Grover Conference on Risk Factors in Pulmonary Hypertension

格罗弗肺动脉高压危险因素会议

• 批准号: 8130165
• 负责人: KAREN A. FAGAN
• 金额: $ 3万
• 依托单位: UNIVERSITY OF SOUTH ALABAMA
• 依托单位国家: 美国
• 财政年份: 2011
• 资助国家: 美国
• 起止时间: 2011-07-01 至 2012-06-30
• 项目状态: 已结题
• 来源: https://reporter.nih.gov/project-details/8130165
• 关键词: Age; Basic Science; Blood Circulation; Blood Vessels; Cellular biology; Cessation of life; Clinic; Clinical; Clinical Management; Communities; Complex; Development; Diagnosis; Diagnostic; Discipline; Disease; Endocrinology; Environmental Risk Factor; Epigenetic Process; Estrogen Metabolism; FDA approved; Female; Gender; Genetic; Genetic Risk; Gonadal Steroid Hormones; Heart; Heart failure; Learning; Life Expectancy; Lung; Lung diseases; Malignant Neoplasms; Meta-Analysis; Minority; Molecular; Molecular Biology; Mutation; Obesity; Patients; Penetrance; Pharmacology; Physiology; Progressive Disease; Publishing; Pulmonary Circulation; Pulmonary Hypertension; Pulmonary artery structure; Randomized Controlled Trials; Reporting; Research; Research Personnel; Risk Factors; Role; Scientist; Senior Scientist; Series; Symptoms; Therapeutic; Translating; Translational Research; Translations; Woman; bone morphogenetic protein receptors; design; drug development; hemodynamics; lectures; meetings; member; mutation carrier; novel; novel therapeutic intervention; posters; practical application; premature; productivity loss; pulmonary arterial hypertension; respiratory; symposium

DESCRIPTION (provided by applicant): Pulmonary arterial hypertension (PAH) is a progressive disease of the pulmonary vasculature resulting in right heart failure and death. Idiopathic and familial forms of PAH (iPAH and fPAH respectively) have a mean age at diagnosis of 35 years and a median survival of 2.8 years if untreated. Mutations in the bone morphogenetic protein receptor 2 (BMPR2) have been identified in at least 70% of FPAH patients. However, the penetrance of these BMPR2 mutations is 10- 20%, suggesting that genetic and/or environmental modifiers are required for disease expression. In addition, females are significantly over-represented amongst FPAH patients. Neither of these phenomena have been adequately explained and this conference will review and discuss recent research with a view to determining the major factors influencing disease penetrance. Basic research findings are intense and ongoing to find treatments for pulmonary arterial hypertension (PAH), highlighting the need for translational research which bridges the gap between lab bench discoveries and practical applications in the clinical realm. As such, it is important for researchers from both basic and clinical fields to meet in focused forums such as the Grover Conference. In the past decade, advances in PAH drug development have centered on factors that may be responsible for the increased penetrance in females and genetic and/or environmental modifiers that are required for disease expression in BMPR2 mutation carriers. These include estrogen metabolism, obesity, novel endogenous vasoactive factors and novel genetic loci interacting epistatically with BMPR2. Talks and poster presentations at this conference will review this rapidly developing field. The impact of genetic and environmental risk factors on the responsiveness of patients to current and emerging therapies will also be discussed. The 2011 Grover Conference brings together experts in the basic research fields of genetics, physiology, pharmacology, endocrinology, as well as clinical approaches dealing with drug development and the clinical management of PAH. Invited speakers include members of the ATS Pulmonary Circulation and Respiratory Cell and Molecular Biology Assemblies at the ATS representing active research groups studying the underlying cellular and molecular mechanisms of PAH. We have also invited key-note speakers from other relevant disciplines as PAH has a complex pathobiology and there are important lessons to be learned from other fields such as obesity, epigenetics, and cancer. Since its inauguration in 1984, the 2011 Grover Conference will be the 15th in this series, representing the longest-standing conference on the pulmonary circulation. Today it remains the principal conference for pulmonary vascular function.

描述（由申请人提供）：肺动脉高压（PAH）是一种肺血管系统的进行性疾病，导致右心衰竭和死亡。特发性和家族性 PAH（分别为 iPAH 和 fPAH）的平均诊断年龄为 35 岁，如果不治疗，中位生存期为 2.8 年。至少 70% 的 FPAH 患者中已发现骨形态发生蛋白受体 2 (BMPR2) 突变。然而，这些 BMPR2 突变的外显率为 10-20%，表明疾病表达需要遗传和/或环境修饰剂。此外，女性在 FPAH 患者中所占比例明显过高。这两种现象都没有得到充分的解释，本次会议将回顾和讨论最近的研究，以确定影响疾病外显率的主要因素。为了寻找肺动脉高压（PAH）的治疗方法，基础研究成果不断涌现，这凸显了转化研究的必要性，以弥合实验室发现与临床领域实际应用之间的差距。因此，对于来自基础和临床领域的研究人员来说，在格罗弗会议等重点论坛上会面非常重要。在过去的十年中，PAH 药物开发的进展集中在可能导致女性外显率增加的因素以及 BMPR2 突变携带者疾病表达所需的遗传和/或环境修饰剂上。这些包括雌激素代谢、肥胖、新型内源性血管活性因子以及与 BMPR2 上位相互作用的新型基因位点。本次会议的演讲和海报展示将回顾这个快速发展的领域。还将讨论遗传和环境风险因素对患者对当前和新兴疗法的反应的影响。 2011 Grover 会议汇集了遗传学、生理学、药理学、内分泌学等基础研究领域以及涉及药物开发和 PAH 临床管理的临床方法的专家。受邀演讲者包括 ATS 肺循环和呼吸细胞和分子生物学协会的成员，代表着研究 PAH 潜在细胞和分子机制的活跃研究小组。我们还邀请了来自其他相关学科的主讲嘉宾，因为 PAH 具有复杂的病理生物学，并且可以从肥胖、表观遗传学和癌症等其他领域吸取重要的经验教训。自 1984 年召开以来，2011 年 Grover 会议将是该系列会议的第 15 次，代表着历史最悠久的肺循环会议。今天，它仍然是肺血管功能的主要会议。