Microvessels and Heart Problems in Sickle Cell Disease
Microvascular and Cardiac Dysfunction in Sickle Cell Disease
Background:
- Small blood vessels (microvessels) in many different organs are affected by diseases such as diabetes and atherosclerosis. These microvessels may also be abnormal in people who have sickle cell disease. Stiffness of the red blood cells leads to problems in the microvessels of the heart and kidneys. However, these problems may not be detected until these organs are severely affected. Researchers want to study problems with microvessels in people with and without sickle cell disease.
Objectives:
- To study how microvessels in the heart and other organs are affected by sickle cell disease.
Eligibility:
- Individuals at least 18 years of age who have sickle cell disease.
- Healthy volunteers at least 18 years of age.
Design:
- Participants will be screened with a physical exam and medical history. Blood and urine samples will be collected.
- All participants will have about 3 to 4 hours of testing for the study. Participants with sickle cell disease who are having a pain crisis at the time they enter the study may be asked to have the testing again when the crisis is over. The repeat testing will occur at least 4 weeks after the pain crisis ends.
- All participants will have the following tests:
- Blood draws to check kidney and liver function, and other blood tests
- Measure of blood flow in the brachial (upper arm) artery
- Heart ultrasound
- Ultrasound scans of arm muscles to study blood flow
- Ultrasound scans after taking vasodilators to increase blood flow
- Healthy volunteers will also have a magnetic resonance imaging scan. It will show blood flow in the heart. This scan will involve another dose of a vasodilator.
研究概览
地位
条件
详细说明
研究类型
注册 (实际的)
联系人和位置
学习地点
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Maryland
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Bethesda、Maryland、美国、20892
- National Institutes of Health Clinical Center, 9000 Rockville Pike
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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
描述
- INCLUSION CRITERIA:
- Adult subject age greater than or equal to 18 years
- Able to give written informed consent
- For SCD groups, must have confirmed diagnosis of sickle cell disease
EXCLUSION CRITERIA:
- Atrial fibrillation or other irregular rhythm that would preclude adequate image acquisition
- Subjects with a contraindication for the ultrasound contrast agent.
- Pregnant or lactating women
- Known obstructive coronary or peripheral vascular disease
- SCD subjects at steady-state must not have acute pain crisis requiring intravenous analgesics within the prior 4 weeks
- SCD subjects in crisis must be within 72 hours of hospital admission
- Subjects with contraindications to MRI scanning will complete all other procedures but will not undergo the MRI scan. Subjects with an estimated glomerular filtration rate of <30 ml/min/1.73 m(2) will not receive gadolinium as per 2011 NHLBI gadolinium administration policy.
- Subjects with a contraindication to regadenson
- Any condition that in the clinical opinion of the investigators renders study procedures inadvisable.
Diagnosis of acute chest syndrome is not an exclusion criteria for this protocol. Subjects may be concurrently enrolled in any other protocols with the exception of investigational new drug studies.
学习计划
研究是如何设计的?
设计细节
- 时间观点:预期
研究衡量的是什么?
主要结果指标
结果测量 |
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To use contrast-enhanced ultrasound to evaluate microvascular blood flow (MBF) and capillary RBC velocity (CBV) in skeletal muscle and the heart (at rest and during vasodilator stress) in patients with SCD in comparison to normal control patient...
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次要结果测量
结果测量 |
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To determine whether MBF and CBV worsen during pain crisis in patients with SCD
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To determine the relation between MBF and CBV and brachial artery flow or LV dysfunction.
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合作者和调查者
调查人员
- 首席研究员:Vandana Sachdev, M.D.、National Heart, Lung, and Blood Institute (NHLBI)
出版物和有用的链接
一般刊物
- Kato GJ, Hebbel RP, Steinberg MH, Gladwin MT. Vasculopathy in sickle cell disease: Biology, pathophysiology, genetics, translational medicine, and new research directions. Am J Hematol. 2009 Sep;84(9):618-25. doi: 10.1002/ajh.21475.
- Hebbel RP, Osarogiagbon R, Kaul D. The endothelial biology of sickle cell disease: inflammation and a chronic vasculopathy. Microcirculation. 2004 Mar;11(2):129-51.
- Morris CR, Kuypers FA, Larkin S, Vichinsky EP, Styles LA. Patterns of arginine and nitric oxide in patients with sickle cell disease with vaso-occlusive crisis and acute chest syndrome. J Pediatr Hematol Oncol. 2000 Nov-Dec;22(6):515-20. doi: 10.1097/00043426-200011000-00009.
- Sachdev V, Sidenko S, Wu MD, Minniti CP, Hannoush H, Brenneman CL, Waclawiw MA, Arai AE, Schechter AN, Kato GJ, Lindner JR. Skeletal and myocardial microvascular blood flow in hydroxycarbamide-treated patients with sickle cell disease. Br J Haematol. 2017 Nov;179(4):648-656. doi: 10.1111/bjh.14918. Epub 2017 Sep 7.
研究记录日期
研究主要日期
学习开始
研究完成
研究注册日期
首次提交
首先提交符合 QC 标准的
首次发布 (估计)
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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