The Abernethy malformation is an extremely rare congenital, extrahepatic, portosystemic shunt. There are many problems associated with this abnormal portovenous shunting and subsequent reduced hepatic portal venous flow. With the advances in non-invasive imaging technologies, these cases diagnosed more numbers; however, presentation of patients varied natural history not completely known. prese...

RATIONALE. With no effective clearance system for ammonia, levels increase rapidly, resulting in cerebral edema with severe neurologic compromise. Dialysis is the only means of rapid removal of ammonia from blood in acute neonatal hyperammonemia, and hemodialysis is preferred over peritoneal dialysis because it is much more effective. If hemodialysis is not available, hemofiltration can be used...

The spf-ash mutation in mice results in reduced hepatic and intestinal ornithine transcarbamylase. However, a reduction in enzyme activity only translates in reduced ureagenesis and hyperammonemia when an unbalanced nitrogen load is imposed. Six-week-old wild-type control and spf-ash mutant male mice from different genetic backgrounds (B6 and ICR) were infused intravenously with [(13)C(18)O]ure...