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Arginine hydrochloride (R-Gene®)
Acute management of hyperammonemic crises in suspected or confirmed urea cycle disorders, except arginase deficiency.
- For suspected urea cycle or ASS or ASL, give: 600mg/kg if (< 20kg) or 12 gram/m2 if (> 20kg) as a loading dose over 90 minutes followed by 600 mg/kg if (< 20kg) or 12 gram/m2 if (> 20kg) as maintenance infusion over 24 hours.
-For OTC and CPS: 200mg/kg if (< 20kg) or 4 gram/ m2 if (> 20kg) as a loading dose over 90 minutes followed by 200 mg/kg if (< 20kg) or 4 gram/ m2 if (> 20kg) as maintenance infusion over 24 hours (22).
10% solution for injection (100 mg/ml)
IV
Hyperchloremic metabolic acidosis, hypokelemia, diarrhea, elevated BUN, creatinine levels, flushing, nausea, vomiting, abdominal cramps, bloating, numbness, headache, phlebitis.
4
22. Kranen S, Keough D, Gordon RB, Emmerson BT. Xanthine-containing calculi during allopurinol therapy. J Urol 1985;133(4):658-9
