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Sodium phenylacetate

Hyperammonaemia in urea cycle disorders and in undiagnosed patient with  hyperammonaemia, no evidence support its use in acute crises of propionic or methylmalonic acidemia.

Acute crises: 0.25 g/kg (<20 kg) or 5.5 g/m2 (>20kg) as a loading dose followed by same dose as maintenance dose over 24 hours (22, 104).

IV or PO

Endocrine metabolic: Hyperammonemia (5%), Hypokalemia (7%), Metabolic acidosis (4%).

Hematologic: Anemia (4%), Disseminated intravascular coagulation (3%).

Neurologic: Cerebral edema (5%), Coma (3%), Neurotoxicity, Seizure (6%).

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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

104. Wraith JE, Clarke LA, Beck M, et al. Enzyme replacement therapy for mucopolysaccharidosis I: a randomized, double-blinded, placebo-controlled, multinational study of recombinant human alpha-L-iduronidase (laronidase). J Pediatr 2004;144(5):581-8 doi: 10.1016/j.jpeds.2004.01.046[published Online First: Epub Date]|.

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