CLINICAL AND BIOLOGICAL PREDICTORS OF TREATMENT RESPONSE IN IDIOPATHIC PULMON

这个子项目是利用由NIH/NCRR资助的中心拨款提供的资源的许多研究子项目之一。子项目和调查员(PI)可能从另一个NIH来源获得了主要资金，因此可能会出现在其他CRISE条目中。列出的机构是针对中心的，而不一定是针对调查员的机构。GCRC资源利用：呼出的一氧化氮收集和前臂脉搏图摘要：在过去的十年里，药物疗法彻底改变了IPAH的治疗，推迟或消除了许多患者对肺移植的需要。然而，肺移植在药物治疗失败的患者的护理中仍然发挥着作用。不幸的是，这导致了一群病情严重、风险极高的患者出现在移植手术中。因此，时机和患者的选择成为这些患者治疗中的一个非常困难的领域。治疗失败的临床标志，如血流动力学监测，通常是很晚的迹象，治疗失败很难定义。对肺动脉高压患者进行非侵入性检测以评估治疗反应是必要的，但目前还不能使用。我们和其他人已经证明，肺动脉高压患者呼出的一氧化氮水平低于健康对照组，而且支气管肺泡灌洗液中的一氧化氮反应产物与肺动脉高压的程度呈负相关。在开始血管扩张剂治疗IPAH的患者中，可以看到他们呼出的一氧化氮水平增加。我们还看到，随着时间的推移，没有任何衡量标准可以预测IPAH患者的死亡率。我们假设IPAH是一种一氧化氮缺乏状态，通过各种方法监测一氧化氮可以用来监测IPAH的时间和对治疗的反应。

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. GCRC Resource Utilization: Exhaled Nitric Oxide Collection and Forearm Pleythsmography Abstract: Medical Therapies have revolutionized the treatment of IPAH over the past decade and have delayed or eliminated the need for lung transplantation for many patients. However, lung transplantation continues to have a role in the care of patients for whom medical treatment fails. Unfortunately, this has resulted in a group of patients who are very sick and extremely high-risk presenting for transplantation. Thus, timing and patient selection has become a very difficult area in the treatment of these patients. Clinical markers of treatment failure, such as hemodynamic monitoring, are often very late signs and treatment failure is very difficult to define. Noninvasive testing for patients with pulmonary hypertension to evaluate the response to therapy is needed, but not available currently. We and others have shown the exhaled nitric oxide levels are lower in patients with pulmonary hypertension than in healthy controls and that nitric oxide reaction products in the bronchoalveolar lavage fluid is inversely related to the degree of pulmonary hypertension. In patients that begin vasodilator therapy for IPAH, an increase in their exhaled nitric oxide levels is seen. We have also seen that no measure over time can predict mortality in IPAH patients. We hypothesize that IPAH is a nitric oxide deficiency state and monitoring nitric oxide by various methods may be used to monitor IPAH over time and response to therapy.