Advertisement

Nutritional Management of Urea Cycle Disorders

      To read this article in full you will need to make a payment

      Purchase one-time access:

      Academic & Personal: 24 hour online accessCorporate R&D Professionals: 24 hour online access
      One-time access price info
      • For academic or personal research use, select 'Academic and Personal'
      • For corporate R&D use, select 'Corporate R&D Professionals'

      Subscribers receive full online access to your subscription and archive of back issues up to and including 2002.

      Content published before 2002 is available via pay-per-view purchase only.

      Subscribe:

      Subscribe to Critical Care Clinics
      Already a print subscriber? Claim online access
      Already an online subscriber? Sign in
      Institutional Access: Sign in to ScienceDirect

      References

        • Brusilow S.W.
        Treatment of urea cycle disorders.
        in: Desnick R.J. Treatment of genetic diseases. Churchill Livingstone, New York1991: 79-94
        • Zimmerman A.
        • Bachmann C.
        • Baumgartner R.
        Severe liver fibrosis in argininosuccinic aciduria.
        Arch Pathol Lab Med. 1986; 110: 136-140
        • Pupim L.B.
        • Flakoll P.J.
        • Ikizler T.A.
        Protein homeostasis in chronic hemodialysis patients.
        Curr Opin Clin Nutr Metab Care. 2004; 7: 89-95
      1. Energy and protein requirements. World Health Organization, Geneva, Switzerland1985
        • Singh R.H.
        • Elsas L.J.
        Nutrition support of patients with disorders of the urea cycle.
        Met Currents. 1994; 7: 1-6
        • Scaglia F.
        • Carter S.
        • O'Brien W.E.
        • et al.
        Effect of alternative pathway therapy on branced chain amino acid metabolism in urea cycle disorder patients.
        Mol Genet Metab. 2004; 81: S79-S85
        • Acosta P.B.
        • Yannicelli S.
        Nutrition support protocols. Ross Products Division, Abbott Laboratories, Columbus, OH2001
        • U.S. Department of Agriculture, Agricultural Research Service
        USDA national nutrient database for standard reference, release 17.
        (Available at:) (Accessed June 8, 2005)
        • Grody W.W.
        • Chang R.J.
        • Panagiotis N.M.
        • et al.
        Menstrual cycle and gonadal steroid effects on symptomatic hyperammonaemia of urea-cycle-based and idiopathic aetiologies.
        J Inherit Metab Dis. 1994; 17: 566-574
        • Wong L.J.
        • Craigen W.J.
        • O'Brien W.E.
        Postpartum coma and death due to carbamoly-phosphate synthetase I deficiency.
        Ann Intern Med. 1994; 120: 216-217
        • Arn P.H.
        • Hauser E.R.
        • Thomas G.H.
        • et al.
        Hyperammonemia in women with a mutation at the ornithine carbamoyltransferase locus.
        N Engl J Med. 1990; 322: 1652-1655
        • Souba W.W.
        • Wilmore D.
        Diet and nutrition in the care of the patient with surgery, trauma and sepsis.
        in: Shils M.E. Olson J.A. Shike M. Modern nutrition in health and disease. 9th edition. Williams & Wilkins, Baltimore (MD)1999: 1589-1618
        • Felig D.M.
        • Brusilow S.W.
        • Boyer J.L.
        Hyperammonemic coma due to parenteral nutrition in a woman with heterozygous ornithine transcarbamylase deficiency.
        Gastroenterology. 1995; 109: 282-284
        • Leão M.
        Valproate as a cause of hyperammonemia in heterozygotes with ornithine-transcarbamylase deficiency.
        Neurology. 1995; 45: 593-594
        • Oechsner M.
        • Steen C.
        • Stürenburg H.J.
        • et al.
        Hyperammonaemic encephalopathy after initiation of valproate therapy in unrecognized ornithine transcarbamylase deficiency.
        J Neurol Neurosurg Psychiatry. 1998; 64: 680-682
        • Sewell A.C.
        • Böhles H.J.
        • Herwig J.
        • et al.
        Neurological deterioration in patients with urea cycle disorders under valproate therapy—a cause for concern.
        Eur J Pediatr. 1995; 154: 593-594
        • Stephens J.R.
        • Levy R.H.
        Effects of valproate and citrulline on ammonium-induced encephalopathy.
        Epilepsia. 1994; 35: 164-171
        • Verbiest H.B.C.
        • Straver J.S.
        • Colombo J.P.
        • et al.
        Carbamyl phosphate synthetase-1 deficiency discovered after valproic acid-induced coma.
        Acta Neurol Scand. 1992; 86: 275-279
        • Mori T.
        • Tsuchiyama A.
        • Nagai K.
        • et al.
        A case of carbamylphosphate synthetase-I deficiency associated with secondary carnitine deficiency—L-carnitine treatment of CPS-I deficiency.
        Eur J Pediatr. 1990; 149: 272-274