Management
Management of ARG1-D requires an integrated approach
The multidisciplinary team around an ARG1-D patient should include not only specialists in metabolic disorders, neurology and genetics, but also dietitians, physiotherapists and other healthcare and allied health professionals1
The current standard of care for ARG1-D relies on symptomatic treatment including dietary protein restriction, food supplements containing essential amino acids and nitrogen scavengers2,3
- NORD Guides for Physicians. The Physician’s Guide to Urea Cycle Disorders. 2012. Available at: https://nucdforg.presencehost.net/file_download/79b6b024-9942-4e78-bcb7-24d6a77eeeeb. Accessed November 14, 2023.
- Haberle J, et al. Suggested guidelines for the diagnosis and managemet of urea cycle disorders. Suggested Orphanet J Rare Dis. 2012;7:32.
- Cederbaum SD, et al. Treatment of Hyperargininaemia due to Arginase Deficiency with a Chemically Defined Diet. J Inherit Metab Dis. 1982;5:95-99.