Institutional Registry of Amyloidosis (Hospital Italiano de Buenos Aires)
试验速览
- 阶段
- 不适用
- 状态
- 已完成
- 入组人数
- 500
- 试验地点
- 1
- 主要终点
- From treatment to folow up, annualy
研究概览
简要总结
- Creating a population-based registry system Amyloidosis prospective epidemiological survey
- risk factors
- diagnosis
- prognosis
- treatment
- monitoring
- survival
- Describe the occurrence of amyloidosis in the population of HIBA, Hospital Italiano de Buenos Aires.
- Describe the characteristics of clinical presentation, evolution and predisposing factors of amyloidosis.
详细描述
Amyloidosis is a systemic disease that is usually a result of misfolded proteins in the form of amorphous fibrillar material in various tissues and can cause progressive dysfunction of the same. The prevalence of amyloidosis varies depending on the population concerned and the type of amyloid. While prevalence in the general population is unknown, according to estimates by the Mayo Clinic this prevalence is 1 in 90 666% in the U.S. In England this disease generated about 0.0084% (1367 / 16232579) of all hospital visits between April 2008 and April 2009.
The most common clinical manifestations include cardiac disease, renal and liver function, but it may vary widely depending on the type of amyloidosis, the organ infected and extent of the deposits. Amyloid infiltration can produce signs and symptoms that may be very similar to other rheumatic diseases. This may suggest potential clinical polymorphic underdiagnosis due to low clinical suspicion.
The registries are organized systems of systematic data collection of a large number of patients quickly and efficiently on a particular disease at a given time.
The main difficulty of the registries is the guarantee of the quality of their data.
The main objectives of the registry are:
研究设计
- 研究类型
- Observational
- 观察模型
- Ecologic Or Community
- 时间视角
- Prospective
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
- •Cases of amyloidosis are captured by electronic medical records whenever the physician register amyloidosis as a patient diagnosis, and/or there is amyloidosis in a biopsy specimen and/or requests for the following studies of an adult patient: plasma kappa and lambda light-chain concentrations, the kappa: lambda ratio, transthoracic Doppler echocardiography, or cardiovascular magnetic resonance examination or pyrophosphate scintigraphy From the possible cases included in the IRA, a prospective review of the electronics health records was performed to confirm the presence of amyloidosis
- •**Inclusion Criteria: 1 AND (2 or 3)
- •Patients over 18 years:
- •Confirmed amyloidosis: Proof of deposit of amyloid pathology by tissue biopsy in abdominal fat, bone marrow, rectum or organ involved (eg, kidney, liver, sural nerve)
- •Clinically compatible case of Amyloidosis :
排除标准
- •Refusal to participate in the study or the informed consent process by the patient or legal representative or refusal to consent to participate in the study in the case of minors.
结局指标
主要结局
From treatment to folow up, annualy
时间窗: at baseline
Baseline description
Treatment patterns
时间窗: From diagnosis, evaluated after every line of treatment or annualy up to 10 years
Type of treatment, duration, adverse events, completition
Epidemiologic characteristics
时间窗: From treatment date until the date of best organ response or documented progression or date of death from any cause, whichever came first, assessed up to 10 years
Organ response
Epidemiologic characteristics
时间窗: From inclusion date (daignosis) until the date of death from any cause or date of last follow up(if not dead) assessed up to 10 years
Survival anual
次要结局
未报告次要终点
研究者
MARIA LOURDES POSADAS MARTINEZ
MD, PHD
Hospital Italiano de Buenos Aires