Pronunciation:
se-be-lye-pase al-fa
Trade Name(s)
Ther. Class.
none assigned
Pharm. Class.
enzymes
Treatment of lysosomal acid lipase (LAL) deficiency.
Being a recombinant form of LAL, it binds to cell surface receptors via glycans expressed on the protein and is subsequently internalized into lysosomes. It then catalyzes the lysosomal hydrolysis of cholesteryl esters and triglycerides to free cholesterol, glycerol, and free fatty acids.
Therapeutic Effect(s):
Improved survival in infants; improved lipids in adults and children.
Absorption: IV administration results in complete bioavailability.
Distribution: Well distributed.
Metabolism and Excretion: Unknown.
Half-life: 5.4–6.6 min.
TIME/ACTION PROFILE (blood levels)
ROUTE | ONSET | PEAK | DURATION |
---|---|---|---|
IV | Unknown | 1.3 hr | Unknown |
Contraindicated in:
Use Cautiously in:
CV: tachycardia
CNS: headache, anxiety
EENT: nasopharyngitis, rhinitis, sneezing
GI: diarrhea, oropharyngeal pain, vomiting, constipation, nausea
Hemat: anemia
Metabolic: ↑ LDL cholesterol, ↑ triglycerides
MS: hypotonia
Resp: cough, hypoxemia
Misc: HYPERSENSITIVITY REACTIONS (INCLUDING ANAPHYLAXIS, URTICARIA), fever
* CAPITALS indicate life-threatening.
Underline indicate most frequent.
Drug-Drug
None known.
Rapidly Progressive LAL Deficiency Presenting within First 6 Months of Age
IV Infants: 1 mg/kg once weekly; may ↑ to 3 mg/kg once weekly if suboptimal response achieved.
IV (Adults and Children): 1 mg/kg every other wk.
Solution for injection: 2 mg/mL
Lab Test Considerations:
May cause ↑ circulating LDL-cholesterol (LDL-C) and triglycerides.
Instruct parent or patient to notify health care professional immediately if signs and symptoms of hypersensitivity reaction (chest tightness; shortness of breath; wheezing; dizziness or faintness; or swelling of face, eyelids, or lips) or if cough or shortness of breath occurs.
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