Journal of Inherited Metabolic Disease

, Volume 28, Issue 6, pp 1133–1135

Hyperammonaemic encephalopathy induced by a commercial very-low-energy diet in a neglected ornithine-carbamoyltransferase-deficient woman

Authors

    • Department of Internal Medicine and Nutrition, Hôpital de HautepierreUniversité Louis Pasteur de Strasbourg
    • Service de Médecine interne et de NutritionHôpital de Hautepierre
  • A. E. Perrin
    • Department of Internal Medicine and Nutrition, Hôpital de HautepierreUniversité Louis Pasteur de Strasbourg
  • E. Forestier
    • Department of Internal Medicine and Nutrition, Hôpital de HautepierreUniversité Louis Pasteur de Strasbourg
  • J. L. Schlienger
    • Department of Internal Medicine and Nutrition, Hôpital de HautepierreUniversité Louis Pasteur de Strasbourg
  • B. Goichot
    • Department of Internal Medicine and Nutrition, Hôpital de HautepierreUniversité Louis Pasteur de Strasbourg
Case Report

DOI: 10.1007/s10545-005-0209-4

Cite this article as:
Vinzio, S., Perrin, A.E., Forestier, E. et al. J Inherit Metab Dis (2005) 28: 1133. doi:10.1007/s10545-005-0209-4

Summary

This case report describes a 31-year-old woman with a neglected ornithine carbamoyltransferase deficiency, admitted for a hyperammonaemic encephalopathy induced by a commercial very low-energy diet. To our knowledge, this is the first reported case and it underlines the potential risk of such a diet in patients with undiagnosed or neglected ornithine carbamoyltransferase deficiency.

Copyright information

© SSIEM and Springer 2005