Inborn errors of metabolism:
Indications for: KANUMA
Lysosomal acid lipase (LAL) deficiency.
Adults and Children:
<1 month: not established. Give as IV infusion over ≥2hrs; consider further prolonging infusion time for doses >1mg/kg or if hypersensitivity reactions occur. May consider a 1hr infusion for the 1mg/kg dose in those who tolerate the infusion. ≥1 month: 1mg/kg once every other week; increase to 3mg/kg once every other week if suboptimal response. Infants with rapidly progressive deficiency within first 6 months of life: initially 1mg/kg once weekly; increase to 3mg/kg once weekly if suboptimal response; may further increase to 5mg/kg once weekly if continued suboptimal response. May pre-treat with antipyretics and/or antihistamines.
KANUMA Warnings/Precautions:
Monitor for hypersensitivity reactions during and after infusion; interrupt or lower infusion rate based on severity. Discontinue immediately and treat if severe hypersensitivity including anaphylaxis occurs. Eggs or egg products allergy. Pregnancy. Nursing mothers.
KANUMA Classification:
Hydrolytic lysosomal cholesteryl ester and triacylglycerol-specific enzyme.
Adverse Reactions:
Diarrhea, vomiting, fever, rhinitis, anemia, cough, nasopharyngitis, urticaria, headache, oropharyngeal pain, asthenia, constipation, nausea.
Generic Drug Availability:
NO
How Supplied:
Single-use vial—1
