Summary
A four-year-old boy with hyperargininaemia had increased urinary excretion of homocitrulline and homoarginine. A single oral lysine load created a marked increase in these amino acids in plasma. A daily oral lysine supplementation resulted in a remarkable urinary leakage of homocitrulline and homoarginine. These findings suggest that the patient had an enhanced synthesis of these amino acids.
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Kato, T., Sano, M., Mizutani, N. et al. Homocitrullinuria and homoargininuria in hyperargininaemia. J Inherit Metab Dis 11, 261–265 (1988). https://doi.org/10.1007/BF01800367
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DOI: https://doi.org/10.1007/BF01800367