Nutritional management of patients with urea cycle disorders

J Inherit Metab Dis. 2007 Nov;30(6):880-7. doi: 10.1007/s10545-007-0718-4. Epub 2007 Nov 25.

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.

Publication types

  • Research Support, N.I.H., Extramural
  • Review

MeSH terms

  • Adolescent
  • Adult
  • Ammonia / metabolism*
  • Child
  • Child, Preschool
  • Diet Therapy / methods
  • Enzymes / deficiency*
  • Enzymes / metabolism
  • Female
  • Humans
  • Hyperammonemia / complications*
  • Hyperammonemia / diagnosis*
  • Hyperammonemia / diet therapy
  • Infant
  • Infant, Newborn
  • Male
  • Metabolism, Inborn Errors / complications*
  • Metabolism, Inborn Errors / diagnosis*
  • Metabolism, Inborn Errors / diet therapy
  • Middle Aged
  • Nervous System Diseases / complications*
  • Nervous System Diseases / diagnosis*
  • Nervous System Diseases / diet therapy
  • Nitrogen / metabolism
  • Nutritional Sciences
  • Treatment Outcome
  • Urea / metabolism*

Substances

  • Enzymes
  • Ammonia
  • Urea
  • Nitrogen