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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).

250 mg tablets

600 mg and 700 mg capsules

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Hyperchloremic metabolic acidosis, hypokelemia,  diarrhea, elevated BUN, creatinine levels, flushing,  nausea, vomiting, abdominal cramps, bloating numbness, headache.

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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

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