PEDIATRIC PULMONARY SCOR FOCUSED ON CYSTIC FIBROSIS

关注囊性纤维化的小儿肺损伤

基本信息

批准号：
3106649
负责人：
WARREN J WARWICK
金额：
$ 60.01万
依托单位：
UNIVERSITY OF MINNESOTA
依托单位国家：
美国
项目类别：
财政年份：
1982
资助国家：
美国
起止时间：
1982-01-01 至 1986-12-31
项目状态：
已结题

来源：
https://reporter.nih.gov/project-details/3106649
关键词：
cystic fibrosis human subject pediatrics

项目摘要

This Specialized Center of Research will continue to provide a central supporting structure for the efforts of many investigators concerned with the pulmonary problems of cystic fibrosis and provide a forum for integration and cooperation between their individual projects. The primary topic of the SCOR is being addressed by both clinical and basic research projects. The former are attempting to find better means of patient care and more sensitive indicators of disease progression while the latter are studying the pathologic mechanisms of lung injury and the basic metabolic defect. The specific goals of the individual projects are: -To use a centralized patient data base and analysis system to coordinate patient care and research studies. -To demonstrate the feasibility and medical effectiveness of using automated analysis of home measurements for improving clinical care. -To continue the evolution of a new pulmonary function test for patients of all ages and to use this test to follow normal lung development in young children and disease progression in CF patients. -To investigate the structure and function of mucopolysaccharide molecules in bronchial mucus. -To evaluate a possible mechanism of CF lung destruction by pulmonary macrophages and continue investigations of their metabolic, structural, and phagocytic properties. -To expand upon observations of enhanced metabolic activities of CF monocytes. -To extend the knowledge of the deranged NADH dehydrogenase found in mitochondria from CF subjects as a probably basic defect in CF cells and investigate the possibility of a prenatal test for CF.

该专业研究中心将继续提供中央许多研究人员的努力的支持结构囊性纤维化的肺部问题并提供论坛他们各个项目之间的整合与合作。初级 SCOR 的主题正在通过临床和基础研究来解决项目。前者正在尝试寻找更好的患者护理方法和更敏感的疾病进展指标，而后者是研究肺损伤的病理机制和基础代谢缺点。各个项目的具体目标是： -使用集中的患者数据库和分析系统来协调患者护理和研究。 -证明使用的可行性和医疗效果自动分析家庭测量结果以改善临床护理。 -继续开发针对以下患者的新肺功能测试所有年龄段，并使用此测试来跟踪年轻人的正常肺部发育儿童和 CF 患者的疾病进展。 -研究粘多糖分子的结构和功能存在于支气管粘液中。 -评估CF肺损伤的可能机制巨噬细胞并继续研究它们的代谢、结构和吞噬特性。 -扩展对 CF 代谢活性增强的观察单核细胞。 -扩展对NADH脱氢酶的了解来自 CF 受试者的线粒体可能是 CF 细胞的基本缺陷，调查 CF 产前检查的可能性。