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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
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.
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| Label | Type | Description | Intervention Names |
|---|---|---|---|
| functions micro and macro-vascular in connection the clinical | Other | 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. |
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| Name | Type | Description | Arm Group Labels | Other Names |
|---|---|---|---|---|
| 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) | Diagnostic Test | The study permit 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 within the 2 preceding years) and the rate of acute chest syndrome (severe if > 0 in the last 2 years). |
| Measure | Description | Time Frame |
|---|---|---|
| Microvascular Function | 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) | Through study completion, an average of 3 years |
| Macrovascular Function (Arterial Stiffness) | What is measured: Arterial rigidity will be evaluated using pulse wave velocity (PWV) measurements. Units: meters/second (m/s) | Assessed through study completion, average follow-up 3 years |
| Measure | Description | Time Frame |
|---|---|---|
| Hematological and Hemorheological Profile Complete blood count | Units: g/dL (hemoglobin), | Through study completion, an average of 3 years |
| Oxidative Stress Profile | Plasma and erythrocyte markers of oxidative stress (e.g., malondialdehyde, glutathione). Units: μmol/L or standardized assay units |
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Inclusion Criteria:
Exclusion Criteria:
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| Name | Affiliation | Role |
|---|---|---|
| Marie BILLAUD, Doctor in the Sickle Cell | Hospital University Center of Pointe-à-Pitre | Principal Investigator |
| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| Hospital University Center of Pointe-à-Pitre | Pointe-à-Pitre | Guadeloupe | 97159 | Guadeloupe |
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SCD 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.
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| Through study completion, average follow-up 3 years |
| Circulating Nitric Oxide Levels | Plasma concentration of nitric oxide metabolites. Units: μmol/L | Through study completion, average follow-up 3 years |
| Circulating Microparticles | Number and origin of circulating microparticles (platelet, leukocyte, erythrocyte, endothelial). Units: particles/μL | Through study completion, average follow-up 3 years |
| Autonomic Nervous System Activity | Heart rate variability (HRV) derived from Holter ECG recordings. Units: ms (standard deviation of NN intervals), normalized units (frequency domain parameters) | Through study completion, average follow-up 3 years |
| Muscle and Cerebral Oxygenation | Oxygen saturation of muscle and brain tissue using near-infrared spectroscopy (NIRS). Units: % oxygen saturation | Through study completion, average follow-up 3 years |
| Physical Activity and Capacity 6-minute walk test distance | Units: meters (walk test), | Through study completion, average follow-up 3 years |
| Effect of Hydroxyurea on Vascular Function | Units: Perfusion units (microvascular), m/s (pulse wave velocity) | Through study completion, average follow-up 3 years |
| Hematological and Hemorheological Profile hematocrit | Units: % (hematocrit), | Thought study completion , an average of 3 years |
| Hematological and Hemorheological Profile blood viscosity parameters | mPa·s (blood viscosity) | Through study completion, an average of 3 years |
| Physical Activity and Capacity oxygen consumption (VO2) | mL/kg/min (VO2) | Through study completion, average follow-up 3 years |
| Physical Activity and Capacity Accelerometer | activity counts | Through study completion, average follow-up 3 years |
| ID | Term |
|---|---|
| D000755 | Anemia, Sickle Cell |
| D006445 | Hemoglobin C Disease |
| ID | Term |
|---|---|
| D000745 | Anemia, Hemolytic, Congenital |
| D000743 | Anemia, Hemolytic |
| D000740 | Anemia |
| D006402 | Hematologic Diseases |
| D006425 | Hemic and Lymphatic Diseases |
| D006453 | Hemoglobinopathies |
| D030342 | Genetic Diseases, Inborn |
| D009358 | Congenital, Hereditary, and Neonatal Diseases and Abnormalities |
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