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

• 批准号: 7950607
• 负责人: SAUL J. KARPEN
• 金额: $ 2.41万
• 依托单位: BAYLOR COLLEGE OF MEDICINE
• 依托单位国家: 美国
• 财政年份: 2008
• 资助国家: 美国
• 起止时间: 2008-12-01 至 2009-11-30
• 项目状态: 已结题
• 来源: https://reporter.nih.gov/project-details/7950607
• 关键词: 2 year old; Bile fluid; Biliary Atresia; Blood; Child; Clinical; Clinical Research; Computer Retrieval of Information on Scientific Projects Database; Databases; Development; Developmental Delay Disorders; Diagnosis; Disease; Epidemiology; Failure; Family; First Degree Relative; Funding; Genetic; Grant; Growth; Infant; Institution; Laboratories; Liver diseases; Operative Surgical Procedures; Outcome; Pathogenesis; Research; Research Personnel; Resources; Risk Factors; Source; Time; Tissue Sample; United States National Institutes of Health; Urine; base; disease natural history; genetic risk factor; prospective; repository; success; total measurement Bilirubin

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. 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 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.

该子项目是利用该技术的众多研究子项目之一 资源由 NIH/NCRR 资助的中心拨款提供。子项目和 研究者 (PI) 可能已从 NIH 的另一个来源获得主要资金， 因此可以在其他 CRISP 条目中表示。列出的机构是 对于中心来说，它不一定是研究者的机构。 1.环境/流行病学/遗传因素导致儿童胆汁淤积性肝病的发病。 2. 诊断时的超声检查结果对于胆道闭锁的诊断具有特异性。 3. 临床参数，例如实验室值（例如术后 6 个月的总胆红素）、各种临床表现（例如腹水），可预测 2 岁时 Kasai 门肠造口术的最终结果。 发育迟缓可以根据系列临床（例如生长障碍）或实验室值（例如总胆红素）来预测。 具体目标 1：建立一个前瞻性数据库，其中包含胆汁淤积性疾病婴儿及其家庭的人口统计和临床信息。 具体目标 2：为这些儿童及其一级亲属的血液、尿液、胆汁和组织样本建立储存库。 具体目标 3：随着时间的推移前瞻性地跟踪这些儿童，以描述疾病的自然史。 具体目标 4：识别风险因素（例如环境、传染性和遗传风险因素）；与发病、结果和治疗成功有关；针对不同的胆汁淤积性疾病，特别强调胆道闭锁。