Arginine Deficiency-Induced Hyperammonemia in a Home Total Parenteral Nutrition-Dependent Patient: A Case Report

Abstract

Background: Patients with short bowel syndrome and renal dysfunction with TPN dependence are at high risk for developing hyperammonemia if the TPN does not contain sufficient quantities of arginine. Providing proper nutrition support is essential in the management of these patients. Methods: We report on a patient with short bowel syndrome, TPN dependence, and normal renal function who developed hyperammonemic encephalopathy due to inadvertent lack of arginine in his TPN. Results: The patient was successfully treated with hemodialysis and an IV arginine infusion to resolve the hyperammonemia. His home TPN was also adjusted such that arginine was added to his subsequent solutions. Conclusions: Our patient underscores the importance of adequate and sustained arginine supplementation to avoid hyperammonemia in TPN dependent patients with short bowel syndrome. ( Journal of Parenteral and Enteral Nutrition 25:286-288, 2001)

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References

Yamada E. , Wakabayashi Y. , Saito A. , et al: Hyperammonemia caused by essential amino acid supplements in patient with short bowel. Lancet 341:1542-1543, 1993

Yokoyama K. , Ogura Y. , Kawabata M. , et al: Hyperammonemia in a patient with short bowel syndrome and chronic renal failure. Nephron 72:693-695, 1996

Arginine Hydrochloride. IN American Hospital Formulary Service Drug Information. McEvoy GK (ed). American Society of Health-System Pharmacists, Inc., Bethesda, 1998, pp 2047-2049

Jeejeebhoy KN : Intestinal disorders: Short bowel syndrome . IN Modern Nutrition in Health and Disease, Shils ME , Olsen JA (eds). Lea & Febiger, Philadelphia, PA, 1994, pp 1036-1041

Halperin ML , Kamel K. : D-lactic acidosis: Turning sugar into acids in the gastrointestinal tract . Kidney Internat 49:1-8, 1995

Wilmore DW , Byrne TA , Persinger RL : Short bowel syndrome: New therapeutic approaches. Curr Prob Surg 34:428-444, 1997

Thurn JR , Pierpont GL , Ludvigsen CW , et al: D-lactate encephalopathy. Am J Med 79:717-721, 1985

Uribarri J. , Oh MS , Carroll HJ : D-Lactic acidosis. A review of clinical presentation, biochemical features, and pathophysiologic mechanisms. Med 77:73-82, 1998

Felig DM , Brusilow SW , Boyer JL : Hyperammonemic coma due to total parenteral nutrition in a woman with heterozygous ornithine transcarbamylase deficiency. Gastroenterology 109:282-284, 1995

10.

Wakabayashi Y. , Yamada E. , Hasegawa T. , et al: Enzymological evidence for the indispensibility of small intestine in the synthesis of arginine from glutamate. Arch Biochem Biophys 291:1-8, 1991

11.

Windmueller HG , Spaeth AE : Source and fate of circulating citrulline . Am J Physiol 241:473-480, 1981

12.

Matthews DE , Fong Y. : Amino acid and protein metabolism. IN Clinical Nutrition Parenteral Nutrition, 2nd edition. Rombeau JL , Caldwell MD (eds). W.B. Sanders Company, Philadelphia , 1993, pp 75-112

13.

Heird WC , Nicholson JF , Driscoll JM , et al: Hyperammonemia resulting from IV alimentation using a mixture of synthetic L-amino acids: A preliminary report. J Pediatr 81:162-165, 1972