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Ornithinemia, Hyperammonemia, and HomocitrullinuriaA Disease Associated With Mental Retardation and Possibly Caused by Defective Mitochondrial Transport
Vanessa Fell;
Rodney J. Pollitt, PhD;
Gwyneth A. Sampson, MRCPsych;
Trevor Wright, MD
Am J Dis Child. 1974;127(5):752-756.
Abstract
The effects of varying dietary protein intake, and of supplements of ornithine, arginine, lysine, and proline on a patient with hyperornithinemia, hyperammonemia, and homocitrullinuria were investigated in an attempt to understand the condition. The homocitrulline excretion is increased with increased dietary lysine, and also by increased protein intake. Supplementary ornithine or arginine lowers plasma ammonia levels and simultaneously decreases the excretion of homocitrulline. A possible cause of this disease is a defect in the transport of ornithine into the mitochondria. Arginine supplements may be useful in the treatment of the condition.
Author Affiliations
Sheffield, England
From the Medical Research Council Unit for Metabolic Studies in Psychiatry, University Department of Psychiatry, Middlewood Hospital, and the Ryegate Centre, Children's Hospital, Sheffield, England.
Footnotes
Received for publication Sept 5, 1973; accepted Nov 8.
Reprint requests to Medical Research Council Unit for Metabolic Studies in Psychiatry, PO Box 134, Middlewood Hospital, Sheffield, S6 ITP, England (Dr. Pollitt).
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