TREATMENT GUIDELINES

The treatment of UCD patients requires a highly coordinated team of specialists trained in caring for patients with inborn errors of metabolism. Emergency management of patients in hyperammonemic coma resulting from a UCD is based on three interdependent principles:

1. Physical removal of the ammonia by dialysis or some form of hemofiltration.
2. Reversal of the catabolic state through caloric supplementation and in extreme cases, hormonal suppression (glucose/insulin drip).
3. Pharmacologic scavenging of excess nitrogen.

These are not consecutive but should be pursued independently in parallel as quickly as possible.¹

To access UCD treatment guideline on the NIH funded Urea Cycle Disorders Consortium web site click here.

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Reference

1. Rare Diseases Clinical Research Network. Urea Cycle Disorders Consortium. Treatment guidelines. Available at: http://rarediseasesnetwork.epi.usf.edu/ucdc/physicians/guidelines-main.htm. Accessed October 2, 2007.