Inborn errors of metabolism:

Indications for: MEPSEVII

Treatment of Mucopolysaccharidosis VII (MPS VII, Sly syndrome).

Limitations of Use:

Effect on the CNS manifestations of MPS VII has not been determined.

Adults and Children:

Premedicate with a non-sedating antihistamine with or without an antipyretic 30–60mins prior to infusion. Give as IV infusion over 4hrs (infuse first 2.5% of total volume over 1hr, then increase rate as tolerated over next 3hrs); see full labeling. 4mg/kg every 2 weeks.

Boxed Warning:


MEPSEVII Warnings/Precautions:

Have appropriate medical support readily available. Should be administered under supervision of healthcare professional. Monitor during and for ≥60mins post-infusion for anaphylaxis; discontinue immediately if a severe systemic reaction occurs. Elderly. Pregnancy. Nursing mothers.

MEPSEVII Classification:

Recombinant human lysosomal beta glucuronidase.

Adverse Reactions:

Infusion site extravasation, diarrhea, rash, anaphylaxis, infusion site swelling, peripheral swelling, pruritus.

Generic Drug Availability:


How Supplied:

Single-dose vial (5mL)—1