The Influence of micro-and Macro Vascular Dysfunction on Clinical Severity in Adults With Sickle Cell Anemia (SS) and Sickle Cell Hemoglobin C Disease (SC)
试验速览
- 阶段
- 不适用
- 状态
- 已完成
- 发起方
- 入组人数
- 49
- 试验地点
- 1
- 主要终点
- Microvascular Function
研究概览
简要总结
The primary aim of this study is to determine the implication of micro and macro vascular function on the clinical severity of SCD (SS, SC, Sß°) adults. The secondary aim of this study is to understand the contribution of several parameters, known to influence vascular function in non-SCD individuals, in SCD
详细描述
TSCD patients are characterized by vascular alterations, with vascular function being severely affected. However, the exact contribution of vascular dysfunction in the clinical severity and the risk for frequent vaso-occlusive crises in SCD is unknown. Furthermore the factors involved in this imbalance remain unclear but it is supposed that cerebral hypoxia, the deficit in nitric oxide, abnormal blood rheology, increased microparticles levels, autonomic nervous system imbalance and a low level of physical activity as well as poor physical fitness might be involved.
- Primary outcome: The primary outcome of the present study is to characterize the level of alteration of micro and macro vascular function in SCD (SS, SC and Sß°) adults measured by heat-mediated vasodilation and peripheral perfusion (Laser Doppler system) and pulse wave velocity and to test relationships between this measured vascular function and clinical severity which is based on the rate of vaso-occlusive crisis (severe if ≥ 3 per year), the rate of acute chest syndrome (severe if > 0 per year) and/or the presence of chronic complications.
- Secondary outcomes: To test the existence of relationships between several factors: biological (hematology, blood rheology, nitric oxide and microparticles), physiological (the autonomic nervous system activity measured by Holter electrocardiogram and tissue oxygenation (muscular and cerebral)) and physical activity level (estimated by questionnaire and accelerometer) and physical fitness (estimated by the six minute walk test with oxygen consumption measurements).
- Study design: This study has been designed as biomedical, monocentric prospective and interventional study
研究设计
- 研究类型
- Interventional
- 分配方式
- Na
- 干预模型
- Single Group
- 主要目的
- Basic Science
- 盲法
- None
入排标准
- 年龄范围
- 18 Years 至 —（Adult, Older Adult）
- 性别
- All
- 接受健康志愿者
- 否
入选标准
- •adults ≥ 18 years old,
- •medical diagnosed with SCD (genotype SS, SC or Sß°) by isoelectrofocusing or HPLC at clinical steady state at the time of the study (i.e., no blood transfusion within the last three months,
- •absence of acute episodes of infection, vaso-occlusive crisis or acute chest syndrome at least one month before inclusion in the study),
- •regularly followed by the Sickle Cell Unit of the Academic Hospital of Pointe-à-Pitre (Guadeloupe) and having signed well informed the letter of agreement.
排除标准
- •not at "steady-state";
- •pregnancy or breast feeding;
- •non-compliant patients to usual care;
- •no signed informed consent
结局指标
主要结局
Microvascular Function
时间窗: Through study completion, an average of 3 years
What is measured: Peripheral microvascular function will be assessed using Laser Doppler flowmetry to measure hyperemia response induced by localized heat. Units: Perfusion units (PU)
Macrovascular Function (Arterial Stiffness)
时间窗: Assessed through study completion, average follow-up 3 years
What is measured: Arterial rigidity will be evaluated using pulse wave velocity (PWV) measurements. Units: meters/second (m/s)
次要结局
- Hematological and Hemorheological Profile Complete blood count(Through study completion, an average of 3 years)
- Oxidative Stress Profile(Through study completion, average follow-up 3 years)
- Circulating Nitric Oxide Levels(Through study completion, average follow-up 3 years)
- Circulating Microparticles(Through study completion, average follow-up 3 years)
- Physical Activity and Capacity Accelerometer(Through study completion, average follow-up 3 years)
- Autonomic Nervous System Activity(Through study completion, average follow-up 3 years)
- Muscle and Cerebral Oxygenation(Through study completion, average follow-up 3 years)
- Physical Activity and Capacity 6-minute walk test distance(Through study completion, average follow-up 3 years)
- Hematological and Hemorheological Profile hematocrit(Thought study completion , an average of 3 years)
- Effect of Hydroxyurea on Vascular Function(Through study completion, average follow-up 3 years)
- Physical Activity and Capacity oxygen consumption (VO2)(Through study completion, average follow-up 3 years)
- Hematological and Hemorheological Profile blood viscosity parameters(Through study completion, an average of 3 years)