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Allopurinol

Disorders causing hyperuricemia
As a challenge drug to induce orotic aciduria

Children: 2-20 mg/kg/day.  Adults: 100-600 mg/day. The dosage range is wide. Generally the recommended dose is what is required to keep the serum uric acid level < 6 mg/dl and avoid xanthine calculi (the side effect of allopurinol)

The dose should be reduced in renal failure.(8-17)
100 and 300 mg tablets
20 mg/mL suspension (extemporaneous preparation)

PO

Dermatologic: Rash (less than 1%), Stevens-Johnson syndrome (less than 1%), Toxic epidermal necrolysis (less than 1%)

Hematologic: Agranulocytosis, Aplastic anemia, Eosinophilia, Myelosuppression, Thrombocytopenia (0.6%)

Hepatic: Granulomatous hepatitis (less than 1% ), Hepatic necrosis (less than 1% ), Hepatotoxicity Immunologic: Immune hypersensitivity reaction.

Renal: Renal failure (less than 1% )

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8. Centre for Evidence-Based Medicine. Secondary Centre for Evidence-Based Medicine  2009. http://www.cebm.net/index.aspx?o=1025.

9. Loche S, Carta D, Muntoni AC, Corda R, Pintor C. Oral administration of arginine enhances the growth hormone response to growth hormone releasing hormone in short children. Acta Paediatr 1993;82(10):883-4

10. Sackett DL, Rosenberg WM, Gray JA, Haynes RB, Richardson WS. Evidence based medicine: what it is and what it isn't. BMJ 1996;312(7023):71-2

11. Alfadhel M, Sirrs S. Enzyme replacement therapy for Fabry disease: some answers but more questions. Ther Clin Risk Manag 2011;7:69-82 doi: 10.2147/TCRM.S11987[published Online First: Epub Date]|.

12. Scheinberg A, Hall K, Lam LT, O'Flaherty S. Oral baclofen in children with cerebral palsy: a double-blind cross-over pilot study. Journal of paediatrics and child health 2006;42(11):715-20 doi: 10.1111/j.1440-1754.2006.00957.x[published Online First: Epub Date]|.

13. Awaad Y, Shamato H, Chugani H. Hemidystonia improved by baclofen and PET scan findings in a patient with glutaric aciduria type I. J Child Neurol 1996;11(2):167-9

14. Schiffmann R, Kopp JB, Austin HA, 3rd, et al. Enzyme replacement therapy in Fabry disease: a randomized controlled trial. JAMA 2001;285(21):2743-9

15. Banikazemi M, Bultas J, Waldek S, et al. Agalsidase-beta therapy for advanced Fabry disease: a randomized trial. Annals of internal medicine 2007;146(2):77-86

16. Strauss KA, Puffenberger EG, Morton DH. Maple Syrup Urine Disease. 1993 doi: NBK1319 [bookaccession][published Online First: Epub Date]|.

17. Chien YH, Hwu WL. A review of treatment of Pompe disease in infants. Biologics : targets & therapy 2007;1(3):195-201

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