Brand Names: Canada
Fabry disease: I.V.: 0.2 mg/kg every 2 weeks
Fabry disease: Refer to adult dosing.
Injection, solution [preservative free]:
Replagal™: 1 mg/1 mL (3.5 mL)
Agalsidase alfa may be confused with agalsidase beta, alglucerase, alglucosidase alfa
Cardiovascular: Flushing (24%)
Central nervous system: Fever (20%), headache (11%)
Neuromuscular & skeletal: Rigors (20%)
Miscellaneous: IgG antibody formation (55%), infusion-related reactions (13%)
1% to 10%:
Cardiovascular: Chest tightness (7%), hypertension (4%), tachycardia (4%), chest pain (2%), edema (2%), peripheral coldness (2%), peripheral edema (2%)
Central nervous system: Dizziness (9%), fatigue (9%), fatigue aggravated (7%), hypersomnia (2%), hypoesthesia (2%), panic attack (2%), pain/discomfort (7%), somnolence (2%), vertigo (2%)
Dermatologic: Acne (9%), erythema (7%), mottled skin(4%), pruritus (4%), dry skin (2%), eczema (2%), rash (2%)
Gastrointestinal: Nausea (9%), dysgeusia (6%), diarrhea (4%), vomiting (4%), abdominal pain (2%), dyspepsia (2%), gastrointestinal upset (2%), stomach cramps (2%), stomach discomfort (2%)
Neuromuscular & skeletal: Myalgia (6%), neuropathic pain (6%), tremor (4%), musculoskeletal discomfort (2%), back pain (2%), limb pain (2%), paraesthesia (2%), weakness (2%)
Ocular: Lacrimation increased (2%), periorbital edema (2%)
Respiratory: Hoarseness (6%), throat tightness (6%), cough (4%), dyspnea (4%), nasopharyngitis (4%), pharyngitis (4%), nasal congestion (2%), snoring (2%), throat irritation (2%)
Miscellaneous: Feeling hot (4%), influenza-like syndrome(2%), parosmia (2%)
<1% (Limited to important or life-threatening): Chills, facial flushing, urticaria
• Antibody formation: The presence IgG antibodies has been observed within 3 months from the onset of therapy in ~55% of treated patients. Approximately 60% of these patients are free of antibodies and >80% demonstrate immune tolerance, based on reduced titers of antibody within 12-18 months.
• Infusion reactions: Mild acute reactions (chills, facial flushing) are common and may occur during or within 1 hour after infusion. Severe reactions (nausea, pyrexia, rigors, tachycardia, urticaria, vomiting) are rare and usually occur within 2-4 months from the onset of therapy. Patients with a history of reactions may be premedicated with oral corticosteroids and antihistamines 1-3 hours prior to subsequent infusions.
• Fabry disease: Common symptoms observed in this patient population may be confused with adverse reactions related to treatment.
• Hepatic impairment: Safety and efficacy have not been established.
Chloroquine: May diminish the therapeutic effect of Agalsidase Alfa. Risk X: Avoid combination
Gentamicin: May diminish the therapeutic effect of Agalsidase Alfa. Risk X: Avoid combination
Gentamicin (Systemic): May diminish the therapeutic effect of Agalsidase Alfa. Risk X: Avoid combination
- Replagal (AU, BE, CH, CZ, DK, EE, ES, FI, IL, NO, NZ, SE, TW)
Metabolism: Plasma; via peptide hydrolysis
Half-life elimination: ~1.5-2 hours
Lidove O, Joly D, Barbey F, et al, “Clinical Results of Enzyme Replacement Therapy in Fabry Disease: A Comprehensive Review of Literature”, Int J Clin Pract, 2007, 61(2):293-302. [PubMed 17263716]
Ries M, Clarke JT, Whybra C, et al, “Enzyme-Replacement Therapy With Agalsidase Alfa in Children With Fabry Disease,” Pediatrics, 2006, 118(3):924-32. [PubMed 16950982]