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

这个子项目是许多研究子项目中利用 资源由NIH/NCRR资助的中心拨款提供。子项目和 调查员(PI)可能从NIH的另一个来源获得了主要资金， 并因此可以在其他清晰的条目中表示。列出的机构是 该中心不一定是调查人员的机构。 1.环境/流行病学/遗传因素导致儿童胆汁淤积性肝病。 2.诊断时超声表现对胆道闭锁的诊断具有特异性。 3.临床参数，如实验室数值(如术后6个月的总胆红素)、各种临床表现的发展(如腹水)，可预测Kasai门肠吻合术2岁前的预后。 发育迟缓可以根据一系列的临床数据(如生长障碍)或实验室数值(如总胆红素)来预测。 具体目标1：建立一个关于胆汁淤积症婴儿及其家庭的人口学和临床信息的前瞻性数据库。 具体目标2：建立这些儿童及其一级亲属的血液、尿液、胆汁和组织样本的储存库。 具体目标3：前瞻性地跟踪这些儿童一段时间，以确定疾病的自然病史。 特殊目标4：确定风险因素(如环境、感染和遗传风险因素)；与发病、结果和治疗成功有关(S)；针对不同的胆汁淤积性疾病，特别强调胆道闭锁。

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.