Abstract Fibrocystic liver disease refers to a heterogeneous group of disorders with shared, but also distinct, pathophysiologic and clinical features. Cystic dilatation of intrahepatic bile duct structures and variable degrees…
Abstract Management in the early postoperative period requires the coordinated efforts of the transplant team and the pediatric intensive care staff, and other key ancillary services, such as interventional radiology,…
Abstract Liver transplantation is the standard of care for end-stage liver disease in children and the vast majority of transplant recipients experience successful outcomes. Better preoperative care, enhanced surgical techniques,…
Abstract Pathologies of mitochondrial structure and function are now recognized as the etiology of a growing list of monogenic mitochondrial disorders as well as contributing to many common diseases. Studying…
Abstract The importance of bile acid synthesis and metabolism to normal physiology and their role in pathophysiological states is well recognized. For such small and relatively simple molecules, bile acids…
Abstract The liver is the largest parenchymal organ in the body and has a complicated circulatory system, with dual afferent blood supply from the portal vein and hepatic artery. Although…
Abstract Non-alcoholic fatty liver disease (NAFLD) is now the most common cause of chronic liver disease in both adults and children worldwide [1]. Increasing industrialization and commercial globalization in Asia,…
Abstract Peroxisomes are ubiquitous subcellular organelles that are found in essentially all animal and plant cells with the exception of mature anucleated erythrocytes. They carry out many essential biochemical processes,…
Abstract Mitochondrial fatty acid β-oxidation (FAO) is an essential component of energy production and homeostasis in humans. During periods of limited glucose supply, FAO in the liver provides energy for…
Abstract Lysosomes are membrane-bound cellular organelles that contain multiple hydrolases needed for the digestion of various macromolecules including mucopolysaccharides, glycosphingolipids, cholesterol esters and oligosaccharides. Moreover, the lysosome is emerging as…
