SEARCHING FOR CLUES TO THE PATHOBIOLOGY OF PULMONARY HYPERTENSION

这个子项目是许多研究子项目中的一个 由NIH/NCRR资助的中心赠款提供的资源。子项目和 研究者（PI）可能从另一个NIH来源获得了主要资金， 因此可以在其他CRISP条目中表示。所列机构为 研究中心，而研究中心不一定是研究者所在的机构。 硬皮病、先天性心脏病和特发性肺动脉高压中肺动脉高压（肺动脉压力增加）的触发因素和机制尚不清楚。此外，没有可靠的早期疾病标志物。更好地了解肺动脉高压的病理生物学可能导致早期检测和新的治疗方法。 我们建议研究肺血管损伤和功能障碍的某些标志物的作用，血管重塑的相关因素，以及肺炎衣原体感染在肺动脉高压病理生物学中的潜在作用。这些变量将在肺动脉高压、特发性肺纤维化、硬皮病伴和不伴肺动脉高压和原发性雷诺氏病患者中进行比较。 没有性别、民族或种族限制。研究受试者的年龄限制为男性60岁，女性65岁，并且没有动脉粥样硬化疾病或主要冠状动脉风险因素，以限制临床或亚临床动脉粥样硬化对结果的潜在影响。没有弱势群体。患者的初始要求是提供60 ml血液样本，进行心脏超声检查，并允许查看其记录。我们将把生物标志物的水平与肺动脉高压的存在和严重程度联系起来。研究数据库将通过数字代码识别每位患者。代码将与患者大学CPI编号匹配，文件将提供给高级研究者和项目管理员。合格的患者将由一名研究者联系参加，并提供书面知情同意书。 如果支持可用，将对参与者进行长达5年的随访，以确定是否有任何标志物可预测肺动脉高压的发生、临床结局或进展速度。参与者可能会被要求每隔2年和4年返回进行体格检查，重复抽血和心脏超声检查。将每年随访大学患者记录，以确定生物标志物与肺动脉高压进展和发展速率之间的关系。未在大学随访的患者将被要求允许将其记录发送给研究团队，并可能被要求返回进行临床重新评价。'

This subproject is one of many research subprojects utilizing the resources provided by a Center grant funded by NIH/NCRR. The subproject and investigator (PI) may have received primary funding from another NIH source, and thus could be represented in other CRISP entries. The institution listed is for the Center, which is not necessarily the institution for the investigator. The trigger and mechanism by which pulmonary hypertension (increase in pressures in the arteries of the lungs) develops in scleroderma, congenital heart disease, and idiopathic pulmonary hypertension is not clear. Further, there are no reliable markers of early disease. A better understanding of the pathobiology of pulmonary hypertension could lead to earlier detection and novel therapies. We propose to investigate the role of certain markers of pulmonary vascular injury and dysfunction, factors involved in vascular remodeling, and the potential contribution of infection with chlamydia pneumonia in the pathobiology of pulmonary hypertension. The variables will be compared in patients with pulmonary arterial hypertension, idiopathic pulmonary fibrosis, scleroderma with and without pulmonary hypertension, and primary Raynaud's disease. There will be no gender, ethnic or racial restrictions. The study participants will be limited by age to 60 years in men and 65 years in women, and have no atherosclerotic diseases or major coronary risk factors so as to limit the potential impact of clinical or subclinical atherosclerosis on the results. There are no vulnerable populations. The initial requirement of the patient is to provide a 60ml sample of blood, have a cardiac ultrasound, and permission to review their records. We will correlate the levels of biomarkers with the presence and severity of pulmonary hypertension. The research database will identify each patient by numeric code. The code will be matched with the patient University CPI number and the file will be available to the senior investigators and project administrator. Eligible patients will be approached to participate by one of the investigators and provided written informed consent. If support is available participants will be followed for up to 5 years to determine whether any of the markers predict the development, clinical outcome, or rate of progression of pulmonary hypertension. Participants may be requested to return at intervals of 2 and 4 years for a physical exam, repeat blood draw, and a cardiac ultrasound. University patient records will be followed annually to determine the relationship between the biomarkers and the rate of progression and development of pulmonary hypertension. Patients not followed in the University will be requested to allow there records to be sent to the study team and may be requested to return for clinical reevaluation.'