Observational Multicenter Study Lasting 12 Months to Determine the Prevalence of Pulmonary Hypertension (PAH) in Patients With Thalassemia Major and Intermedia and Verify the Suitability of Common Diagnostic Criteria in This Population
试验速览
- 阶段
- 不适用
- 状态
- 已完成
- 入组人数
- 1,500
- 试验地点
- 8
- 主要终点
- Determination of the prevalence
研究概览
简要总结
This is a multicenter observational case-control analysis lasting 12 months aimed at determining the prevalence of pulmonary hypertension (PAH) in patients with Thalassemia Major and Intermedia. The patients will be followed, treated and examined according to the best standard clinical practice dictated by the Italian Society for the study of Hemoglobinopathies (SITE), Thalassemia International Federation (TIF)and the Task Force for Diagnosis and Treatment of Pulmonary Hypertension of European Society of Cardiology (ESC); European Respiratory Society (ERS); International Society of Heart and Lung Transplantation (ISHLT) guidelines.
详细描述
The most recent International Classification of pulmonary arterial hypertension (PHA) include Hemoglobinopathies in Class I. At present there is no determination of the prevalence of this disease in a large population of thalassemic patients followed in a uniform way. The diagnostic criteria used for the normal population may not be suitable for a population such as thalassemia patients who present features like chronic anemia, iron overload, liver disease, endocrine disorders, etc.
The criteria used to define the disease (PHA) will be those ones dictated by the above mentioned guidelines.
Primary Objective of the study is the determination of the prevalence and severity of PHA in thalassemia syndromes, recently introduced in Class I of the Classification of PHA.
Secondary objectives are:
Critical evaluation of current diagnostic criteria derived from those applied to the general population, taking into account the peculiarities of the observed disease in the thalassemic population.
研究设计
- 研究类型
- Observational
- 观察模型
- Case Control
- 时间视角
- Cross Sectional
入排标准
- 年龄范围
- 18 Years 至 80 Years（Adult, Older Adult）
- 性别
- All
- 接受健康志愿者
- 否
入选标准
- •Patients with Thalassemia Major Patients or Intermediate referring to Centres using Web-Thal medical record (a clinical data sheet used for congenital anemias. Info: www.thalassemia.it)
排除标准
- •Patients who are considered potentially unreliable and/or not cooperative
结局指标
主要结局
Determination of the prevalence
时间窗: 12 months
Determination of the prevalence, defined as the total number of cases in the population, divided by the number of individuals in the population.
次要结局
- Critical evaluation of current diagnostic criteria(12 months)
研究者
Dr. Gian Luca Forni
Principal Investigator and Chief of Centre for Microcythemia an Congenital Anemias - Hematology
Ente Ospedaliero Ospedali Galliera