Summary
The nutritional management of patients with urea cycle disorders (UCDs) involves restriction of dietary protein along with provision of adequate protein-free energy, essential amino acid supplements, and vitamins and minerals in combination with nitrogen-scavenging drugs. The present paper discusses nutrition therapy for a range of circumstances: during an acute hyperammonaemic episode and at hospital discharge; before, during, and after surgery; and for lifelong chronic management of UCDs.
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Communicating editor: Michael Gibson
Competing interests: None declared
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Singh, R.H. Nutritional management of patients with urea cycle disorders. J Inherit Metab Dis 30, 880–887 (2007). https://doi.org/10.1007/s10545-007-0718-4
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DOI: https://doi.org/10.1007/s10545-007-0718-4