SCOR-LUNG BIOLOGY AND DISEASES IN INFANTS AND CHILDREN

这项来自科罗拉多大学儿科系的建议， 寻求5年的资金，建立一个新的计划，调查 新生儿持续性肺动脉高压（PPHN）。PPHN是一个 相对频繁（1：500活产）的不明原因疾病 除非治疗，否则与高发病率和死亡率（25-50%）相关 进行侵入性的，昂贵的，有点危险的体外循环 膜肺氧合（ECMO）。 我们建议调查初始损伤（如氧化剂和 血液动力学应激）和这些损伤导致 改变的血管反应性和血管重塑（增加的基质， 血管细胞增殖），PPHN的临床标志。 每年约有80-100例患者可用于研究， 成立PPHN提到儿童医院，丹佛，和20 出生于大学医院的高危人群， 已经开发了检测程序。对机制的基本见解 在PPHN手术将从患者，几种动物模型， 新生儿肺动脉高压，以及细胞和分子研究。的 数据将有助于早期发现和干预以及更好的治疗 旨在有效地舒张血管和恢复耗尽的抗氧化剂 的防御合作.该计划将提供早期发现，更好的治疗， 最终预防PPHN。

This proposal from the Department of Pediatrics, University of Colorado, seeks 5 year funding to establish a new program which investigates persistent pulmonary hypertension of the newborn (PPHN). PPHN is a relatively frequent (1:500 live births) disease of unknown origin associated with a high morbidity and mortality (25-50%) unless treated for days with invasive, expensive, and somewhat risky extracorporeal membrane oxygenation (ECMO). We propose to investigate initial insults (such as oxidant and hemodynamic stress) and mechanisms through which these insults lead to altered vasoreactivity and vascular remodeling (increased matrix, and vascular cell proliferation), clinical hallmarks of PPHN. Available for study are approximately 80-100 patients per year with established PPHN referred to The Children's Hospital, Denver, and 20 born to a high risk population at the University Hospital, where early detection procedures have been developed. Basic insights into mechanisms operating in PPHN will be from the patients, several animal models of neonatal pulmonary hypertension, and cellular and molecular studies. The data will allow early detection and interventions and better therapies aimed at effective vasodilation and restoration of depleted antioxidant defenses. The program will provide early detection, better treatment, and ultimately, prevention, of PPHN.