Nutritional management of urea cycle disorders. Crit Care Clin 2005 Oct;21(4 Suppl):S27-35
Date
10/18/2005Pubmed ID
16227113DOI
10.1016/j.ccc.2005.08.003Scopus ID
2-s2.0-26844432821 (requires institutional sign-in at Scopus site) 48 CitationsAbstract
Nutritional management of patients who have urea cycle disorders is one of the most challenging tasks in clinical nutrition. The degree to which protein intake should be restricted in urea cycle disorders requires complex calculations which depend on many variables such as specific enzyme defect, age-related growth rate, current health status, level of physical activity, amount of free amino acids administered, energy intake, residual urea cycle function, family lifestyle, use of nitrogen-scavenging medications, and the patient's eating behaviors. This paper presents two case histories and a series of recommendations outlining the nutrition management of urea cycle disorders. It also identifies difficulties that arise in the course of treatment, and suggests practical solutions for overcoming them.
Author List
Singh RH, Rhead WJ, Smith W, Lee B, Sniderman King L, Summar MAuthor
William Rhead MD, PhD Adjunct Professor in the Pediatrics department at Medical College of WisconsinMESH terms used to index this publication - Major topics in bold
Acute DiseaseAdolescent
Adult
Amino Acid Metabolism, Inborn Errors
Child
Child, Preschool
Chronic Disease
Citrullinemia
Female
Humans
Hyperammonemia
Infant, Newborn
Male
Ornithine Carbamoyltransferase Deficiency Disease
Practice Guidelines as Topic
Urea
