← Search & browse all drugs
ALIMENTARY TRACT AND METABOLISM › OTHER ALIMENTARY TRACT AND METABOLISM PRODUCTS › OTHER ALIMENTARY TRACT AND METABOLISM PRODUCTS › Enzymes
asfotase alfa
A16AB13
Forms & Strengths
- Injection: 18 mg/0.45 mL solution in a single-dose vial
- Injection: 28 mg/0.7 mL solution in a single-dose vial
- Injection: 40 mg/mL solution in a single-dose vial
- Injection: 80 mg/0.8 mL solution in a single-dose vial
Adult Dosing
- Perinatal/infantile- and juvenile-onset hypophosphatasia: 2 mg/kg subcutaneously three times weekly
- Alternative regimen: 1 mg/kg subcutaneously six times weekly
Pediatric Dosing
- Perinatal/infantile- and juvenile-onset hypophosphatasia (all pediatric ages): 2 mg/kg subcutaneously three times weekly
- Alternative regimen: 1 mg/kg subcutaneously six times weekly
- Dose adjustment may be warranted based on weight changes in growing children
Indications
- Treatment of pediatric-onset hypophosphatasia (HPP)
- Management of manifestations including skeletal abnormalities, functional impairment, and respiratory complications
Mechanism of Action
- Recombinant human tissue-nonspecific alkaline phosphatase (TNSALP) Fc-enzyme fusion protein
- Substitutes for deficient TNSALP enzyme in hypophosphatasia
- Hydrolyzes substrates such as inorganic pyrophosphate (PPi), pyridoxal 5'-phosphate (PLP), and phosphoethanolamine (PEA)
- Reduces extracellular PPi accumulation, restoring normal bone mineralization and skeletal development
Contraindications
- History of life-threatening hypersensitivity to asfotase alfa or any of its excipients
Adverse Reactions
- Injection site reactions (erythema, pain, pruritus, discoloration, lipodystrophy)
- Hypersensitivity reactions including anaphylaxis
- Ectopic calcifications (conjunctival, corneal, and nephrocalcinosis)
- Lipodystrophy at injection sites
- Pyrexia, irritability, and headache
Drug Interactions
- No formal clinical drug-drug interaction studies have been conducted
- Potential theoretical interference with diagnostic assays measuring circulating pyridoxal phosphate levels
Curated Content: Needs Vetting Before Put to Clinical Use