ウィルソン病における心臓の関与 (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.
調査の概要
状態
条件
研究の種類
入学 (推定)
段階
- 適用できない
連絡先と場所
研究連絡先
- 名前：Amélie YAVCHITZ
- 電話番号：01 48 03 64 54
- メール：ayavhitz@for.paris
研究連絡先のバックアップ
- 名前：Aurélia POUJOIS
- 電話番号：01 48 30 66 56
- メール：apoujois@for.paris
研究場所
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-
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Paris、フランス、75019
- 募集
- Fondation Adolphe de Rothschild
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コンタクト：
- Aurélia Poujois
- 電話番号：0148036656
- メール：apoujois@for.paris
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-
参加基準
適格基準
就学可能な年齢
健康ボランティアの受け入れ
受講資格のある性別
説明
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
研究計画
研究はどのように設計されていますか？
デザインの詳細
- 主な目的：他の
- 割り当て：なし
- 介入モデル：単一グループの割り当て
- マスキング：なし（オープンラベル）
武器と介入
参加者グループ / アーム |
介入・治療 |
|---|---|
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他の：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. |
この研究は何を測定していますか？
主要な結果の測定
結果測定 |
メジャーの説明 |
時間枠 |
|---|---|---|
|
Contrast-enhanced cardiac MRI - Day 0
時間枠：Day 0
|
Percentage of patients with abnormal contrast-enhanced cardiac MRI results |
Day 0
|
|
Contrast-enhanced cardiac MRI - Day 0
時間枠：Day 0
|
Description of abnormalities (frequency and percentage) |
Day 0
|
|
Transthoracic echocardiography - Day 0
時間枠：Day 0
|
Percentage of patients with abnormal transthoracic echocardiography results |
Day 0
|
|
Transthoracic echocardiography - Day 0
時間枠：Day 0
|
Description of abnormalities (frequency and percentage) |
Day 0
|
|
Chest computed tomography scan without contrast - Day 0
時間枠：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
時間枠：Day 0
|
Percentage of patients with abnormal electrocardiogram results |
Day 0
|
|
Electrocardiogram - Day 0
時間枠：Day 0
|
Description of abnormalities (frequency and percentage) |
Day 0
|
|
Clinical examination - Day 0
時間枠：Day 0
|
Percentage of patients with abnormal clinical examination |
Day 0
|
|
Clinical examination - Day 0
時間枠：Day 0
|
Description of abnormalities (frequency and percentage) |
Day 0
|
|
Blood and urine tests - Day 0
時間枠：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
時間枠：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
時間枠：Day 0
|
Percentage of patients with abnormal blood pressure tests results |
Day 0
|
|
Lying and standing blood pressure tests - Day 0
時間枠：Day 0
|
Description of abnormalities (frequency and percentage) |
Day 0
|
|
Implantable loop recorder or ECG holter recorder- Day 21
時間枠：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
時間枠：Day 21
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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
時間枠：Year 3
|
Percentage of patients with abnormal transthoracic echocardiography results |
Year 3
|
|
Transthoracic echocardiography - Year 3
時間枠：Year 3
|
Description of abnormalities (frequency and percentage) |
Year 3
|
|
Contrast-enhanced cardiac MRI - Year 3
時間枠：Year 3
|
Percentage of patients with abnormal contrast-enhanced cardiac MRI results |
Year 3
|
|
Contrast-enhanced cardiac MRI - Year 3
時間枠：Year 3
|
Description of abnormalities (frequency and percentage) |
Year 3
|
|
Chest computed tomography scan without contrast - Year 3
時間枠：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
時間枠：Year 3
|
Percentage of patients with abnormal electrocardiogram results |
Year 3
|
|
Electrocardiogram - Year 3
時間枠：Year 3
|
Description of abnormalities (frequency and percentage) |
Year 3
|
|
Clinical examination - Year 3
時間枠：Year 3
|
Percentage of patients with abnormal clinical examination |
Year 3
|
|
Clinical examination - Year 3
時間枠：Year 3
|
Description of abnormalities (frequency and percentage) |
Year 3
|
|
Blood and urine tests - Year 3
時間枠：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
時間枠：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
時間枠：Year 3
|
Percentage of patients with abnormal blood pressure tests results |
Year 3
|
|
Lying and standing blood pressure tests - Year 3
時間枠：Year 3
|
Description of abnormalities (frequency and percentage) |
Year 3
|
協力者と研究者
研究記録日
主要日程の研究
研究開始 (実際)
一次修了 (推定)
研究の完了 (推定)
試験登録日
最初に提出
QC基準を満たした最初の提出物
最初の投稿 (実際)
学習記録の更新
投稿された最後の更新 (実際)
QC基準を満たした最後の更新が送信されました
最終確認日
詳しくは
本研究に関する用語
追加の関連 MeSH 用語
その他の研究ID番号
- APS_2021_9
個々の参加者データ (IPD) の計画
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