A PROSPECTIVE DATABASE OF INFANTS WITH CHOLESTASIS

该子项目是利用NIH/NCRR资助的中心赠款提供的资源的许多研究子项目之一。子项目和研究者（PI）可能从另一个NIH来源获得主要资金，因此可以在其他CRISP条目中表示。所列机构为中心，不一定是研究者所在机构。胆道闭锁是儿科肝病发病率和死亡率的主要原因，也是儿科肝移植最常见的适应症。尽管它的临床重要性，很少有人知道广泛的基本问题，关于胆道闭锁。胆道闭锁的最佳治疗方法尚不清楚。目前还没有关于胆道闭锁患儿护理重要方面的前瞻性随机试验。关于胆道闭锁的大多数已发表数据是轶事，基于单中心经验。目前还没有关于胆道闭锁的治疗和结局的综合登记研究。 胆道闭锁研究联盟（BARC）是一个由9个临床研究中心和一个数据协调中心组成的NIH资助的网络，其目标是研究婴儿的胆道闭锁，新生儿肝炎和其他胆汁淤积性疾病。本研究的主要目的是建立（1）包含临床信息的数据库和（2）来自患有新生儿肝脏疾病（如胆道闭锁和新生儿肝炎）的儿童的血液和组织样本的储存库，以促进对这些重要的儿童肝脏问题的研究。本研究将：1.在招募时和随访期间收集关于每个受试者的详细临床和人口统计学信息，2.在诊断时和随访期间从受试者获得并储存血液和尿液样品，3.获得并储存在诊断期间取出的肝脏和胆管组织以及胆汁（即，活检）或在手术或移植时，并且不需要用于诊断目的4.收集入组时父母的人口统计学和病史，以及5.在入组时从亲生父母处获取并储存血液。

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. Biliary atresia is the leading cause of morbidity and mortality from pediatric liver disease and is the most common indication for pediatric liver transplantation. Despite its clinical importance, little is known of a wide range of fundamental issues regarding biliary atresia. Optimal approaches to the medical management of biliary atresia are unknown. No prospective randomized trials of important aspects of the care of children with biliary atresia have been performed. The majority of published data regarding biliary atresia is anecdotal and based upon a single center experience. Comprehensive registries of the management and outcome of biliary atresia do not exist. The Biliary Atresia Research Consortium (BARC) is an NIH-funded network of 9 clinical sites and a data coordinating center whose goal is to study biliary atresia, neonatal hepatitis and other cholestatic diseases in the infant. The primary objectives of this research are to establish (1) a database containing clinical information and (2) a repository of blood and tissue samples from children with neonatal liver diseases such as biliary atresia and neonatal hepatitis to facilitate research in these important liver problems in children. This study will: 1. collect detailed clinical and demographic information about each subject at enrollment and during follow up, 2. obtain and store blood and urine samples from the subject at diagnosis and during follow up, 3. obtain and store liver and biliary tissue and bile that are removed during diagnosis (i.e., biopsy) or at time of surgery or transplant and that are not needed for diagnostic purposes 4. collect demographic and medical history of parents at enrollment, and 5. obtain and store blood from the biological parents at enrollment.