IV 20 mg/kg once every 2 wk. Infantile-onset Pompe disease (IOPD) May be increased to 40 mg/kg every other wk or decreased to 20 mg/kg every other wk. Patient 1.25-5 kg Max infusion rate: 9.6 mg/kg/hr. Late-onset Pompe diseasePatient 1.25-5 kg Max infusion rate: 4.8 mg/kg/hr.
Discontinue use if severe hypersensitivity, anaphylaxis or infusion-associated reactions occur. Advanced Pompe disease; patients susceptible to fluid vol overload or w/ acute underlying resp illness or compromised cardiac &/or resp function. Immunogenicity. Concomitant use of general anaesth for central venous catheter or other surgical procedures in IOPD patients w/ cardiac hypertrophy. May affect ability to drive & use machines. Hepatic & moderate or severe renal impairment. Pregnancy & lactation. Infants <6 mth.
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