Cardiac Involvement in Wilson's Disease
试验速览
- 阶段
- 不适用
- 状态
- 招募中
- 入组人数
- 150
- 试验地点
- 2
- 主要终点
- Chest computed tomography scan without contrast - Day 0
研究概览
简要总结
Heart damage by copper accumulation has been reported in Wilson's Disease. However, the disease epidemiology is still poorly understood. A number of studies on pediatric populations have not shown any significant cardiac involvement apart from early dysautonomia. This could suggest that the clinical manifestations related to the copper accumulation in the heart appears with the duration of the disease. Case-control studies on adult populations have highlighted various electrocardiographic (ECG) abnormalities more frequent in patients with Wilson's Disease than in healthy volunteers, but all these studies involved small number of patients (maximum 60). The hypothesis is that there is cardiac involvement in Wilson's Disease, requiring screening, follow-up and appropriate support.
研究设计
- 研究类型
- Interventional
- 分配方式
- Na
- 干预模型
- Single Group
- 主要目的
- Other
- 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
- •Adult patient with Wilson's disease confirmed by a Leipzig score ≥ 4
- •Express consent to participate in the study by the patient or legal guardian in the case of patients under guardianship or by the patient assisted by his curator in the case of patients under guardianship
- •Member of or beneficiary of a Social Security scheme
排除标准
- •Absolute or relative contraindication to MRI or contrast media
- •Pregnant, parturient or breast-feeding women: a urine pregnancy test will be carried out in women of childbearing age
- •Patient with hepatic decompensation (Child-Pugh score stage C)
- •Patient in neuro-psychiatric decompensation
结局指标
主要结局
Chest computed tomography scan without contrast - Day 0
时间窗: Day 0
Percentage of patients with abnormal coronary artery calcium score Calcium score : The higher the coronary calcium score, the greater the cardiovascular risk. A score of 0 (min) means that no calcium is seen in the heart. A score greater than 300 is a sign of very high to severe disease.
Contrast-enhanced cardiac MRI - Day 0
时间窗: Day 0
Percentage of patients with abnormal contrast-enhanced cardiac MRI results
Chest computed tomography scan without contrast - Year 3
时间窗: Year 3
Percentage of patients with abnormal coronary artery calcium score Calcium score : The higher the coronary calcium score, the greater the cardiovascular risk. A score of 0 (min) means that no calcium is seen in the heart. A score greater than 300 is a sign of very high to severe disease.
Electrocardiogram - Year 3
时间窗: Year 3
Percentage of patients with abnormal electrocardiogram results
Transthoracic echocardiography - Day 0
时间窗: Day 0
Percentage of patients with abnormal transthoracic echocardiography results
Electrocardiogram - Day 0
时间窗: Day 0
Percentage of patients with abnormal electrocardiogram results
Clinical examination - Day 0
时间窗: Day 0
Percentage of patients with abnormal clinical examination
Blood and urine tests - Day 0
时间窗: Day 0
Percentage of patients with abnormal blood and urine tests results Blood tests performed : lipid profile, glycosylated hemoglobin (HbA1C), cardiac enzymes (troponin C, NT-proBNP), ultra-sensitive C-reactive protein, sodium level, potassium level, urea and creatinine clearance, TSH, fibrinogen Urine test performed : proteinuria
Lying and standing blood pressure tests - Day 0
时间窗: Day 0
Percentage of patients with abnormal blood pressure tests results
Implantable loop recorder or ECG holter recorder- Day 21
时间窗: Day 21
Percentage of patients with abnormal implantable loop record (or ECG holter record) The device will record from Day 0 to Day 21 Implantable loop recorder assessment will be only performed on patients with syncope.
Transthoracic echocardiography - Year 3
时间窗: Year 3
Percentage of patients with abnormal transthoracic echocardiography results
Contrast-enhanced cardiac MRI - Year 3
时间窗: Year 3
Percentage of patients with abnormal contrast-enhanced cardiac MRI results
Clinical examination - Year 3
时间窗: Year 3
Percentage of patients with abnormal clinical examination
Blood and urine tests - Year 3
时间窗: Year 3
Percentage of patients with abnormal blood or urine tests results Blood tests performed : lipid profile, glycosylated hemoglobin (HbA1C), cardiac enzymes (troponin C, NT-proBNP), ultra-sensitive C-reactive protein, sodium level, potassium level, urea and creatinine clearance, TSH, fibrinogen Urine test performed : proteinuria
Lying and standing blood pressure tests - Year 3
时间窗: Year 3
Percentage of patients with abnormal blood pressure tests results
次要结局
未报告次要终点