All the biological system drugs
Enzymes. Taliglucerase Alfa 200 U/vial. VIAL: 1× 200 U. The recomm. dosage for
tmt.-naïve adult &ped. pts. above 2 yrs.
of age and older is 60U/ kg of bdy. wt.
admin. every other wk. as a 60 to 120
min. IV. See lit.
Indic. for long-term enzyme replac. ther.
(ERT) for adult. & ped. pts. above 2 yrs.
with a confirm. diagnos. of Type 1
Enzyme Replacement Therapy. Alglucoside Alfa 50 mg. VIAL (pwdr. for conc. infus. sol.): 1.
20 mg/kg bdy wt I.V. infus. every 2 wks.
Initial rate: 1 mg/ kg/hr. Grad. incr. by 2
mg/kg/ hr. every 30 mins. if no signs
IARs, incr. until max. rate: 7 mg /kg/hr.
Tmt. by physician experienced in manage.
Pompe dis. or other inherited
Long-term enzyme replace. ther. (ERT) in
comfirmed Pompe dis.(acid alphaglucosidase
The benefits in pts with late-onset Pompe
dis. have not been establ.
C/I: Life-threat. hypersens., when rechall.