Pathologic Manifestations Congenital and acquired alterations in hepatic structure and function (acute or chronic) can be manifest by varying patterns of reaction of the liver to cell injury. Hepatocyte injury can be caused by viral infection, drugs or toxins, hypoxia,…
An inherited metabolic disorder is an increasingly common indication for liver transplantation (LT), accounting for more than 15% of pediatric cases worldwide and up to 30% in some specialist centers. Urea cycle disorders are now the most common indication in…
Introduction Liver transplantation has become the standard of care for children with an array of disease processes. Collectively, progressive hepatic disease with complications of end-stage liver disease (ESLD), metabolic disease with and without hepatic structural involvement, pediatric acute liver failure…
Abbreviations A1AT α 1 -antitrypsin ABC ATP-binding cassette AGS Alagille syndrome ASBT apical sodium-dependent bile acid transporter BRIC benign recurrent intrahepatic cholestasis CMV cytomegalovirus FIC1 familial intrahepatic cholestasis 1 JAG1 jagged-1 gene MCT medium-chain triglyceride MDR3 multidrug resistance protein 3 MMR…
Metabolic liver diseases may manifest as acute, life-threatening illnesses in the neonatal period or as chronic liver disease in adolescence or adulthood, with progression to liver failure, cirrhosis, or HCC. In a 2015 report of the Scientific Registry of Transplant…
Anatomy THE LIVER AND BILIARY TREE are derived from the endoderm of the dorsal foregut during the late third to the early fourth week of gestation. By the sixth week, the fetal liver primarily serves as a hematopoietic organ, while…