Longitudinal Assessment of Exercise Capacity and Vascular Function in Patients With CF
研究概览
地位
条件
详细说明
Cystic Fibrosis (CF) is the most common fatal genetic disease in North America. The most disturbing aspect of CF is the associated premature death, most often due to respiratory complications. Clinical manifestations of CF include not only lung dysfunction, but many other systemic consequences as well. Systemic oxidative stress and exercise intolerance are established phenotypes in patients with CF. Additionally, for the first time the investigators have recently published the presence of systemic endothelial dysfunction in a cohort of young patients with CF who exhibited normal oxygen saturation and spirometric function.
Exercise intolerance, the limitation of the ability to perform exercise at the expected level, has been shown to predict mortality in patients with CF independent of lung function. Exercise capacity (VO2 peak), an objective measurement of exercise tolerance, drops approximately 5-8% per year in patients with CF. This excessive decay in exercise capacity not only leads to more pulmonary infections and deterioration of lung function, it represents a 5-8 fold decline compared to healthy sedentary adults. Preventing the excessive annual reduction in exercise capacity is essential to increasing the quality of life and longevity of patients with CF. However, a critical barrier to improving exercise capacity in CF is the investigators lack of knowledge regarding the different physiological mechanisms that contribute to exercise intolerance. It is important to emphasize that decreases in lung function (FEV1) do not always contribute to reductions in VO2 peak. Furthermore, less than 2% of patients who have an FEV1 greater than 50% predicted will have a significant drop in hemoglobin oxygen saturation (SpO2) during maximal exercise. These data suggest that mechanisms other than lung function induced hypoxemia may be contributing to exercise intolerance in patients with CF.
研究类型
注册 (实际的)
联系人和位置
学习地点
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Georgia
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Augusta、Georgia、美国、30912
- Georgia Prevention Institute
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参与标准
资格标准
适合学习的年龄
接受健康志愿者
有资格学习的性别
取样方法
研究人群
描述
Inclusion Criteria:
- Patients diagnosed with Cystic Fibrosis (homozygous deltaF508del)
- Prescribed Orkambi
- Men and women (> 18 yrs. old)
- Boys and girls (7 -17 yrs. old)
- FEV1 percent predicted > 40%
- Resting oxygen saturation (room air) >85%
- Patients with or without CFRD
- Traditional CF-treatment medications
- Clinically stable for past 28 days (no exacerbations or change in medical status)
- Healthy Controls
Exclusion Criteria:
- Children 6 yrs. old and younger
- FEV1 percent predicted < 40%
- Resting oxygen saturation (room air) < 85%
- Clinical diagnosis of heart disease
- Pulmonary artery hypertension
- Febrile illness within two weeks of visit
- Current smokers
- Currently pregnant or nursing
- Individuals on vaso-active medications (i.e. nitrates, beta blockers, ACE inhibitors, etc.)
- Use of VX-770 within 6 months prior to Visit 1
- History of solid organ transplantation
- Presence of a condition or abnormality that in the opinion of the investigator would compromise the safety of the patient or the quality of the data.
学习计划
研究是如何设计的?
设计细节
研究衡量的是什么?
主要结果指标
结果测量 |
措施说明 |
大体时间 |
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Maximal exercise capacity
大体时间:1 year
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Subjects will perform the maximal exercise tests on an electronically braked cycle ergometer using the Godfrey protocol.
Expired gases will be collected using a Parvo Medics True One metabolic cart for determination of exercise capacity (VO2 peak).
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1 year
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次要结果测量
结果测量 |
措施说明 |
大体时间 |
---|---|---|
Flow mediated dilation
大体时间:1 year
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The brachial artery FMD test will be performed according to the recent tutorial on the ultrasonic assessment of FMD and shear rate will be calculated as the stimulus of the vasodilatory response.
Briefly, subjects will lie in the supine position for 20 minutes to obtain hemodynamic steady state.
A blood pressure cuff (Hokanson) will be placed around the forearm (distal to the Doppler transducer) and rapidly inflated to 250 mmHg for 5 minutes (circulatory arrest).
Simultaneous ultrasound images of the vessel (B-mode) and Doppler waveforms will be collected 10 seconds prior to and for 2 minutes following deflation of the cuff.
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1 year
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合作者和调查者
调查人员
- 首席研究员:Ryan Harris, PhD、Augusta University
研究记录日期
研究主要日期
学习开始
初级完成 (实际的)
研究完成 (实际的)
研究注册日期
首次提交
首先提交符合 QC 标准的
首次发布 (实际的)
研究记录更新
最后更新发布 (实际的)
上次提交的符合 QC 标准的更新
最后验证
更多信息
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