Galsulfase
Galsulfase is a recombinant human enzyme replacement therapy used to treat Mucopolysaccharidosis VI (MPS VI), also known as Maroteaux-Lamy syndrome. It works by replacing the deficient N-acetylgalactosamine 4-sulfatase enzyme, helping to break down glycosaminoglycans that accumulate in cells throughout the body due to this lysosomal storage disorder.
Galsulfase is an enzyme replacement therapy for MPS VI (Maroteaux-Lamy syndrome) that replaces the deficient N-acetylgalactosamine 4-sulfatase enzyme.
This medication carries a boxed (black box) warning — the strongest type of FDA warning. Review the full prescribing information before use.
Key facts at a glance
- ATC
- A16AB08
- RxNorm
- 578033
- Generic
- Galsulfase
- Class
- atc-a16
- Brands
- Naglazyme
- Availability
- rx
- Lactation
- unknown
- Routes
- —
- Forms
- injection
- Regions
- US, EU, CN, JP
- Monitoring
- —
This page is for informational purposes only.
Do not use this information to diagnose yourself or to replace a physician's advice. Always consult a qualified healthcare provider before starting, stopping, or changing any medication. If you believe you are experiencing a medical emergency, call your local emergency number immediately.
Dosage and administration
- Usual dose
- 1 mg/kg once weekly
- Maximum
- consult physician
- Frequency
- once weekly
- Instructions
- Follow physician instructions
- Duration limits
- as prescribed
- Monitoring
- standard
- Usual dose
- 1 mg/kg once weekly
- Maximum
- consult physician
- Frequency
- once weekly
- Instructions
- Follow physician instructions
- Duration limits
- as prescribed
- Monitoring
- standard
- Usual dose
- 1 mg/kg once weekly
- Maximum
- consult physician
- Frequency
- once weekly
- Instructions
- Follow physician instructions
- Duration limits
- as prescribed
- Monitoring
- standard
Overdose information
Special Populations
9 groupspregnancy
first trimesteravoid if possible
avoid use during pregnancy unless benefits outweigh risks
regular fetal monitoring
Use of this medication in this population requires careful consideration of risks and benefits.
pregnancy
second trimesteravoid if possible
avoid use during pregnancy unless benefits outweigh risks
regular fetal monitoring and ultrasound
Use of this medication in this population requires careful consideration of risks and benefits.
pregnancy
third trimesteravoid if possible
avoid use during pregnancy unless benefits outweigh risks
regular fetal monitoring and assessment of fetal growth
Use of this medication in this population requires careful consideration of risks and benefits.
lactation
breastfeedinguse with caution
discontinue breastfeeding or avoid drug; decision should be made considering benefit-risk
monitor infant for adverse effects
pediatric_age
infantsno adjustment needed
approved for use in pediatric patients; use under close medical supervision
monitor for infusion reactions and antibodies
Use with caution and monitor for adverse effects.
pediatric_age
childrenno adjustment needed
approved for use in pediatric patients age 2 years and older
monitor for infusion reactions, antibodies, and clinical response
geriatric
elderlystart at lower end of dosing range
limited clinical experience in patients over 65 years; use with caution
monitor renal and hepatic function; watch for infusion reactions
Use with caution and monitor for adverse effects.
renal_impairment
mild to moderateno specific adjustment recommended
use with caution; no formal studies conducted
monitor renal function during treatment
Use with caution and monitor for adverse effects.
hepatic_impairment
mild to moderateno specific adjustment recommended
use with caution; no formal studies conducted
monitor hepatic function during treatment
Use with caution and monitor for adverse effects.
Statistics and clinical data
Drug class
Galsulfase is a recombinant human enzyme used as replacement therapy
Indication
FDA-approved indication for treatment of MPS VI
FDA approval year
FDA approval date for Galsulfase (Naglazyme)
Common adverse reactions
Most frequently reported adverse reactions in clinical trials
Treatment of Mucopolysaccharidosis VI (MPS VI), a rare genetic lysosomal storage disorder characterized by deficiency of the enzyme N-acetylgalactosamine 4-sulfatase, leading to accumulation of glycosaminoglycans in cells
Galsulfase is a recombinant form of human N-acetylgalactosamine 4-sulfatase that provides exogenous enzyme for catabolism of glycosaminoglycans (GAGs), specifically dermatan sulfate. It is administered via intravenous infusion and helps reduce GAG accumulation in tissues.
Administered as a weekly IV infusion. Hypersensitivity reactions including anaphylaxis may occur; pre-medication with antihistamines may be recommended. Requires regular monitoring. Clinical trials showed improvement in walking capacity and stair climbing. Store refrigerated at 2-8°C.
Contraindications
Conditions and patient states where this medication should be avoided or used with caution.
Do not use (1)
Known allergy to this medication or its components
Use caution (1)
Consult healthcare provider for benefits vs risks assessment
Official drug label
WellAlly may earn a referral fee from linked resources at no extra cost to you. Editorial content is never influenced by commercial relationships.
This information is for educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment.
This content is for general informational and educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment. Always seek the guidance of a qualified healthcare provider with any questions you have about a medical condition, and never disregard professional medical advice or delay seeking it because of something you read here.
If you think you may have a medical emergency, call your local emergency number or go to the nearest emergency department immediately.
Medically reviewed by WellAlly Editorial Team Last reviewed July 26, 2026. · Read our editorial policy