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The dosage is wide 15-50 mg/kg/day in 2 or 3 divided doses, maximum 1000 mg/day. However, the dose depends on monitoring free urine cystine level and modify the dose in order to maintain a level below 200 mmol/mmol of creatinine (8, 9, 120, 121).

Tablet, 100 mg

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8. Centre for Evidence-Based Medicine. Secondary Centre for Evidence-Based Medicine  2009. http://www.cebm.net/index.aspx?o=1025.

9. Loche S, Carta D, Muntoni AC, Corda R, Pintor C. Oral administration of arginine enhances the growth hormone response to growth hormone releasing hormone in short children. Acta Paediatr 1993;82(10):883-4

120. Tabatabaie L, Klomp LW, Berger R, de Koning TJ. L-serine synthesis in the central nervous system: a review on serine deficiency disorders. Mol Genet Metab 2010;99(3):256-62 doi: 10.1016/j.ymgme.2009.10.012[published Online First: Epub Date]|.

121. Matsuo S, Inoue F, Takeuchi Y, Yoshioka H, Kinugasa A, Sawada T. Efficacy of tryptophan for the treatment of nonketotic hyperglycinemia: a new therapeutic approach for modulating the N-methyl-D-aspartate receptor. Pediatrics 1995;95(1):142-6