DATABASE OF INFANTS WITH CHOLESTASIS

胆汁淤积婴儿数据库

• 批准号: 7604617
• 负责人: KATHLEEN SCHWARZ
• 金额: $ 0.03万
• 依托单位: JOHNS HOPKINS UNIVERSITY
• 依托单位国家: 美国
• 财政年份: 2006
• 资助国家: 美国
• 起止时间: 2006-12-01 至 2007-09-16
• 项目状态: 已结题
• 来源: https://reporter.nih.gov/project-details/7604617
• 关键词: Address; Adult; Affect; Age-Years; Attention; Bile fluid; Biliary; Biliary Atresia; Blood; Child; Childhood; Cholestasis; Clinical; Communities; Computer Retrieval of Information on Scientific Projects Database; Cytoprotection; Databases; Development; Diagnosis; Disease; Etiology; Family; Female; Funding; Future; Gene Proteins; Genomics; Grant; Hepatobiliary; Infant; Injury; Institution; Investigation; Knowledge; Life; Liver; Liver diseases; Natural History; Neonatal; Pathogenesis; Pattern; Plasma; Population Study; Process; Proteomics; Recovery; Research; Research Personnel; Resources; Serum; Siblings; Source; Specimen; Techniques; Time; Tissue Banks; Tissue Sample; Tissues; United States National Institutes of Health; Viral; clinical research site; day; improved; liver transplantation; male; neonatal hepatitis; novel; protein expression; repository; research study; tool

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.

这个子项目是许多研究子项目中的一个 由NIH/NCRR资助的中心赠款提供的资源。子项目和 研究者（PI）可能从另一个NIH来源获得了主要资金， 因此可以在其他CRISP条目中表示。所列机构为 研究中心，而研究中心不一定是研究者所在的机构。 本研究的主要目的是建立（1）包含临床信息的数据库和（2）来自新生儿肝病（如胆道闭锁和新生儿肝炎）儿童的血液和组织样本库，以促进对这些重要儿童肝脏问题的研究。使用该数据库和知识库的例子是研究胆道闭锁和新生儿肝炎的发病机制和自然史，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表达的模式。 研究人群将包括在胆道闭锁研究联盟（BARC）临床研究中心诊断时小于或等于180日龄的胆汁淤积婴儿（男性和女性）。为了研究自然史，将对受试者进行随访，直至10岁，接受肝移植，或者对于无胆道闭锁的儿童，直至所有治疗完全恢复。 多中心的研究者网络将解决病因学，发病机制，自然史，诊断和新的治疗方法的问题，最具破坏性的儿科疾病之一，发生在生命的最初几个月，胆道闭锁，和相关疾病，如特发性新生儿肝炎。虽然这些疾病并不常见，但对儿童，兄弟姐妹及其家庭的影响以及频繁的肝移植需求，要求科学界比过去更关注。此外，从这些研究中获得的知识很有可能影响胆汁淤积性肝病成人的诊断和治疗，并提高我们对调节胆汁流动，细胞保护以及肝脏和胆道发育的基本过程的理解。从患有各种新生儿胆汁淤积性疾病的儿童中建立血清和组织标本库，将是目前和未来研究婴儿肝胆损伤病因和发病机制的宝贵工具。