Journal
JOURNAL OF PARENTERAL AND ENTERAL NUTRITION
Volume 37, Issue 1, Pages 134-137Publisher
SAGE PUBLICATIONS INC
DOI: 10.1177/0148607112447815
Keywords
hyperammonemia; parenteral nutrition; urea cycle disorders
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Hyperammonemia is a metabolic derangement that can be potentially fatal. Primary hyperammonemia due to urea cycle enzyme deficiency is usually discovered in neonates but rarely can present in adulthood. Late-onset manifestations of urea cycle disorders can go unnoticed, until they become life threatening. The authors report a 28-year-old man who developed hyperammonemia in the hospital following parenteral nutrition (PN), leading to cerebral edema, which was fatal despite resolution of the hyperammonemia with cessation of PN and the use of continuous renal replacement therapy. (JPEN J Parenter Enteral Nutr. 2013;37:134-137)
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