Naglazyme (galsulfase)
Metabolic Disease Enzyme Replacement - Mucopolysaccharidosis Intravenous Vial (ML)
Last reviewed · Source: First Databank (FDB) drug data
Overview
Galsulfase is a biosynthetic (recombinant DNA origin) form of N-acetylgalactosamine 4-sulfatase (arylsulfatase B, ASB), a lysosomal enzyme that catalyzes the hydrolysis of the sulfate ester from terminal N-acetylgalactosamine 4-sulfate residues of glycosaminoglycans (GAG) chondroitin 4-sulfate and dermatan sulfate.
Galsulfase is a biosynthetic (recombinant DNA origin) form of N-acetylgalactosamine 4-sulfatase, a lysosomal enzyme that catalyzes the hydrolysis of the sulfate ester from terminal N-acetylgalactosamine 4-sulfate residues of glycosaminoglycan (GAG) chondroitin 4-sulfate and dermatan sulfate. In patients with MPS VI, galsulfase provides an exogenous source of N-acetylgalactosamine 4-sulfatase for uptake into lysosomes and catabolism of glycosaminoglycans.
USES: Galsulfase is used to treat a certain rare genetic problem (Maroteaux-Lamy syndrome - MLS). Galsulfase replaces a certain natural substance that is missing in people with MLS. This medication improves how far you can walk and the number of stairs you can climb up.
Galsulfase does not correct the genetic problem, and treatment must be continued for life.
Generic name: galsulfase
Pronunciation: (gal-SUL-fase)
Drug class: Metabolic Disease Enzyme Replacement - Mucopolysaccharidosis
Therapeutic class: Metabolic Disease Enzyme Replacement Agents
Reference product & biosimilars
No reference, biosimilar or interchangeable associated with this drug.
Today’s intelligence on Naglazyme
Indications
The following indications are listed for Naglazyme:
- Maroteaux-Lamy Syndrome
Also known as: Mucopolysaccharidosis Type Vi.
Dosing
No dosing information is available for this tab.
Galsulfase is administered by IV infusion.
Galsulfase injection concentrate must be diluted prior to administration. (See Dilution under Dosage and Administration: Administration.) Solutions of galsulfase should be prepared using PVC containers and administered with a PVC infusion set equipped with an inline, low-protein-binding filter with a pore diameter of 0.2 mcm.
| Drug label | Dosing type | Instructions |
|---|---|---|
| Naglazyme 5 Mg/5 Ml Vial | Maintenance | Infuse 1 mg/kg over 4 hour(s) by intravenous route once weekly. |
No dosing information is available for this tab.
Drug interactions
No formal drug interaction studies have been performed.
There are no contraindication interactions for this drug.
There are no severe interactions for this drug.
There are no moderate interactions for this drug.
Contraindications
No known contraindications.
There are no contraindication contraindications for this drug.
There are no severe contraindications for this drug.
- Chronic heart failure
- Pulmonary disease
Adverse reactions
The most common adverse effects reported during clinical studies of galsulfase include headache, fever, arthralgia, vomiting, upper respiratory tract infections, abdominal pain, diarrhea, ear pain, cough, and otitis media. Other adverse effects reported in more than 10% of patients receiving galsulfase in the 6-month placebo-controlled study include pain, conjunctivitis, dyspnea, rigors, chest pain, pharyngitis, areflexia, increased corneal opacification, facial edema, gastroenteritis, hypertension, malaise, nasal congestion, and umbilical hernia.
Less Frequent
- Chest pain
- Conjunctivitis
- Gastroenteritis
- Hypersensitivity drug reaction
- Hypertension
Rare or Very Rare
- Abdominal herniation
- Acute respiratory failure
- Anaphylaxis
- Apnea
- Bradycardia
- Bronchospastic pulmonary disease
- Corneal opacity
- Cyanosis
- Facial edema
- Glomerulonephritis
- Hyporeflexia
- Hypotension
- Hypoxia
- Kidney disease with reduction in glomerular filtration rate (GFr)
- Severe dyspnea
- Shock
- Thrombocytopenic disorder
- Upper respiratory infection
More Frequent
- Acute abdominal pain
- Chills
- Dyspnea
- Earache
- Fever
- Headache disorder
- Nausea
- Pain
- Pruritus of skin
- Skin rash
- Urticaria
- Vomiting
Less Frequent
- Acute bacterial otitis media
- Arthralgia
- Cough
- Diarrhea
- Hearing loss
- Nasal congestion
- Pharyngitis
Rare or Very Rare
- Erythema
- Malaise
- Pallor
- Paresthesia
- Tachycardia
- Tachypnea
Precautions
No enhanced messaging available.
Safety and efficacy not established in children younger than 5 years of age.
Category B. (See Users Guide.) (See Clinical Surveillance Program under Uses: Mucopolysaccharidosis VI.)
Not known whether galsulfase is distributed into milk. Use with caution in nursing women. (See Clinical Surveillance Program under Uses: Mucopolysaccharidosis VI.)
Clinical studies of galsulfase did not include patients older than 29 years of age. It is not known whether geriatric patients (65 years of age and older) respond differently than younger patients.
ICD codes
| Code | Type | Description |
|---|---|---|
| Maroteaux-lamy syndrome | ||
| E76.29 | ICD10CM | Other mucopolysaccharidoses |
ICD code information from First Databank (FDB).
Formulary status
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Last reviewed · Source: First Databank (FDB) drug data
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