Homocitrullinuria and homoargininuria in hyperargininaemia
Journal of Inherited Metabolic Disease1987Vol. 11(3), pp. 261–265
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Abstract
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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