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Surrogate Biomarkers for Disease Progression in IPF

IPF 疾病进展的替代生物标志物

基本信息

批准号：
7911854
负责人：
NAFTALI KAMINSKI
金额：
$ 50.19万
依托单位：
UNIVERSITY OF PITTSBURGH AT PITTSBURGH
依托单位国家：
美国
项目类别：
财政年份：
2009
资助国家：
美国
起止时间：
2009-08-10 至 2011-07-31
项目状态：
已结题

来源：
https://reporter.nih.gov/project-details/7911854
关键词：
Acute Address Alveolar Apoptosis Bioinformatics Biological Assay Biological Markers Biopsy Blood Blood Cells Blood gas Blood specimen Cells Chronic Clinical Clinical Practice Guideline Coagulation Process Complement Cytokine Activation Data Databases Deltastab Deterioration Diagnosis Diagnostic Diffuse Disease Disease Outcome Disease Progression Dyspnea Echocardiography Epithelial Epithelial Cells Evaluation Exercise Extracellular Matrix Fibroblasts Fibrosis Gene Expression Gene Expression Profile Genes Genetically Modified Animals Genomics Growth Factor Hamman-Rich syndrome Host Defense Host Defense Mechanism Hypersensitivity Immune Immunity Individual Injury Integration Host Factors Literature Lung Lung Transplantation Lung diseases Malignant neoplasm of lung Measurement Measures Medical Methods Molecular Biology Molecular Profiling Mononuclear Multiple Sclerosis Nature Organ Outcome Study Pathway interactions Patients Pattern Peripheral Pharmaceutical Preparations Physiological Plasma Plasma Proteins Procedures Process Progressive Disease Proteins Protocols documentation Pulmonary function tests Relative (related person)Research Personnel Resolution Respiratory physiology Risk Role Sampling Serum Severity of illness Signal Transduction Spirometry Stream Structure of parenchyma of lung Surrogate Markers T-Lymphocyte Technology Testing Time Transforming Growth Factors Translating United States National Institutes of Health WNT Signaling Pathway Walking Work X-Ray Computed Tomography advanced disease base cell injury chemokine clinically relevant cohort combinatorial cytokine defense response design follow-up osteopontin overexpression peripheral blood programs protein expression protein profiling pulmonary function research study response response to injury single molecule syndecan treatment effect

项目摘要

Idiopathic pulmonary fibrosis (IPF) is a progressive fibrotic disease of the lung unaffected by currently available medical therapies. Although traditionally considered a chronic progressive disease it has been recently recognized that patient's course may differ and that while some patients have a chronic slow decline in their pulmonary functions others have a more accelerated course characterized by "acute exacerbations". The variable course of the disease and the recent observation that improvement in patient survival does not have to be associated with an improvement in traditional pulmonary function tests, suggest that traditional methods for tracking IPF progression may at best serve as chroniclers of a forgone conclusion and not provide any useful information about disease progression. We hypothesize that host defense factors, potentially but not necessarily associated with the primary cause of the disease, determine the rate of disease progression. Characterizing these factors will allow us to identify an integrated set of surrogate biomarkers in the peripheral blood that correlate and potentially predate IPF progression. The proposal has the following specific aims: 1. To create, using the Simmons Center ILD Database and referral base, and the Short term IPF outcome study, a cohort of carefully characterized IPF patients that will be prospectively clinically followed up according to current clinical practice guidelines. 2. To analyze protein expression levels in the serum of target molecules using a multianalyte bead based protein profiling assays assay. 3. To identify a gene expression signature in PBMC that is diagnostic and relevant to disease progression and treatment effect. 4. To determine the role of adaptive immunity in IPF disease progression.

特发性肺纤维化（Idiopathic pulmonary fibrosis， IPF）是一种不受当前疾病影响的进行性肺纤维化疾病