence in Marfan patients that a stiffer aorta increases risk for rupture.
For some time, clinical care has focused on what type of exercise these patients should avoid due to risk for aortic dissection. Little clinical emphasis has been placed on encouraging patients to engage in routine and safe exercise such as walking. These young patients also frequently choose sedentary lifestyles, most likely due to limitations imposed by parents as well as adolescents' own perceptions of what is safe for them and their physical capabilities. Another complicating factor is that these patients often experience difficulty coping with their diagnosis. Informed by this evidence, the investigators propose to collaboratively investigate whether regular exercise improves aortic health and coping skills in adolescent Marfan patients.
研究设计
- 研究类型
- Interventional
- 分配方式
- Na
- 干预模型
- Single Group
- 主要目的
- Prevention
- 盲法
- None
入排标准
- 年龄范围
- 10 Years 至 19 Years（Child, Adult）
- 性别
- All
- 接受健康志愿者
- 否
入选标准
- •10-19 years of age,
- •MFS by revised Ghent criteria,
- •Cardiac clearance to exercise by the primary cardiologist.
排除标准
- •Ventricular dysfunction,
- •Prior history of aortic surgery.
结局指标
主要结局
To determine if 6 months of regular physical activity improves aortic stiffness in MFS patients. exercise, a decrease in expressed biomarkers, and an increase in COPE scores of MFS patients.
时间窗: 6 months of intervention
The investigators' primary outcome measure is aortic stiffness measured by arterial tonometry (pulse wave velocity).
次要结局
- To determine if 6 months of regular physical activity decreases aortic stiffness and rate of aortic root dilation in Marfan mice.(6 months of intervention)
- To determine if 6 months of regular physical activity improves coping skills in Marfan patients.(6 months of intervention)
- To determine if 6 months of regular physical activity improves aortic stiffness and the biomarker profile in MFS patients.(6 months of intervention)
研究者
Seda Tierney
Assistant Professor in Pediatrics
Stanford University