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{{DrugProjectFormSinglePage
{{DrugProjectFormSinglePage
|authorTag={{RB}}
|genericName=laronidase
|aOrAn=a
|aOrAn=a
|drugClass=Enzyme
|indicationType=treatment
|indicationType=treatment
|indication=Hurler and Hurler-Scheie forms of Mucopolysaccharidosis I
|hasBlackBoxWarning=Yes
|hasBlackBoxWarning=Yes
|adverseReactions=<!--Black Box Warning-->
|adverseReactions=pyrexia, chills, blood pressure increased, tachycardia, and oxygen saturation decreased, rash, upper respiratory tract infection, injection site reaction, hyperreflexia, paresthesia, flushing,
|blackBoxWarningTitle=<span style="color:#FF0000;">ConditionName: </span>
|blackBoxWarningBody=<i><span style="color:#FF0000;">ConditionName: </span></i>


* Content
 
<!--Black Box Warning-->
|blackBoxWarningTitle=<span style="color:#FF0000;">WARNING: </span>
|blackBoxWarningBody=<i><span style="color:#FF0000;">RISK OF ANAPHYLAXIS: </span></i>
 
* Life-threatening anaphylactic reactions have been observed in some patients during ALDURAZYME® infusions.  Therefore, appropriate medical support should be readily available when ALDURAZYME is administered.  Patients with compromised respiratory function or acute respiratory disease may be at risk of serious acute exacerbation of their respiratory compromise due to infusion reactions, and require additional monitoring.


<!--Adult Indications and Dosage-->
<!--Adult Indications and Dosage-->


<!--FDA-Labeled Indications and Dosage (Adult)-->
<!--FDA-Labeled Indications and Dosage (Adult)-->
|fdaLIADAdult======Condition1=====
|fdaLIADAdult=====Indications====
 
ALDURAZYME® (laronidase) is indicated for patients with Hurler and Hurler-Scheie forms of Mucopolysaccharidosis I (MPS I) and for patients with the Scheie form who have moderate to severe symptoms.  The risks and benefits of treating mildly affected patients with the Scheie form have not been established.
* Dosing Information
 
:* Dosage
 
=====Condition2=====
 
* Dosing Information
 
:* Dosage
 
=====Condition3=====
 
* Dosing Information
 
:* Dosage


=====Condition4=====
ALDURAZYME has been shown to improve pulmonary function and walking capacity.  ALDURAZYME has not been evaluated for effects on the central nervous system manifestations of the disorder.


* Dosing Information
====Dosage====
Recommended Dose
The recommended dosage regimen of ALDURAZYME is 0.58 mg/kg of body weight administered once weekly as an intravenous (IV) infusion.  Pretreatment is recommended 60 minutes prior to the start of the infusion and may include antihistamines, antipyretics, or both [SEE WARNINGS AND PRECAUTIONS (5)].


:* Dosage
Each vial of ALDURAZYME provides 2.9 milligrams (mg) of laronidase in 5.0 milliliters (mL) of solution and is intended for single use only.  Do not use the vial more than one time.  The concentrated solution for infusion must be diluted with 0.9% Sodium Chloride Injection, USP, to a final volume of 100 mL or 250 mL, using aseptic techniques.  The final volume of the infusion is determined by the patient’s body weight.  Patients with a body weight of 20 kg or less should receive a total volume of 100 mL.  Patients with a body weight greater than 20 kg should receive a total volume of 250 mL [see DOSAGE AND ADMINISTRATION (2.2)].  For patients with underlying cardiac or respiratory compromise and weighing up to 30 kg, physicians may consider diluting ALDURAZYME in a volume of 100 mL and administering at a decreased infusion rate [see DOSAGE AND ADMINISTRATION (2.2), WARNINGS AND PRECAUTIONS (5.3) and ADVERSE REACTIONS (6.3)].


<!--Off-Label Use and Dosage (Adult)-->
2.2 Instructions for Use
Prepare and use ALDURAZYME according to the following steps. Use aseptic techniques. Prepare ALDURAZYME using low-protein-binding containers and administer with a low-protein-binding infusion set equipped with an in-line, low-protein-binding 0.2 micrometer (µm) filter. There is no information on the compatibility of diluted ALDURAZYME with glass containers.


<!--Guideline-Supported Use (Adult)-->
Determine the number of vials to be diluted based on the patient's weight and the recommended dose of 0.58 mg/kg, using the following equation:
|offLabelAdultGuideSupport======Condition1=====
                    Patient's weight (kg) x 1 mL/kg of ALDURAZYME = Total number mL of ALDURAZYME
                          Total number mL of ALDURAZYME ÷ 5 mL per Vial = Total number of Vials.


