威尔逊病的心脏受累 (WIL-HEART)
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.
研究概览
地位
条件
研究类型
注册 (估计的)
阶段
- 不适用
联系人和位置
学习联系方式
- 姓名：Amélie YAVCHITZ
- 电话号码：01 48 03 64 54
- 邮箱：ayavhitz@for.paris
研究联系人备份
- 姓名：Aurélia POUJOIS
- 电话号码：01 48 30 66 56
- 邮箱：apoujois@for.paris
学习地点
-
-
-
Paris、法国、75019
- 招聘中
- Fondation Adolphe de Rothschild
-
接触：
- Aurélia Poujois
- 电话号码：0148036656
- 邮箱：apoujois@for.paris
-
-
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 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
Exclusion Criteria:
- 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
学习计划
研究是如何设计的？
设计细节
- 主要用途：其他
- 分配：不适用
- 介入模型：单组作业
- 屏蔽：无（打开标签）
武器和干预
参与者组/臂 |
干预/治疗 |
|---|---|
|
其他：Cases
The intervention consists in setting up a morphological and rhythmological cardiological follow-up patients with confirmed Wilson disease. It will require the wearing of a long Holter duration (21 days) and for some patients the installation of an implantable cardiac monitor.The long-time ECG holter is used to record heart rhythm for 21 days and detect possible arrhythmias (accelerations of the heart) or conduction disorders (slowdowns of the heart). |
The intervention consists of setting up a morphological and rhythmological cardiological follow-up of patients with confirmed Wilson's disease. It will require the wearing of a long-term Holter (21 days) and for some patients the installation of an implantable cardiac monitor. |
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 |
|---|---|---|
|
Contrast-enhanced cardiac MRI - Day 0
大体时间：Day 0
|
Percentage of patients with abnormal contrast-enhanced cardiac MRI results |
Day 0
|
|
Contrast-enhanced cardiac MRI - Day 0
大体时间：Day 0
|
Description of abnormalities (frequency and percentage) |
Day 0
|
|
Transthoracic echocardiography - Day 0
大体时间：Day 0
|
Percentage of patients with abnormal transthoracic echocardiography results |
Day 0
|
|
Transthoracic echocardiography - Day 0
大体时间：Day 0
|
Description of abnormalities (frequency and percentage) |
Day 0
|
|
Chest computed tomography scan without contrast - Day 0
大体时间：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. |
Day 0
|
|
Electrocardiogram - Day 0
大体时间：Day 0
|
Percentage of patients with abnormal electrocardiogram results |
Day 0
|
|
Electrocardiogram - Day 0
大体时间：Day 0
|
Description of abnormalities (frequency and percentage) |
Day 0
|
|
Clinical examination - Day 0
大体时间：Day 0
|
Percentage of patients with abnormal clinical examination |
Day 0
|
|
Clinical examination - Day 0
大体时间：Day 0
|
Description of abnormalities (frequency and percentage) |
Day 0
|
|
Blood and urine tests - Day 0
大体时间：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 |
Day 0
|
|
Blood and urine tests - Day 0
大体时间：Day 0
|
Description of abnormalities (frequency and percentage) 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 |
Day 0
|
|
Lying and standing blood pressure tests - Day 0
大体时间：Day 0
|
Percentage of patients with abnormal blood pressure tests results |
Day 0
|
|
Lying and standing blood pressure tests - Day 0
大体时间：Day 0
|
Description of abnormalities (frequency and percentage) |
Day 0
|
|
Implantable loop recorder or ECG holter recorder- Day 21
大体时间：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. |
Day 21
|
|
Implantable loop recorder or ECG holter recorder- Day 21
大体时间：Day 21
|
Description of abnormalities (frequency and percentage) The device will record from Day 0 to Day 21 Implantable loop recorder assessment will be only performed on patients with syncope. |
Day 21
|
|
Transthoracic echocardiography - Year 3
大体时间：Year 3
|
Percentage of patients with abnormal transthoracic echocardiography results |
Year 3
|
|
Transthoracic echocardiography - Year 3
大体时间：Year 3
|
Description of abnormalities (frequency and percentage) |
Year 3
|
|
Contrast-enhanced cardiac MRI - Year 3
大体时间：Year 3
|
Percentage of patients with abnormal contrast-enhanced cardiac MRI results |
Year 3
|
|
Contrast-enhanced cardiac MRI - Year 3
大体时间：Year 3
|
Description of abnormalities (frequency and percentage) |
Year 3
|
|
Chest computed tomography scan without contrast - Year 3
大体时间：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. |
Year 3
|
|
Electrocardiogram - Year 3
大体时间：Year 3
|
Percentage of patients with abnormal electrocardiogram results |
Year 3
|
|
Electrocardiogram - Year 3
大体时间：Year 3
|
Description of abnormalities (frequency and percentage) |
Year 3
|
|
Clinical examination - Year 3
大体时间：Year 3
|
Percentage of patients with abnormal clinical examination |
Year 3
|
|
Clinical examination - Year 3
大体时间：Year 3
|
Description of abnormalities (frequency and percentage) |
Year 3
|
|
Blood and urine tests - Year 3
大体时间：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 |
Year 3
|
|
Blood and urine tests - Year 3
大体时间：Year 3
|
Description of abnormalities (frequency and percentage) 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 |
Year 3
|
|
Lying and standing blood pressure tests - Year 3
大体时间：Year 3
|
Percentage of patients with abnormal blood pressure tests results |
Year 3
|
|
Lying and standing blood pressure tests - Year 3
大体时间：Year 3
|
Description of abnormalities (frequency and percentage) |
Year 3
|
合作者和调查者
研究记录日期
研究主要日期
学习开始 (实际的)
初级完成 (估计的)
研究完成 (估计的)
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
与本研究相关的术语
其他相关的 MeSH 术语
其他研究编号
- APS_2021_9
计划个人参与者数据 (IPD)
计划共享个人参与者数据 (IPD)？
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