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Arginine
Chronic management of hyperammonemia secondary to urea cycle disorders (e.g. Argininosuccinic acid synthetase deficiency and Arginino succinic acid lyase deficiency and N-Acetylglutamate synthetase deficiency).
- For argininosuccinic acid synthetase deficiency (ASS) and argininosuccinic acid lyase deficiency (ASL): 400-700 mg/kg/day div. Q8 hr or 8.8 –15.4 g/m2/day div. Q8 hr.
- For N-Acetylglutamate synthetase deficiency (NAGS deficiency): 170 mg /kg/day div. Q8 hr.(22).
600 mg and 700 mg capsules
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22. Kranen S, Keough D, Gordon RB, Emmerson BT. Xanthine-containing calculi during allopurinol therapy. J Urol 1985;133(4):658-9