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J Inherit Metab Dis. 2007 Nov;30(6):880-7. Epub 2007 Nov 25.

Nutritional management of patients with urea cycle disorders.

Author information

1
Emory Genetics Metabolic Nutrition Program, Department of Human Genetics, Emory University, 2165 North Decatur Road, Decatur, GA 30033, USA. rsingh@genetics.emory.edu

Abstract

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.

PMID:
18034368
DOI:
10.1007/s10545-007-0718-4
[Indexed for MEDLINE]

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