BILIARY ATRESIA RESEARCH CONSORTIUM (BARC): A PROSPECTIVE DATABASE OF INFANT

胆道闭锁研究联盟 (BARC)：婴儿前瞻性数据库

基本信息

批准号：
8356666
负责人：
SAUL J. KARPEN
金额：
$ 3.26万
依托单位：
BAYLOR COLLEGE OF MEDICINE
依托单位国家：
美国
项目类别：
财政年份：
2010
资助国家：
美国
起止时间：
2010-12-01 至 2011-11-30
项目状态：
已结题

来源：
https://reporter.nih.gov/project-details/8356666
关键词：
2 year old Bile fluid Biliary Atresia Blood Child Clinical Clinical Research Databases Development Developmental Delay Disorders Diagnosis Disease Epidemiology Failure Family First Degree Relative Funding Genetic Grant Growth Infant Laboratories Liver diseases National Center for Research Resources Operative Surgical Procedures Outcome Pathogenesis Principal Investigator Research Research Infrastructure Resources Risk Factors Source Time Tissue Sample United States National Institutes of Health Urine abstracting base cost disease natural history genetic risk factor prospective repository success total measurement Bilirubin

2 year old Bile fluid Biliary Atresia Blood Child Clinical Clinical Research Databases Development Developmental Delay Disorders Diagnosis Disease Epidemiology Failure Family First Degree Relative Funding Genetic Grant Growth Infant Laboratories Liver diseases National Center for Research Resources Operative Surgical Procedures Outcome Pathogenesis Principal Investigator Research Research Infrastructure Resources Risk Factors Source Time Tissue Sample United States National Institutes of Health Urine abstracting base cost disease natural history genetic risk factor prospective repository success total measurement Bilirubin

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项目摘要

This subproject is one of many research subprojects utilizing the resources provided by a Center grant funded by NIH/NCRR. Primary support for the subproject and the subproject's principal investigator may have been provided by other sources, including other NIH sources. The Total Cost listed for the subproject likely represents the estimated amount of Center infrastructure utilized by the subproject, not direct funding provided by the NCRR grant to the subproject or subproject staff. ABSTRACT HYPOTHESIS 1. Environmental/epidemiological/genetic factors contribute to the pathogenesis of cholestatic liver diseases in children. 2. Ultrasonographic findings at diagnosis are specific for the diagnosis of biliary atresia. 3. Clinical parameters, such as laboratory values, (e.g. total bilirubin at 6 months post-surgery), the development of various clinical findings (e.g. ascities), are predictive of the evantual outcome of the Kasai Portoenterostomy by 2 years of age. Developmental delay can be predicted based upon serial clinical (e.g. growth failure) or laboratory values (e.g. total bilirubin). SPECIFIC AIMS Specific Aim 1: To establish a prospective database with demographic and clinical information about infants with cholestatic disease and their families. Specific Aim 2: To establish repositories for blood, urine, bile, and tissue samples from these children and their first-degree relatives. Specific Aim 3: To prospectively follow these children over time to characterize the natural history of the disease. Specicic Aim 4: To identify risk factors (such as, environmental, infectious and genetic risk factors) related to onset, to outcome and to the success of treatment(s) for the different cholestatic diseases, with special emphasis on biliary atresia.

该副本是利用资源的众多研究子项目之一由NIH/NCRR资助的中心赠款提供。对该子弹的主要支持而且，副投影的主要研究员可能是其他来源提供的包括其他NIH来源。列出的总费用可能代表subproject使用的中心基础架构的估计量， NCRR赠款不直接向子弹或副本人员提供的直接资金。抽象的假设 1。环境/流行病学/遗传因素有助于儿童胆汁淤积性肝病的发病机理。 2。诊断时的超声检查结果特异性是胆汁闭锁的诊断。 3。临床参数，例如实验室值（例如，手术后6个月的总胆红素），各种临床发现的发展（例如腹部）可以预测Kasai Portoenterolotolotolotolotolotolotolotolotolotolotolotolotolotolotolotolotolotolotolotolotolotolotolotolotolotolotolotolotolotolotolot的发展。可以根据串行临床（例如生长失败）或实验室值（例如总胆红素）预测发育延迟。具体目标具体目的1：建立一个前瞻性数据库，其中包含有关胆汁淤积性疾病及其家人的婴儿的人口和临床信息。特定目的2：建立来自这些儿童及其一级亲属的血液，尿液，胆汁和组织样本的存储库。特定目的3：随着时间的流逝，前瞻性地跟随这些孩子来表征疾病的自然史。特定目的4：确定与发作，结局和对不同胆汁疾病的治疗成功有关的危险因素（例如环境，感染和遗传危险因素），并特别强调了胆道闭锁。