Human Subjects Core B

临床核心的主要目标是帮助验证炎症和疾病的翻译相关性。 在项目1-3的动物模型中，细胞代谢的改变有助于PH。这一重要的联系将 推进未被发现的机制的临床相关性，并将是迈向致动的第一步 根据这些发现为PAH患者制定新的诊断和治疗策略。中概述的 核心的具体目标和任务，我们将通过提供广泛的表型和 对PAH患者和对照样本(外周血细胞、血浆、血清、肺)进行仔细分类 组织、RNA和DNA)到PPG的3个项目。我们将收集和记录连续和纵向 登记的潜在患者的表型数据，包括临床、血流动力学和晚期 超声心动图评价右心功能。我们将协助研究设计并执行 对所有三个项目进行生物统计分析。患者样本将由以下3组组成：1)血液 来自患者和精心配对的对照组的样本在肺动脉高压和 科罗拉多大学风湿病诊所(前瞻性研究)，2)肺组织 高血压突破倡议(PHBI)和3)PVD的血清、血浆、RNA、DNA和肺组织 科罗拉多大学生物库(回溯性研究)。样本将从4个不同的地方收集 患者群体：1)结缔组织病相关性肺动脉高压(CTD-PAH)，2)特发性肺动脉高压 3)无PAH的CTD(CTD伴/不伴PAH)和4)正常对照组。临床核心是 处于完美的位置，支持拟议的PPG的翻译研究目标。我们已经组建了一支 调查组是研究执行方面的专家，例如在项目中提出的研究。我们的 通过利用病人护理(诊断和随访)所需的临床资源，极大地促进了使命的执行， 科罗拉多大学PVD中心目前的资源，包括生物库和正在进行的 资助的PHBI。临床核心将与项目负责人和病理/流式细胞术密切合作 实现这些目标的核心。

The primary goal of the Clinical Core is to help validate the translational relevance by which inflammation and alterations in cellular metabolism contribute to PH in the animal models of Projects 1-3. This important link will advance the clinical relevance of the uncovered mechanisms, and will be the initial step towards actuating novel diagnostic and therapeutic strategies from these discoveries for patients with PAH. As outlined in the specific aims and tasks of the Core, we will facilitate this goal by providing extensively phenotyped and carefully categorized PAH patient and control samples (peripheral blood cells, plasma, serum, lung tissue, RNA and DNA) to the 3 Projects of the PPG. We will collect and record serial and longitudinal phenotypic data on enrolled prospective patients, including clinical, hemodynamic and advanced echocardiographic assessment of right ventricular function. We will assist in study design and perform the biostatistical analysis for all three Projects. The patient samples will consist of the following 3 cohorts: 1) Blood samples from patients and carefully matched controls followed in the Pulmonary Hypertension and Rheumatology clinics at the University of Colorado (prospective studies), 2) Lung tissue from the Pulmonary Hypertension Breakthrough Initiative (PHBI) and 3) Serum, plasma, RNA, DNA and lung tissue from the PVD Biorepository at the University of Colorado (retrospective studies). The samples will be collected from 4 distinct patient populations: 1) Connective tissue disease associated PAH (CTD-PAH), 2) Idiopathic pulmonary arterial hypertension (IPAH) and 3) CTD without PAH (CTD w/o PAH) and 4) Normal Controls. The clinical core is perfectly situated to support the translational research goals of the proposed PPG. We have assembled a group of investigators expert in the performance of studies such as those proposed within the Projects. Our mission is greatly facilitated by leveraging clinical resources required for patient care (diagnosis and follow-up), the current resources of the University of Colorado PVD center including the biorepository and by the ongoing funded PHBI. The Clinical Core will work closely with the Project leaders and the Pathology/Flow Cytoemtry Core to meet these goals.