A PROSPECTIVE DATABASE OF INFANTS WITH CHOLESTASIS

点击翻译按钮获取中文摘要

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.