Vestronidase alfa-vjbk

What is Vestronidase alfa?[edit | edit source]

Vestronidase alfa (MEPSEVII) is a recombinant human lysosomal beta glucuronidase used for the treatment of Mucopolysaccharidosis VII (MPS VII, Sly syndrome).

This medicine is used in pediatric and adult patients for the treatment of Mucopolysaccharidosis VII (MPS VII, Sly syndrome).

Mucopolysaccharidosis VII (MPS VII or Sly syndrome) is a lysosomal disorder characterized by the deficiency of GUS that results in GAG accumulation in cells throughout the body leading to multisystem tissue and organ damage.
Vestronidase alfa-vjbk is a recombinant form of human GUS and is intended to provide exogenous GUS enzyme for uptake into cellular lysosomes. Mannose-6-phosphate (M6P) residues on the oligosaccharide chains allow binding of the enzyme to cell surface receptors, leading to cellular uptake of the enzyme, targeting to lysosomes and subsequent catabolism of accumulated GAGs in affected tissues.

Limitations of Useː

The effect of MEPSEVII on the central nervous system manifestations of MPS VII has not been determined.

Premedication with a non-sedating antihistamine with or without an anti-pyretic is recommended 30 to 60 minutes prior to the start of the infusion.

Recommended Dosageː

The recommended dosage of MEPSEVII is 4 mg/kg administered by intravenous infusion every two weeks.

Administration

MEPSEVII should be administered under the supervision of a healthcare professional with the capability to manage anaphylaxis.
Prepare MEPSEVII according to the following steps using aseptic technique.
Administer the infusion over approximately 4 hours. In the first hour of infusion, infuse 2.5% of the total volume. After the first hour, the rate can be increased to infuse the remainder of the volume over 3 hours as tolerated.
Use an infusion set equipped with an in-line, low-protein binding 0.2 micron filter to administer the diluted MEPSEVII solution.
Do not flush the line containing MEPSEVII to avoid a rapid bolus of infused enzyme. Due to the low infusion rate, additional saline may be added through a separate line (piggyback or Y tube) to maintain sufficient intravenous flow to prevent clotting or line blockage.
Do not infuse with other products in the infusion tubing. Compatibility with other products has not been evaluated.
Use MEPSEVII immediately after dilution and complete the infusion within 42 hours from the time of dilution. Discard any unused product.
If immediate use is not possible, the diluted solution may be stored up to 36 hours under refrigeration at 2°C to 8°C (36°F to 46°F) followed by up to 6 hours at room temperature up to a maximum of 25°C (77°F).

This medicine is available in fallowing doasage form:

This medicine is available in fallowing brand namesː

The most common side effects of this medicine include:

Anaphylaxis to MEPSEVII was reported.
Anaphylaxis can be life-threatening.
MEPSEVII should be administered under the supervision of a healthcare professional with the capability to manage anaphylaxis.
Patients should be observed for 60 minutes after MEPSEVII administration.
If severe systemic reactions occur, including anaphylaxis, immediately discontinue the MEPSEVII infusion and provide appropriate medical treatment.

In case of overdose, call the poison control helpline of your country. In the United States, call 1-800-222-1222.

Overdose related information is also available online at poisonhelp.org/help.
In the event that the victim has collapsed, had a seizure, has trouble breathing, or can't be awakened, immediately call emergency services. In the United States, call 911.

There are no available data on MEPSEVII use in pregnant women to determine a drug-associated risk of adverse developmental outcomes.

The safety and effectiveness of MEPSEVII have been established in pediatric patients less than 18 years of age.

VESTRONIDASE ALFA

SODIUM PHOSPHATE, MONOBASIC, DIHYDRATE SODIUM CHLORIDE HISTIDINE POLYSORBATE 20

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