Agalsidase Beta Brand Name– Fabrazyme
What is Agalsidase Beta
Agalsidase beta (Fabrazyme®) is a recombinant human a-galactosidase A enzyme that is given by intravenous infusion.
Patients with Fabry disease have a deficiency of the lysosomal enzyme, ceramidetrihexosidase or a-galactosidase A, which breaks down glycosphingolipids (predominantly globotriaosylceramide or GL-3). Glycosphingolipids accumulate in the lining of blood vessels in the heart, kidney, and other organs in patients without an adequate presence of a-galactosidase A.
Crises of severe pain in the extremities (acroparesthesias), hypohidrosis, corneal opacities and dysfunction of several organs (kidney, brain, heart) are the primary manifestations, and patients often have decreased life expectancy and experience renal failure, cardiomyopathy and cerebrovascular accidents.
Replacement of the deficient enzyme may reduce levels of the accumulated glycolipids in various tissues and attenuate disease symptoms.
Indications
- Fabry disease
Side Effects
- abdominal pain
- anaphylactoid reactions
- angioedema
- antibody formation
- anxiety
- arrhythmia exacerbation
- arthralgia
- asthenia
- ataxia
- back pain
- bradycardia
- bronchospasm
- cardiac arrest
- chest pain (unspecified)
- chills
- cough
- depression
- diarrhea
- dizziness
- dysphagia
- dyspnea
- edema
- erythema
- fatigue
- fever
- flushing
- headache
- hearing loss
- heart failure
- hyperhidrosis
- hypertension
- hypoesthesia
- hypotension
- hypoxia
- infection
- infusion-related reactions
- lacrimation
- lymphadenopathy
- muscle cramps
- myalgia
- myocardial infarction
- nasal congestion
- nausea
- nephrotic syndrome
- pallor
- palpitations
- paresthesias
- pharyngitis
- pruritus
- rash
- renal failure (unspecified)
- rhinorrhea
- sinus tachycardia
- sinusitis
- stroke
- tinnitus
- urticaria
- vasculitis
- vertigo
- vomiting
- wheezing
- xerostomia
Monitoring Parameters
- CBC with differential
- ECG
- echocardiogram
- serum creatinine
- urinalysis
Contraindications
- breast-feeding
- cardiac disease
- children
- geriatric
- infants
- mannitol hypersensitivity
- neonates
- pregnancy
Interactions
There are no drug interactions associated with Agalsidase Beta products.
