Grover Conference on Risk Factors in Pulmonary Hypertension

描述（由申请方提供）：肺动脉高压（PAH）是一种导致右心衰竭和死亡的肺血管系统进行性疾病。特发性和家族性PAH（分别为iPAH和fPAH）诊断时的平均年龄为35岁，如果未经治疗，中位生存期为2.8年。骨形态发生蛋白受体2（BMPR 2）的突变已在至少70%的FPAH患者中发现。然而，这些BMPR 2突变的突变率为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患者中的比例明显过高。这些现象都没有得到充分的解释，本次会议将审查和讨论最近的研究，以确定影响疾病复发率的主要因素。基础研究发现正在紧张和持续地寻找肺动脉高压（PAH）的治疗方法，突出了转化研究的必要性，这弥合了实验室实验室发现与临床领域实际应用之间的差距。因此，来自基础和临床领域的研究人员在Grover Conference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发病率增加的因素以及BMPR 2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、新的内源性血管活性因子和与BMPR 2上位性相互作用的新的遗传位点。本次会议的演讲和海报展示将回顾这一迅速发展的领域。遗传和环境风险因素对患者对当前和新兴疗法的反应性的影响也将被讨论。2011年格罗弗会议汇集了遗传学，生理学，药理学，内分泌学基础研究领域的专家，以及药物开发和PAH临床管理的临床方法。受邀演讲者包括ATS肺循环和呼吸细胞和分子生物学大会的成员，代表研究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题演讲者，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学到重要的经验教训。自1984年成立以来，2011年格罗弗会议将是该系列的第15届会议，代表了肺循环方面历史最悠久的会议。今天，它仍然是肺血管功能的主要会议。

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.