* Developed by:
Round up to the next whole vial.  Remove the required number of vials from the refrigerator to allow them to reach room temperature.  Do not heat or microwave vials.
Before withdrawing the ALDURAZYME from the vial, visually inspect each vial for particulate matter and discoloration.  The ALDURAZYME solution should be clear to slightly opalescent and colorless to pale yellow.  Some translucency may be present in the solution.  Do not use if the solution is discolored or if there is particulate matter in the solution.
Withdraw and discard a volume of the 0.9% Sodium Chloride Injection, USP from the infusion bag, equal to the volume of ALDURAZYME concentrate to be added.
Slowly withdraw the calculated volume of ALDURAZYME from the appropriate number of vials using caution to avoid excessive agitation. Do not use a filter needle, as this may cause agitation. Agitation may denature ALDURAZYME, rendering it biologically inactive.
Slowly add the ALDURAZYME solution to the 0.9% Sodium Chloride Injection, USP using care to avoid agitation of the solutions. Do not use a filter needle.
Gently rotate the infusion bag to ensure proper distribution of ALDURAZYME. Do not shake the solution.
The entire infusion volume (100 mL for patients weighing 20 kg or less and 250 mL for patients weighing greater than 20 kg) should be delivered over approximately 3 to 4 hours. The initial infusion rate of 10 µg/kg/hr may be incrementally increased every 15 minutes during the first hour, as tolerated, until a maximum infusion rate of 200 µg/kg/hr is reached. The maximum rate is then maintained for the remainder of the infusion (2-3 hours), as outlined in Tables 1  and 2.
Administer the diluted ALDURAZYME solution to patients using a low-protein-binding infusion set equipped with a low-protein-binding 0.2 µm in-line filter.


* Class of Recommendation:  
[[File:Laronidase dosage.png|none|400px]]
 
|offLabelAdultGuideSupport=There is limited information regarding <i>Off-Label Guideline-Supported Use</i> of {{PAGENAME}} in adult patients.
* Strength of Evidence:
 
* Dosing Information
 
:* Dosage
 
=====Condition2=====
 
There is limited information regarding <i>Off-Label Guideline-Supported Use</i> of {{PAGENAME}} in adult patients.


<!--Non–Guideline-Supported Use (Adult)-->
<!--Non–Guideline-Supported Use (Adult)-->
|offLabelAdultNoGuideSupport======Condition1=====
|offLabelAdultNoGuideSupport=There is limited information regarding <i>Off-Label Non–Guideline-Supported Use</i> of {{PAGENAME}} in adult patients.
 
* Dosing Information
 
:* Dosage
 
=====Condition2=====
 
There is limited information regarding <i>Off-Label Non–Guideline-Supported Use</i> of {{PAGENAME}} in adult patients.


<!--Pediatric Indications and Dosage-->
<!--Pediatric Indications and Dosage-->


<!--FDA-Labeled Indications and Dosage (Pediatric)-->
<!--FDA-Labeled Indications and Dosage (Pediatric)-->
|fdaLIADPed======Condition1=====
|fdaLIADPed=====Indications====
* Mucopolysaccharidosis, Type I (Hurler and Hurler-Scheie forms)


* Dosing Information
====Dosage====
 
* (6 months or older) 0.58 mg/kg of body weight as an IV infusion once a week; pretreat 60 minutes before infusion with antihistamines and/or antipyretics
:* Dosage
|offLabelPedGuideSupport=There is limited information regarding <i>Off-Label Guideline-Supported Use</i> of {{PAGENAME}} in pediatric patients.
 
=====Condition2=====
 
There is limited information regarding <i>FDA-Labeled Use</i> of {{PAGENAME}} in pediatric patients.
 
<!--Off-Label Use and Dosage (Pediatric)-->
 
<!--Guideline-Supported Use (Pediatric)-->
|offLabelPedGuideSupport======Condition1=====
 
* Developed by:
 
* Class of Recommendation:
 
* Strength of Evidence:
 
* Dosing Information
 
:* Dosage
 
=====Condition2=====
 
There is limited information regarding <i>Off-Label Guideline-Supported Use</i> of {{PAGENAME}} in pediatric patients.


