Avalglucosidase alfa

Generic name
Avalglucosidase alfa
Brand name
ATC Code
A16AB22

Avalglucosidase alfa

Dosages
Side effects in children
Warnings & precautions in children
Contra-indications in children

Interactions
PK
Renal impairment
References

Pharmacokinetics in children

  Infantile onset Pompe disease Late onset Pompe disease
Dose 20 mg/kg 40 mg/kg 20 mg/kg
Cmax (microg/ml) 175-189 205-403 273
Vd (l) 3,5 - 5,4 3,4
Klaring (l/hour) 0,53-0,70 0,87
T1/2 (hour) 0,6-1,19 1,55

Source SmPC

dose recommendation of formulary compared to licensed use (on-label versus off-label)

No information is present at this moment.

Available formulations

No information is present at this moment.

Dosages

Enzyme replacement therapy in Pompe’s disease
  • Intravenous
    • ≥ 6 months
      • 20 mg/kg/dose every 2 weeks. If necessary, in infantile onset of disease, increase to 40 mg/kg/dose based on effect and tolerance.
      • Directions for administration:

        Start the infusion at an initial rate of 1 mg/kg/hour and gradually increase every 30 minutes to 3-5-7 mg/kg/hour if there are no signs of infusion-related reactions (IARs). Measure vital functions before each increase.

Renal impaiment in children > 3 months

No information available on dose adjustment in renal impairment.

The complete list of all undesirable drug reactions can be found in the national Summary of Product Characteristics (SmPC) – click here

Side effects in children

The side effect profile in children is similar to that in adults.

The complete list of all contra-indications can be found in the national Summary of Product Characteristics (SmPC) – click here

Contra-indications

No information available on specific contra indications in children.

The complete list of all warnings and precautions can be found in the national Summary of Product Characteristics (SmPC) – click here

Warnings & precautions

No information available on specific warnings and precautions in children.

Interactions

The complete list of all interactions can be found in the national Summary of Product Characteristics (SmPC) – click here

OTHER ALIMENTARY TRACT AND METABOLISM PRODUCTS

This pages provides a list of drugs from the same ATC class for comparison. This does not necessarily mean that these drugs are interchangeable.

Amino acids and derivatives
A16AA06
A16AA05
A16AA01
A16AA04
Enzymes
A16AB03
A16AB04
A16AB07
A16AB13
A16AB17
A16AB08
A16AB09
A16AB02
A16AB05
A16AB14
A16AB10
Various alimentary tract and metabolism products
A16AX10
A16AX09
A16AX
A16AX18
A16AX06
A16AX04
A16AX07
A16AX
A16AX08
A16AX07
A16AX05
AMINO ACIDS AND DERIVATIVES
A16AA06
A16AA05
A16AA01
A16AA04

Reference

  1. Sanofi B.V., SmPC Nexviadyme (EU/1/21/1579/001) Rev.5; 08-08-2024, www.ema.europa.eu

Changes

Therapeutic Drug Monitoring


Overdose