A PROSPECTIVE DATABASE OF INFANTS WITH CHOLESTASIS

胆汁淤积婴儿的前瞻性数据库

• 批准号: 7718122
• 负责人: FREDERICK J SUCHY
• 金额: $ 0.17万
• 依托单位: ICAHN SCHOOL OF MEDICINE AT MOUNT SINAI
• 依托单位国家: 美国
• 财政年份: 2008
• 资助国家: 美国
• 起止时间: 2008-03-01 至 2009-02-28
• 项目状态: 已结题
• 来源: https://reporter.nih.gov/project-details/7718122
• 关键词: Affect; Bile fluid; Biliary; Biliary Atresia; Birth; Characteristics; Cholestasis; Clinical Research; Computer Retrieval of Information on Scientific Projects Database; Databases; Development; Disease; Environment; Etiology; Extrahepatic; Functional disorder; Funding; Future; Genetic; Giant Cells; Grant; Hepatitis; Hepatobiliary; Hepatocyte; Infant; Institution; Investigation; Knowledge; Lesion; Life; Live Birth; Liver; Metabolic; Neonatal; Physiology; Research; Research Personnel; Resources; Series; Source; Therapeutic; United States National Institutes of Health; biliary tract; liver biopsy; neonatal hepatitis; novel diagnostics; prospective

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.

该副本是利用众多研究子项目之一 由NIH/NCRR资助的中心赠款提供的资源。子弹和 调查员（PI）可能已经从其他NIH来源获得了主要资金， 因此可以在其他清晰的条目中代表。列出的机构是 对于中心，这不一定是调查员的机构。 新生儿（出生时）胆汁淤积性疾病是一组在生命后三个月内发生的一组肝胆疾病，其中胆汁流量受损。 总体上有2500名活产的1分之一受到新生儿胆固性疾病的影响。 新生儿胆汁淤积的两个最常见原因是双性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，大约有15,000个活生生中的8000至1分之一，其特征是在生命的三个月内完全纤维化的肝外胆汁胆树（肝脏以外）开放。 特发性新生儿肝炎是一种描述性术语，用于长时间的新生儿胆汁淤积病例，其中特征性的“巨型细胞肝炎”病变存在于肝活检中，并且没有标识其他感染性，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能占新生儿胆汁淤积病例的30-40％。 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. 显然，胆道闭锁和特发性新生儿肝炎的病因仍然鲜为人知，并且未来的新诊断，预防和治疗策略的未来发展将需要更好地了解因果关系。 BARC将提供一个理想的环境，在该环境中，通过假设指导的研究同时研究多个提出的病因。