A Rare Case of Hyperammonemia Complication of High-Protein Parenteral Nutrition

Abstract

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.

Keywords

hyperammonemia parenteral nutrition urea cycle disorders

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References

LaBuzetta

Yao

Bourque

Zivin

. Adult non-hepatic hyperammonemia: a case report and differential diagnosis. Am J Med. 2010;123:885-891.

Mäkelä

Lang

Korpela

. Toxic encephalopathy with hyperammonaemia during high-dose salicylate therapy. Acta Neurol Scand. 1980;61(3):146-156.

Ambrosetto

Riva

Baruzzi

. Hyperammonemia in asterixis induced by carbamazepine: two case reports. Acta Neurol Scand. 1984;69(3): 186-189.

TzBertrand

Faro

Cantwell Pakis

. Intravenous ribavirin and hyperammonaemia in an immunocompromised patient infected with adenovirus. Pharmacotherapy. 2000;20(10):1216-1220.

Sekas

Paul

. Hyperammonemia and carnitine deficiency in a patient receiving sulfadiazine and pyrimethamine. Am J Med. 1993;95(1):112-113.

Ryder

Olson

Kahnoski

Karn

Oei

. Hyperammonemia after transurethral resection of the prostate: a report of 2 cases. J Urol. 1984;132(5):995-997.

Clay

Hainline

. Hyperammonemia in the ICU. Chest. 2007; 132(4):1368-1378.

Ben-Ari

Dalal

Morry

. Adult onset ornithine transcarbamylase (OTC) deficiency unmasked by the Atkins’ diet. J Hepatol. 2010;52(2):292-295.

Bates

Lewis

Burnett

. Late-onset carbamoyl phosphate synthetase 1 deficiency in an adult cured by liver transplantation. Liver Transpl. 2011;17(12):1481-1484.

10.

Treem

. Inherited and acquired syndromes of hyperammonaemia and encephalopathy in children. Semin Liver Dis. 1994;14(3):236-258.

11.

Batshaw

. Hyperammonemia. Curr Probl Pediatr. 1984;14(11):1-69.

12.

Kotani

Shiota

Umemoto

Tsuritani

Hoshiai

. Carbamyl phosphate synthetase deficiency and postpartum hyperammonemia. Am J Obstet Gynecol. 2010;203(1):e10-e11.

13.

Shawcross

Jalan

. The pathophysiologic basis of hepatic encephalopathy: central role for ammonia and inflammation. Cell Mol Life Sci. 2005;62(19-20):2295-2304.

14.

Ong

Aggarwal

Krieger

. Correlation between ammonia levels and the severity of hepatic encephalopathy. Am J Med. 2003;114(3):188-193.

15.

Chang

Fang

Chen

Huang

. Continuous venovenous haemofiltration in hyperammonaemic coma of an adult with non-diagnosed partial ornithine transcarbamylase deficiency. Nephrol Dial Transplant. 1999;14(5):1282-1284.

16.

Leonarda

McKiernanb

. The role of liver transplantation in urea cycle disorders. Mol Genet Metab. 2004;81:S74-S78.

17.

Morioka

Kasahara

Takada

. Current role of liver transplantation for the treatment of urea cycle disorders: a review of the worldwide English literature and 13 cases at Kyoto University. Liver Transpl. 2005;11(11):1332-1342.

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