Nutritional management of patients with urea cycle disorders.
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.
Singh
R H
RH
Emory Genetics Metabolic Nutrition Program, Department of Human Genetics, Emory University, 2165 North Decatur Road, Decatur, GA 30033, USA. rsingh@genetics.emory.edu
D000293:Adolescent; D000328:Adult; D000641:Ammonia / Q000378:metabolism*; D002648:Child; D002675:Child, Preschool; D004035:Diet Therapy / Q000379:methods; D004798:Enzymes / Q000172:deficiency* / Q000378:metabolism; D005260:Female; D006801:Humans; D022124:Hyperammonemia / Q000150:complications* / Q000175:diagnosis* / Q000178:diet therapy; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D008661:Metabolism, Inborn Errors / Q000150:complications* / Q000175:diagnosis* / Q000178:diet therapy; D008875:Middle Aged; D009422:Nervous System Diseases / Q000150:complications* / Q000175:diagnosis* / Q000178:diet therapy; D009584:Nitrogen / Q000378:metabolism; D052756:Nutritional Sciences; D016896:Treatment Outcome; D014508:Urea / Q000378:metabolism*
D004798:Enzymes; D000641:Ammonia; D014508:Urea; D009584:Nitrogen