2010 NHLBI/LAM Foundation International Lymphangioleiomyomatosis Research Confere

描述(由申请人提供)： LAM基金会2010年国际淋巴管肌瘤病研究会议将聚焦于女性致命肺部疾病淋巴管肌瘤病(LAM)中发生的平滑肌增殖和囊性肺破坏的分子机制。这是第十届辛辛那提LAM年会，已经从一个专门的家庭/患者会议发展成为LAM和结节性硬化症的最先进的会议。基础科学会议将重点研究LAM中平滑肌增殖的细胞和遗传基础，并将重点关注外源生长因子、性激素、遗传突变、细胞内信号机制和转录调控变化的作用。其他课程将探讨肺内的平滑肌渗透与肺实质囊性重塑之间的联系，包括LAM细胞对金属蛋白酶和结缔组织基质的阐述。同时举行的临床会议将探讨LAM的棘手管理问题，包括激素治疗、航空旅行、怀孕、肺移植和胸膜并发症。基础和临床科学轨道已得到协调，允许所有参与者参加主题会议，并在会议之间自由交流。一个以病人为中心的研讨会将为100名LAM患者和100名LAM家庭讨论关键的临床问题，预计将参加研讨会，并将在会议的最后一天由基础和临床轨道主席总结科学会议和举行圆桌讨论。每届会议将由一名实地专家主持，并邀请发言者以及从提交的摘要库中挑选的发言者。有意识地努力吸引相关学科的资深调查人员，这些调查人员可以补充现有LAM调查人员的专业知识。由讨论带头人主持的讨论将是所有会议的重要组成部分。杰出的初级调查人员将获得旅行补助金，并将有机会以平台和海报的形式介绍他们的研究。本次会议的目标是为领先的专家提供一个论坛，讨论LAM的分子和细胞基础，回顾LAM的临床管理，展示尖端研究，整合概念，确定创造性和创新性的研究方向。公共卫生相关性：这项建议将为2010年LAM研究会议(4月9日至11日，俄亥俄州辛辛那提)提供部分支持，这是LAM研究的主要论坛。今年的基础和临床科学主席是内科科学家Elizabeth Henske博士和Lisa Young博士，前者是TSC遗传学家，长期对LAM感兴趣，后者是一名临床科学家，对LAM的临床试验和生物标记物感兴趣。会议的主要目标是：1)回顾LAM的研究进展，2)吸引新的研究人员参与LAM研究，3)寻找有前途的LAM研究新方向，4)试图就棘手的临床问题达成共识。

DESCRIPTION (provided by applicant): The LAM Foundation 2010 Lymphangioleiomyomatosis International Research Conference will focus on the molecular mechanisms of smooth muscle proliferation and cystic lung destruction that occur in the fatal lung disease of women, Lymphangioleiomyomatosis (LAM). This is the tenth annual Cincinnati LAM conference, which has evolved from a dedicated family/patient conference to a state of the art meeting for LAM and tuberous sclerosis. The basic science sessions will highlight research on the cellular and genetic basis of smooth muscle proliferation in LAM, and will focus on the role of exogenous growth factors, sex hormones, genetic mutations, intracellular signaling mechanisms and changes in transcriptional regulation. Additional sessions will explore the link between smooth muscle infiltration of the lungs and the cystic remodeling of pulmonary parenchyma, including the elaboration of metalloproteinases and connective tissue matrix by LAM cells. Concurrent clinical sessions will explore difficult management issues in LAM, including hormonal therapy, air travel, pregnancy, lung transplantation and pleural complications. The Basic and Clinical Science tracks have been coordinated to allow all participants to attend keynote sessions and to traffic freely between sessions. A patient-oriented seminar will address key clinical issues for one hundred LAM patients and 100 LAM families who are expected to attend, and will culminate in a summary of scientific sessions by the Basic and Clinical Track Chairs and a roundtable discussion on the last day of the conference. Each session will be chaired by an expert in the field and will have invited speakers as well as speakers chosen from the pool of submitted abstracts. There has been a conscious effort to attract established investigators from related disciplines who can complement the expertise of the current pool of LAM investigators. Discussion facilitated by a discussion leader will be an important component of all sessions. Outstanding junior investigators will be awarded travel grants and will be given the opportunity to present their research in platform and poster formats. The goal of this conference is to provide a forum for leading experts to discuss the molecular and cellular basis of LAM, review the clinical management of LAM, to present cutting edge research, to integrate concepts and to identify creative and innovative research directions. PUBLIC HEALTH RELEVANCE: This proposal will provide partial support for the 2010 LAM Research Conference (April 9-11, Cincinnati, OH), which is the primary forum for LAM research. The basic and clinical science chairs this year are physician scientists Drs. Elizabeth Henske, a TSC geneticist with a longstanding interest LAM, and Dr. Lisa Young, a clinical scientist with an interest in clinical trials and biomarkers in LAM. The primary objectives for the conference are to: 1) to review research developments in LAM, 2) to attract new investigators to LAM research, 3) to identify promising new LAM research directions, and 4) to attempt to reach consensus on difficult clinical issues.