A PROSPECTIVE DATABASE OF INFANTS WITH CHOLESTASIS

该子项目是利用 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来源获得主要资金， 因此可以在其他CRISP条目中表示。列出的机构是 中心，不一定是研究者的机构。 新生儿（出生时）胆汁淤积性疾病是一组发生在生命的前三个月内的肝胆疾病，其中胆汁流动受损。 总的来说，每2500例活产中就有1例患有新生儿胆汁淤积症。 新生儿胆汁淤积症的两个最常见的原因是胆道闭锁和特发性新生儿肝炎。 胆道闭锁是这些疾病中最常见的，发生率约为1/8000至1/15000活产，其特征是在出生后3个月内肝外胆道树（肝脏外）开口完全纤维化闭塞。 特发性新生儿肝炎是一个描述性术语，用于新生儿长期胆汁淤积的病例，在肝活检中存在特征性的“巨细胞肝炎”病变，并且没有发现其他感染，遗传，代谢或阻塞性原因。 在各种系列中，特发性新生儿肝炎可占所有新生儿胆汁淤积病例的30-40%。 虽然胆道闭锁和特发性新生儿肝炎是本项目的主要重点，但胆道闭锁临床研究联盟（BARC）可能会研究新生儿胆汁淤积的许多其他原因，从而可能对肝细胞（肝细胞）和胆道生理学和病理生理学产生新的认识和理解。 很明显，胆道闭锁和特发性新生儿肝炎的病因仍然知之甚少，新的诊断，预防和治疗策略的未来发展将需要更好地了解致病因素。 BARC将提供一个理想的环境，通过假设指导的调查，同时调查多个拟议的病因。

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. Neonatal (at birth) cholestatic disorders are a group of hepatobiliary diseases occurring within the first three months of life in which bile flow is impaired. Overall 1 in 2500 live births is affected with a neonatal cholestic disorder. The two most common causes of neonatal cholestasis are bilary atresia and idiopathic neonatal hepatitis. Biliary atresia is the most common of these disorders, occuring in approximately 1 in 8000 to 1 in 15,000 live births, and characterized by complete fibrotic obliteration of the opening of the extrahepatic biliary tree (outside the liver) within three months of life. Idiopathic neonatal hepatitis is a is a descriptive term used for cases of prolonged neonatal cholestasis in which the characteristic "giant cell hepatitis" lesion is present on liver biopsy, and in which no other infectious, genetic, metabolic, or obstructive cause is identified. In various series, idiopathic neonatal hepatitis may comprise up to 30-40% of all cases of neonatal cholestasis. Although biliary atresia and idiopathic neonatal hepatitis are the main focus of this project, there are many other causes of neonatal cholestasis that may be investigated by the Bilary Atresia Clinical Research Consortium (BARC) potentially leading to new knowledge and understanding of hepatocyte (liver cells) and biliary physiology and pathophysiology. It is clear that the etiologies of biliary atresia and idiopathic neonatal hepatitis remain poorly understood and that the future development of new diagnostic, preventative and therapeutic strategies will require a better understanding of the causative factors. BARC will provide an ideal environment in which to investigate multiple proposed etiologies simultaneously through hypothesis-directed investigations.