Mepsevii (vestronidase alfa-vjbk)
Metabolic Disease Enzyme Replacement - Mucopolysaccharidosis Intravenous Vial (ML)
Last reviewed · Source: First Databank (FDB) drug data
Overview
Vestronidase alfa-vjbk is a biosynthetic (recombinant DNA origin) form of human beta-glucuronidase, a lysosomal enzyme that catabolizes glycosaminoglycans.
Vestronidase alfa-vjbk is a recombinant form of human beta-glucuronidase, a lysosomal enzyme that catabolizes the glycosaminoglycans dermatan sulfate, chondroitin sulfate, and heparan sulfate. The drug is produced using a Chinese hamster ovary cell line.
Mucopolysaccharidosis VII (MPS VII, Sly syndrome) is a lysosomal storage disorder characterized by a deficiency of beta-glucuronidase. (See Uses: Mucopolysaccharidosis VII.) In patients with this condition, vestronidase alfa-vjbk provides an exogenous source of beta-glucuronidase for catabolism of glycosaminoglycans (dermatan sulfate, chondroitin sulfate, and heparan sulfate) that accumulate and cause manifestations of the disorder. Cellular uptake of vestronidase alfa-vjbk into lysosomes is mediated by the binding of mannose-6-phosphate-terminated oligosaccharide chains of the drug to mannose-6-phosphate receptors. In clinical studies of MPS VII patients, treatment with vestronidase alfa-vjbk reduced urinary excretion of chondroitin sulfate and dermatan sulfate.
Serum concentrations of vestronidase alfa-vjbk appear to increase in an approximately dose-proportional manner at doses ranging from 1 to 4 mg/kg. Mean elimination half-life of vestronidase alfa-vjbk is 155 minutes (range: 51-213 minutes). The drug is metabolized by proteolytic degradation into small peptides and amino acids and is not expected to be eliminated through renal or fecal excretion.
Generic name: vestronidase alfa-vjbk
Drug class: Metabolic Disease Enzyme Replacement - Mucopolysaccharidosis
Therapeutic class: Metabolic Disease Enzyme Replacement Agents
Reference product & biosimilars
No reference, biosimilar or interchangeable associated with this drug.
Today’s intelligence on Mepsevii
Indications
The following indications are listed for Mepsevii:
- Mucopolysaccharidosis Type Vii
Also known as: Beta-Glucuronidase Deficiency, Gusb Deficiency, Sly Syndrome.
Dosing
The recommended dosage of vestronidase alfa-vjbk for the treatment of mucopolysaccharidosis VII (MPS VII) in adults and pediatric patients is 4 mg/kg administered by IV infusion once every 2 weeks.
Vestronidase alfa-vjbk is administered by IV infusion.
Vestronidase alfa-vjbk should be administered using an inline, low-protein-binding filter with a pore diameter of 0.2 mcm. The IV line containing vestronidase alfa-vjbk should not be flushed to avoid a rapid bolus of the drug. Due to the slow rate of infusion, additional 0.9% sodium chloride injection may be infused through a separate line to maintain sufficient IV flow and prevent clotting or blockage of the IV tubing.
Compatibility with other drugs has not been evaluated; therefore, vestronidase alfa-vjbk must not be infused through the same IV line with other drugs.
Vestronidase alfa-vjbk injection for IV infusion should be stored at 2-8degreesC and protected from light; the vials should not be frozen or shaken. Vials of vestronidase alfa-vjbk contain no preservatives and are intended for single use only.
| Drug label | Dosing type | Instructions |
|---|---|---|
| Mepsevii 10 Mg/5 Ml Vial | Maintenance | Infuse 4 mg/kg over 4 hour(s) by intravenous route every 2 weeks. |
No dosing information is available for this tab.
Drug interactions
Formal drug interaction studies with vestronidase alfa-vjbk have not been performed to date.
There are no contraindication interactions for this drug.
There are no severe interactions for this drug.
There are no moderate interactions for this drug.
Contraindications
The manufacturer states that there are no known contraindications to the use of vestronidase alfa-vjbk.
There are no contraindication contraindications for this drug.
There are no severe contraindications for this drug.
- No disease contraindications
Adverse reactions
Adverse effects reported in patients receiving vestronidase alfa-vjbk include extravasation, diarrhea, rash, anaphylaxis, infusion site swelling, peripheral swelling, and pruritus.
More Frequent
- Anaphylaxis
- Extravasation injury
More Frequent
- Diarrhea
- Injection site sequelae
- Peripheral edema
- Pruritus of skin
- Skin rash
Precautions
No enhanced messaging available.
Most of the patients (approximately 70%) in the vestronidase alfa-vjbk clinical program were younger than 18 years of age. Pediatric patients as young as 5 months of age have been treated with the drug in clinical studies.
Serum concentrations of the drug in pediatric patients younger than 5 years of age appear to be similar to the concentrations observed in older children and adults.
There are no available data on vestronidase alfa-vjbk use in pregnant women. In animal studies, administration of vestronidase alfa-vjbk to pregnant rats and rabbits during the period of organogenesis resulted in no maternal toxicity or adverse developmental outcomes at maternal exposures of up to 1.6 and 10 times, respectively, the exposure at the recommended human dose.
It is not known whether vestronidase alfa-vjbk is distributed into human or animal milk. The effects of the drug on the breast-fed infant or on milk production also are not known.
The developmental and health benefits of breast-feeding should be considered along with the woman's clinical need for vestronidase alfa-vjbk and any potential adverse effects on the breast-fed infant from the drug or underlying maternal condition.
Clinical trials evaluating vestronidase alfa-vjbk did not include any patients 65 years of age or older. It is not known whether geriatric patients respond differently than younger patients.
ICD codes
| Code | Type | Description |
|---|---|---|
| Mucopolysaccharidosis type VII | ||
| E76.29 | ICD10CM | Other mucopolysaccharidoses |
ICD code information from First Databank (FDB).
Formulary status
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Last reviewed · Source: First Databank (FDB) drug data
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