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Sodium phenylbutyrate

Chronic management of urea cycle disorders.

0.45-0.6 g/kg/day if <20 kg; 9.9-13 g/m2/day in larger patients (22, 104, 105).

500 mg tablet

Available also as powder

PO

Endocrine metabolic: Acidosis (14%), Hypoalbuminemia (11%).

Hematologic: Anemia (9%).

Reproductive: Amenorrhea (23%), Irregular periods (23%).

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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]|.

105. Chapman KA, Gropman A, Macleod E, et al. Acute management of propionic acidemia. Mol Genet Metab 2012;105(1):16-25 doi: 10.1016/j.ymgme.2011.09.026[published Online First: Epub Date]

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