DATABASE OF INFANTS WITH CHOLESTASIS

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。 这项研究的主要目标是建立(1)包含临床信息的数据库和(2)来自患有胆道闭锁和新生儿肝炎等新生儿肝病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。使用该数据库和资料库的例子包括研究胆道闭锁和新生儿肝炎的发病机制和自然病史，或通过病毒、基因组和蛋白质组学技术评估组织标本和血浆中细胞基因和蛋白质的表达模式。 研究人群将包括在胆道闭锁研究联盟(BARC)临床站点确诊时小于或等于180天的胆汁淤积婴儿，男性和女性。为了研究自然历史，受试者将被跟踪到10岁，进行肝移植，或者对于没有胆道闭锁的儿童，直到完全从所有治疗中恢复。 多中心研究人员网络将解决病因、发病机制、自然病史、诊断以及对出生后几个月发生的最具破坏性的儿科疾病之一、胆道闭锁和相关疾病(如特发性新生儿肝炎)的新疗法等问题。尽管这些疾病并不常见，但对儿童、兄弟姐妹及其家庭的影响以及对肝脏移植的频繁需求，要求科学界比过去更多地关注。此外，从这些研究中获得的知识很有可能影响成人胆汁淤积性肝病的诊断和治疗，并提高我们对调节胆汁流动、细胞保护以及肝脏和胆管发育的基本过程的理解。各种新生儿胆汁淤积症患儿血清和组织标本库的建立将为目前和未来研究婴儿肝胆损伤的病因和发病机制提供宝贵的工具。

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. The primary objectives of this research study are to establish (1) a database containing clinical information and (2) a repository of blood and tissue samples from children with neonatal liver disease such as biliary atresia and neonatal hepatitis to facilitate research in these important liver problems in children. Examples of the use of this database and repository are to study the pathogenesis and natural history of biliary atresia and neonatal hepatitis or to evaluate patterns of cellular gene and protein expression in tissue specimens and plasma by viral, genomic and proteomic techniques. The study population will consist of infants, both male and female, with cholestasis who are less than or equal to 180 days old at the time of diagnosis at a Biliary Atresia Research Consortium (BARC) clinical site. In order to study the natural history, subjects will be followed until 10 years of age, liver transplantation or, for children without biliary atresia, until complete recovery off of all therapy. The multi-center network of investigators will address issues of etiology, pathogenesis, natural history, diagnosis, and novel treatments for one of the most devastating pediatric illnesses that occur in the first few months of life, biliary atresia, and related disorders such as idiopathis neonatal hepatitis. Although these disorders are not common, the effects on children, siblings, and their families and the frequent need for Liver transplantation, mandate a greater degree of attention by the scientific community than in the past. Moreover, knowledge gained from these investiagtions has a high likelihood of affecting diagnosis and treatment of adults with cholestatic liver disease and improving our understanding of fundamental processes regulating bile flow, cytoprotection, and liver and biliary development. The development of a serum and tissue bank of specimens from children with various neonatal cholestatic disorders will be invaluable tool for current and future investigations into the etiology and pathogenesis of hepatobiliary injury to the infant.