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Nutritional management of patients with urea cycle disorders
Author(s) -
Singh R. H.
Publication year - 2007
Publication title -
journal of inherited metabolic disease
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.462
H-Index - 102
eISSN - 1573-2665
pISSN - 0141-8955
DOI - 10.1007/s10545-007-0718-4
Subject(s) - urea cycle , medicine , urea , dietary management , intensive care medicine , pediatrics , chemistry , amino acid , biochemistry , arginine
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.