TREATMENT GUIDELINES
The treatment of patients with UCDs requires a highly coordinated team of specialists trained in caring for patients with inborn errors of metabolism. The long-term goal of chronic management of UCDs is to maintain normal plasma ammonia levels while promoting normal growth and development.1
Chronic management includes:
- Dietary protein restriction
- Amino acid supplementation
- Pharmacotherapy to facilitate waste nitrogen excretion
Patients should be regularly monitored to assess how well their disease is being managed. In addition, a comprehensive plan should facilitate responding to a wide variety of needs and complications, which can include the following1
- Low-protein diet and medications
- Management of acute episodes
- Need for elective surgery
- Neurologic and developmental evaluation
- Feeding evaluation and therapy
- Physical and occupational therapy
- Speech therapy
- School and educational services
- Social services
- Psychological services
- Genetic counseling
To access UCD treatment guideline on the NIH funded Urea Cycle Disorders Consortium web site click here.
Click here for important safety information
Click here for Full Prescribing Information
Reference
1. Berry GT, Steiner RD. Long-term management of patients with urea cycles disorders.
J Pediatr. 2001;138:S56-S61.
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