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Attempted dietary treatment of a boy with hyperammonemia due to ornithine transferase deficiency

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Abstract

Dietary treatment of a male patient suffering from the delayed-onset type of OCT deficiency was attempted. Control of the hyperammonemia was attempted by restriction of protein intake, guided by monitoring the plasma ammonia and regular checking of the serum amino acid levels. The influence of supplementary citric acid or lactulose therapy on the plasma ammonia level was investigated and found to be negligible. The therapeutic effect of supplying ornithine and arginine (an essential amino acid in urea cycle disorders) is described. Despite intensive dietary treatment over two and a half years, a incorrigible hyperammonemic crisis resulted in the sudden death of our patients.

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Authors and Affiliations

  1. Het Wilhelmina Kinderziekenhuis, University Children's Hospital, Nieuwe Gracht 137, Utrecht, The Netherlands

    C. van der Heiden, J. Desplanque, M. Brink, P. K. de Bree & S. K. Wadman

  2. Het Emma Kinderziekenhuis, Children's Hospital, Spinozastraat 52, Amsterdam, The Netherlands

    H. D. Bakker

Authors
  1. C. van der Heiden
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  2. H. D. Bakker
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  3. J. Desplanque
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  4. M. Brink
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  5. P. K. de Bree
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  6. S. K. Wadman
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van der Heiden, C., Bakker, H.D., Desplanque, J. et al. Attempted dietary treatment of a boy with hyperammonemia due to ornithine transferase deficiency. Eur J Pediatr 128, 261–272 (1978). https://doi.org/10.1007/BF00445611

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  • DOI: https://doi.org/10.1007/BF00445611

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