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0.5 mg/kg weekly over 1-3 hour(s) (88-90).

IV solution must be diluted in 100 ml of 0.9 sodium chloride injection, each vial contains 2mg/ml solution of idursulfase protein (6mg) in an extractable volume of 3 ml and for single use only.

IV

Hypertention, pruritic rash, headache, fever, urticaria and infusion reaction.

1b

88. Chalmers RA, de Sousa C, Tracey BM, Stacey TE, Weaver C, Bradley D. L-carnitine and glycine therapy in isovaleric acidaemia. J Inherit Metab Dis 1985;8 Suppl 2:141-2

89. Herrick AL, McColl KE, Moore MR, Cook A, Goldberg A. Controlled trial of haem arginate in acute hepatic porphyria. Lancet 1989;1(8650):1295-7 doi: S0140-6736(89)92688-3 [pii][published Online First: Epub Date]|.

90. Andersson HC, Shapira E. Biochemical and clinical response to hydroxocobalamin versus cyanocobalamin treatment in patients with methylmalonic acidemia and homocystinuria (cblC). J Pediatr 1998;132(1):121-4 doi: S0022-3476(98)70496-2 [pii][published Online First: Epub Date]|.