The nutritional management of urea cycle disorders.

Institute of Child Health and Great Ormond Street Hospital for Children, London, United Kingdom.
Journal of Pediatrics (Impact Factor: 3.74). 02/2001; 138(1 Suppl):S40-4;discussion S44-5. DOI: 10.1067/mpd.2001.111835
Source: PubMed

ABSTRACT Diet is one of the mainstays of the treatment of patients with urea cycle disorders. The protein intake should be adjusted to take account of the inborn error and its severity and the patient's age, growth rate, and individual preferences. Currently, the widely used standards for protein intake are probably more generous than necessary, particularly for those with the more severe variants. Most patients, except those with arginase deficiency, will need supplements of arginine, but the value of other supplements including citrate and carnitine is unclear. Any patient on a low-protein diet should be monitored clinically and with appropriate laboratory tests. All should have an emergency (crisis) regimen to prevent decompensation during periods of metabolic stress.

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