法布瑞氏症 Fabry's disease
#仿單變更2022

Enzyme
Agalsidase alfa is a recombinant form of the enzyme alpha-galactosidase-A, which catalyzes the hydrolysis of globotriaosylceramide (Gb-3), cleaving a terminal galactose residue from the molecule. Accumulation of globotriaosylceramide (Gb-3) may occur within the tissues of patients with Fabry disease.

1. Distribution: Vd 7-15 L (steady-state); 17% of body weight
2. Metabolism: via peptide hydrolysis
3. Total body clearance: 2.3 mL/min/kg; (children) 4.2 mL/min/kg : (adolescents) 3.1 mL/min/kg
4. Elimination Half Life: 42 to 117 minutes

Hypersensitivity to agalsidase alfa or to any component of the product

Fetal risk cannot be ruled out. (TH)

The manufacturer recommends using with caution in breastfeeding women.

1. Immunologic: Antibody development
2. Miscellaneous: Infusion related reaction, fever
3. Cardiovascular: Chest pain, flushing, hypertension, livedo reticularis, oxygen saturation decreased, palpitations, peripheral edema, tachycardia
4. Central nervous system: Altered sense of smell, dizziness, fatigue, feeling hot, feeling of heaviness, headache, hypersomnia, hypoesthesia, local discomfort, malaise, neuralgia, pain, paresthesia, rigors, sensation of cold
5. Dermatologic: Acne vulgaris, erythema, hyperhidrosis, pruritus, rash at injection site, skin rash, urticaria
6. Gastrointestinal: Abdominal distress, abdominal pain, diarrhea, dysgeusia, nausea, nausea and vomiting, vomiting
7. Hypersensitivity: Anaphylaxis, angioedema, hypersensitivity reaction
8. Neuromuscular: Arthralgia, back pain, joint swelling, limb pain, myalgia, swelling of extremities, tremor, weakness
9. Ophthalmic: Increased lacrimation, reduced blinking
10. Otic: Tinnitus
11. Respiratory: Cough, dyspnea, flu-like symptoms, hoarseness, increased bronchial secretions, nasopharyngitis, pharyngeal edema, pharyngitis, rhinorrhea

0.2 mg/kg IV over 40 minutes every other week

1. Safety and efficacy in pediatric patients aged 0 to 6 years has not been established
2. ≥7 years: 0.2 mg/kg IV over 40 minutes every other week

No dosage adjustment necessary.

There are no dosage adjustments provided in the manufacturer's labeling; has not been studied

Storage:
1. Store vial under refrigeration between 2 and 8 degrees C.
2. Solution is stable up to 24 hours at 25 degrees C; discard unused solution
Administration:
1. Dilute total volume required in 100 mL NS and mix gently (do not shake)
2. Administer as soon as possible after dilution; product contains no preservative
3. Administer over 40 minutes via an IV line with an integral filter; do not infuse concomitantly with other agents