BILIARY ATRESIA STUDY (BASIC)

胆道闭锁研究（基础）

• 批准号: 7607801
• 负责人: JOHN C BUCUVALAS
• 金额: $ 0.56万
• 依托单位: CINCINNATI CHILDRENS HOSP MED CTR
• 依托单位国家: 美国
• 财政年份: 2007
• 资助国家: 美国
• 起止时间: 2007-02-15 至 2007-11-30
• 项目状态: 已结题
• 来源: https://reporter.nih.gov/project-details/7607801
• 关键词: Adult; Autoimmune Process; Bile fluid; Biliary; Biliary Atresia; Body Fluids; Child; Cirrhosis; Clinical; Computer Retrieval of Information on Scientific Projects Database; Disease; Disease Progression; Funding; Genetic; Genetic Materials; Grant; Infant; Inflammatory; Institution; Live Birth; Natural History; Obstruction; Procedures; Process; Purpose; Research; Research Personnel; Resources; Sampling; Source; Teenagers; United States National Institutes of Health; biliary tract; liver transplantation; neonate

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 (BA) is a progressive inflammatory process involving the biliary tree. As the disease progresses, there is loss of patency of the lumen and obstruction to bile flow. BA occurs in one in 8-15,000 live births resulting in 250-400 new cases per year in the US. Untreated, the disease leads to complete biliary obstruction with cirrhosis, and is uniformly fatal. After a hepatoportoenterostomy (Kasai procedure), children have a variable disease progression with less than 20% surviving beyond the teen years without liver transplantation. Little is known about the factors that cause biliary atresia nor the factors that influence disease progression. A variety of genetic, autoimmune and environmental influences have been hypothesized to be important. Most studies to date have focused on the neonate and young child with BA, yet the older surviving child with BA can provide important information about genetics, as well as, natural history. The purpose of this study is to collect the pertinent clinical information, genetic material and body fluid samples obtained from older infants, children and adults with 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。 胆道闭锁（BA）是一种涉及胆道树的进行性炎症过程。 随着疾病的进展，管腔失去通畅并且胆汁流动受阻。 BA 的发生率为八分之一至 15,000 名活产婴儿，导致美国每年新增 250 至 400 例病例。 如果不治疗，这种疾病会导致胆道完全梗阻并伴有肝硬化，并且都是致命的。肝门肠造口术（Kasai 手术）后，儿童的疾病进展情况各不相同，如果不进行肝移植，只有不到 20% 的儿童能在青少年时期存活下来。对于导致胆道闭锁的因素和影响疾病进展的因素知之甚少。 多种遗传、自身免疫和环境影响被认为是重要的。 迄今为止，大多数研究都集中在患有 BA 的新生儿和幼儿上，但幸存的患有 BA 的年长儿童可以提供有关遗传学和自然史的重要信息。 本研究的目的是收集从患有胆道闭锁的较大婴儿、儿童和成人获得的相关临床信息、遗传物质和体液样本。