윌슨병의 심장 침범 (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.
연구 개요
상태
상태
정황
정황
개입 / 치료
개입 / 치료
연구 유형
연구 유형
등록 (추정된)
등록
단계
단계
- 해당 없음
연락처 및 위치
연구 연락처
연구 연락처
- 이름: Amélie YAVCHITZ
- 전화번호: 01 48 03 64 54
- 이메일: ayavhitz@for.paris
연구 연락처 백업
- 이름: Aurélia POUJOIS
- 전화번호: 01 48 30 66 56
- 이메일: apoujois@for.paris
연구 장소
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Paris, 프랑스, 75019
- 모병
- Fondation Adolphe de Rothschild
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연락하다:
- Aurélia Poujois
- 전화번호: 0148036656
- 이메일: apoujois@for.paris
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-
참여기준
자격 기준
자격 기준
공부할 수 있는 나이
건강한 자원 봉사자를 받아들입니다
연구 대상 성별
설명
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
공부 계획
연구는 어떻게 설계됩니까?
디자인 세부사항
- 주 목적: 다른
- 할당: 해당 없음
- 중재 모델: 단일 그룹 할당
- 마스킹: 없음(오픈 라벨)
팔의 수
무기와 개입
참가자 그룹 / 팔참가자 그룹 / 팔 |
개입 / 치료개입 / 치료 |
|---|---|
|
다른: 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. |
연구는 무엇을 측정합니까?
주요 결과 측정
주요 결과 측정
결과 측정 |
측정값 설명 |
기간 |
|---|---|---|
|
Contrast-enhanced cardiac MRI - Day 0
기간: Day 0
|
Percentage of patients with abnormal contrast-enhanced cardiac MRI results |
Day 0
|
|
Contrast-enhanced cardiac MRI - Day 0
기간: Day 0
|
Description of abnormalities (frequency and percentage) |
Day 0
|
|
Transthoracic echocardiography - Day 0
기간: Day 0
|
Percentage of patients with abnormal transthoracic echocardiography results |
Day 0
|
|
Transthoracic echocardiography - Day 0
기간: Day 0
|
Description of abnormalities (frequency and percentage) |
Day 0
|
|
Chest computed tomography scan without contrast - Day 0
기간: 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
기간: Day 0
|
Percentage of patients with abnormal electrocardiogram results |
Day 0
|
|
Electrocardiogram - Day 0
기간: Day 0
|
Description of abnormalities (frequency and percentage) |
Day 0
|
|
Clinical examination - Day 0
기간: Day 0
|
Percentage of patients with abnormal clinical examination |
Day 0
|
|
Clinical examination - Day 0
기간: Day 0
|
Description of abnormalities (frequency and percentage) |
Day 0
|
|
Blood and urine tests - Day 0
기간: 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
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|
Blood and urine tests - Day 0
기간: 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
기간: Day 0
|
Percentage of patients with abnormal blood pressure tests results |
Day 0
|
|
Lying and standing blood pressure tests - Day 0
기간: Day 0
|
Description of abnormalities (frequency and percentage) |
Day 0
|
|
Implantable loop recorder or ECG holter recorder- Day 21
기간: 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
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Implantable loop recorder or ECG holter recorder- Day 21
기간: 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
기간: Year 3
|
Percentage of patients with abnormal transthoracic echocardiography results |
Year 3
|
|
Transthoracic echocardiography - Year 3
기간: Year 3
|
Description of abnormalities (frequency and percentage) |
Year 3
|
|
Contrast-enhanced cardiac MRI - Year 3
기간: Year 3
|
Percentage of patients with abnormal contrast-enhanced cardiac MRI results |
Year 3
|
|
Contrast-enhanced cardiac MRI - Year 3
기간: Year 3
|
Description of abnormalities (frequency and percentage) |
Year 3
|
|
Chest computed tomography scan without contrast - Year 3
기간: 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
기간: Year 3
|
Percentage of patients with abnormal electrocardiogram results |
Year 3
|
|
Electrocardiogram - Year 3
기간: Year 3
|
Description of abnormalities (frequency and percentage) |
Year 3
|
|
Clinical examination - Year 3
기간: Year 3
|
Percentage of patients with abnormal clinical examination |
Year 3
|
|
Clinical examination - Year 3
기간: Year 3
|
Description of abnormalities (frequency and percentage) |
Year 3
|
|
Blood and urine tests - Year 3
기간: 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
기간: 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
기간: Year 3
|
Percentage of patients with abnormal blood pressure tests results |
Year 3
|
|
Lying and standing blood pressure tests - Year 3
기간: Year 3
|
Description of abnormalities (frequency and percentage) |
Year 3
|
공동 작업자 및 조사자
연구 기록 날짜
연구 주요 날짜
연구 시작 (실제)
연구 시작
기본 완료 (추정된)
기본 완료
연구 완료 (추정된)
연구 완료
연구 등록 날짜
최초 제출
최초 제출
QC 기준을 충족하는 최초 제출
QC 기준을 충족하는 최초 제출
처음 게시됨 (실제)
처음 게시됨
연구 기록 업데이트
마지막 업데이트 게시됨 (실제)
마지막 업데이트 게시됨
QC 기준을 충족하는 마지막 업데이트 제출
QC 기준을 충족하는 마지막 업데이트 제출
마지막으로 확인됨
마지막으로 확인됨
추가 정보
이 연구와 관련된 용어
추가 관련 MeSH 약관
기타 연구 ID 번호
기타 연구 ID 번호
- APS_2021_9
개별 참가자 데이터(IPD) 계획
개별 참가자 데이터(IPD)를 공유할 계획입니까?
약물 및 장치 정보, 연구 문서
미국 FDA 규제 의약품 연구
미국 FDA 규제 기기 제품 연구
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