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1mg/kg every 2 weeks as IV infusion over 2-4 hours.(1, 3)

5 mg and 35 mg single-use vials for reconstitution to yield (5mg/mL).

IV

Infusion reaction consisted of one or more of the following: chills, fever, feeling hot or cold, dyspnea, nausea, flushing, headache, vomiting, paresthesia, fatigue, pruritus, pain in extremity, hypertension, chest pain, throat tightness, abdominal pain, dizziness, tachycardia,

nasal congestion, diarrhea, edema peripheral, myalgia, back pain, pallor, bradycardia, urticaria, hypotension, face edema, rash, and somnolence.

1b

1. Scriver CR. The metabolic & molecular bases of inherited disease. 8th ed. New York ; London: McGraw-Hill, 2001.

3. Wilcken B. Rare diseases and the assessment of intervention: what sorts of clinical trials can we use? J Inherit Metab Dis 2001;24(2):291-8