The nutritional management of urea cycle disorders - 04/05/12
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. (J Pediatr 2001;138:S40-S45)
Le texte complet de cet article est disponible en PDF.Plan
Reprint requests: James Leonard, PhD, FRCP, Biochemistry, Endocrinology and Metabolism Unit, Institute of Child Health, 30 Guilford St, London WC1N 1EH, UK. |
Vol 138 - N° 1S
P. S40-S45 - janvier 2001 Retour au numéroBienvenue sur EM-consulte, la référence des professionnels de santé.
L’accès au texte intégral de cet article nécessite un abonnement.
Déjà abonné à cette revue ?