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UCD Guides

Medication and Formula Management

UCD MEDICATIONS

We would like to acknowledge the valuable contributions of our industry partners who have dedicated resources to improve the quality of life for individuals with urea cycle disorders. Twenty-five years ago, there was just one option available. Today, we are pleased to see a variety of choices for families, recognizing that a personalized approach is essential in meeting diverse needs.

  • Ravicti® (phenylbutyrate in a glycerol liquid) for treatment of UCDs. Buphenyl® powder or capsules (sodium phenylbutyrate) for treatment of UCD

  • Olpruva® (sodium phenylbutyrate) oral suspension for treatment of UCD

  • Pheburane® granules (coated pellets, tasteless sodium phenylbutyrate) for treatment of UCD

  • Generic sodium phenylbutyrate

  • Generic maintenance therapy for the treatment of chronic hyperammonemia due to NAGS deficiency

  • Maintenance therapy for the treatment of chronic hyperammonemia due to NAGS deficiency

Disclaimer: Information is provided for reference only and does not constitute an endorsement of any product or manufacturer by NUCDF.

MEDICAL FORMULAS FOR UCDs

SUPPLEMENTS FOR UCDs

CITRULLINE AND ARGININE

Note: only pharmaceutical grade citrulline and arginine are recommended for use in Urea Cycle Disorders. Over-the-counter products can be of questionable potency, containing fillers, and quality and are not recommended. Underdosing could result.

Montiff Inc. - Suppliers of pharmaceutical grade L-citrulline and L-arginine. 
(Gives discount to NUCDF families)
3205 Santa Monica Blvd.
Los Angeles, CA 90025
Phone: (877)820-4883 Fax: (310) 582-8939
www.montiff.com

Vitaflo - Suppliers of pharmaceutical-grade amino acid supplements (available by prescription)

Jo Mar Labs - Suppliers of pharmaceutical grade L-citrulline and L-arginine.

WARNING: 

Feb 14, 2014 FDA DRUG RECALL - CITRULLINE: 

L-citrulline manufactured by Medisca, a supplier to hospitals and pharmacies, was recalled. Lots 95482/A, 95482/B, 95482/C, 95482/D &96453/A, 96453/B, 96453/C, 96453/D contained ZERO citrulline. The product contained a different amino acid, L-acetyl-leucine. This resulted in reports of severe side effects including metabolic instability, hyperammonemia, hair loss, stomach pains and other symptoms. The FDA issued a warning and manufacturer recall HERE. Please notify your pharmacy and your metabolic physician immediately if you notice a change or difference in the usual color, consistency, or odor of UCD treatment amino acid supplements. The amino acid supplements can be tested for purity to ensure that the product contains the labeled quality/strength. If you suspect an issue with your citrulline or arginine, contact NUCDF for more information on how to get the product tested.

Disclaimer: Information is provided for reference only and does not constitute an endorsement of any product or manufacturer by NUCDF.

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