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 are to establish (1) a database containing clinical information and (2) a repository of blood and tissue samples from children with neonatal liver diseases such as biliary atresia and neonatal hepatitis to facilitate research in these important liver problems in children. This database and the specimens will be available for ancillary studies that will investigate 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. This study will: 1. collect detailed clinical and demographic information about each subject at enrollment and during follow up, 2. obtain and store blood and urine samples from the subject at diagnosis and during follow up, 3. obtain and store liver and biliary tissue and bile that are removed during diagnosis (i.e., biopsy) or at time of surgery or transplant and that are not needed for diagnostic purposes 4. collect demographic and medical history of parents at enrollment, and 5. obtain and store blood from the biological parents at enrollment. Samples of blood, urine, bile and tissue will be stored in repositories for future research. The data and biological specimens will be used for detailed study into the mechanisms and causes of liver problems in young children in order to try to better diagnose and manage these conditions. The subject will receive standard-of-care treatment and will not be restricted in type of treatment or from changes in treatment, such as newer treatments as they are developed. The subjects may not directly benefit from participation in this research, but in the future other children with similar problems may benefit from new information that may lead to better medical care.
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