This acute illness protocol is a guideline for healthcare professionals treating patients with arginase deficiency and presenting to the emergency department with illness or hyperammonemia. Arginase deficiency is a urea cycle defect (UCD). The protocol was developed under the direction of Dr. Harvey Levy, Senior Associate in Medicine/Genetics at Children’s Hospital Boston, and Professor of Pediatrics at Harvard Medical School.
Hyperammonemic crises in patients with UCD are medical emergencies and must be treated as such to avoid death or serious brain injury.
Click to view the complete protocol as a PDF
Disclaimer
The care of patients with metabolic disorders is complex and it is essential that a metabolic specialist be contacted as rapidly as possible. This protocol is only a guideline and should not be used for definitive treatment without metabolic consultation. Please read our Terms of Use.