<!--Non–Guideline-Supported Use (Pediatric)-->
<!--Non–Guideline-Supported Use (Pediatric)-->
|offLabelPedNoGuideSupport======Condition1=====
|offLabelPedNoGuideSupport=There is limited information regarding <i>Off-Label Non–Guideline-Supported Use</i> of {{PAGENAME}} in pediatric patients.
 
* Dosing Information
 
:* Dosage
 
=====Condition2=====
 
There is limited information regarding <i>Off-Label Non–Guideline-Supported Use</i> of {{PAGENAME}} in pediatric patients.


<!--Contraindications-->
<!--Contraindications-->

Revision as of 20:47, 22 April 2015

Laronidase
Black Box Warning
Adult Indications & Dosage
Pediatric Indications & Dosage
Contraindications
Warnings & Precautions
Adverse Reactions
Drug Interactions
Use in Specific Populations
Administration & Monitoring
Overdosage
Pharmacology
Clinical Studies
How Supplied
Images
Patient Counseling Information
Precautions with Alcohol
Brand Names
Look-Alike Names

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Rabin Bista, M.B.B.S. [2]

Disclaimer

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Black Box Warning

WARNING:
See full prescribing information for complete Boxed Warning.
RISK OF ANAPHYLAXIS:
  • Life-threatening anaphylactic reactions have been observed in some patients during ALDURAZYME® infusions. Therefore, appropriate medical support should be readily available when ALDURAZYME is administered. Patients with compromised respiratory function or acute respiratory disease may be at risk of serious acute exacerbation of their respiratory compromise due to infusion reactions, and require additional monitoring.

Overview

Laronidase is a Enzyme that is FDA approved for the treatment of Hurler and Hurler-Scheie forms of Mucopolysaccharidosis I. There is a Black Box Warning for this drug as shown here. Common adverse reactions include pyrexia, chills, blood pressure increased, tachycardia, and oxygen saturation decreased, rash, upper respiratory tract infection, injection site reaction, hyperreflexia, paresthesia, flushing,.

Adult Indications and Dosage

FDA-Labeled Indications and Dosage (Adult)

Indications

ALDURAZYME® (laronidase) is indicated for patients with Hurler and Hurler-Scheie forms of Mucopolysaccharidosis I (MPS I) and for patients with the Scheie form who have moderate to severe symptoms. The risks and benefits of treating mildly affected patients with the Scheie form have not been established.

ALDURAZYME has been shown to improve pulmonary function and walking capacity. ALDURAZYME has not been evaluated for effects on the central nervous system manifestations of the disorder.

Dosage

Recommended Dose The recommended dosage regimen of ALDURAZYME is 0.58 mg/kg of body weight administered once weekly as an intravenous (IV) infusion. Pretreatment is recommended 60 minutes prior to the start of the infusion and may include antihistamines, antipyretics, or both [SEE WARNINGS AND PRECAUTIONS (5)].

Each vial of ALDURAZYME provides 2.9 milligrams (mg) of laronidase in 5.0 milliliters (mL) of solution and is intended for single use only. Do not use the vial more than one time. The concentrated solution for infusion must be diluted with 0.9% Sodium Chloride Injection, USP, to a final volume of 100 mL or 250 mL, using aseptic techniques. The final volume of the infusion is determined by the patient’s body weight. Patients with a body weight of 20 kg or less should receive a total volume of 100 mL. Patients with a body weight greater than 20 kg should receive a total volume of 250 mL [see DOSAGE AND ADMINISTRATION (2.2)]. For patients with underlying cardiac or respiratory compromise and weighing up to 30 kg, physicians may consider diluting ALDURAZYME in a volume of 100 mL and administering at a decreased infusion rate [see DOSAGE AND ADMINISTRATION (2.2), WARNINGS AND PRECAUTIONS (5.3) and ADVERSE REACTIONS (6.3)].

2.2 Instructions for Use Prepare and use ALDURAZYME according to the following steps. Use aseptic techniques. Prepare ALDURAZYME using low-protein-binding containers and administer with a low-protein-binding infusion set equipped with an in-line, low-protein-binding 0.2 micrometer (µm) filter. There is no information on the compatibility of diluted ALDURAZYME with glass containers.

Determine the number of vials to be diluted based on the patient's weight and the recommended dose of 0.58 mg/kg, using the following equation: 

                   Patient's weight (kg) x 1 mL/kg of ALDURAZYME = Total number mL of ALDURAZYME
                         Total number mL of ALDURAZYME ÷ 5 mL per Vial = Total number of Vials.

Round up to the next whole vial. Remove the required number of vials from the refrigerator to allow them to reach room temperature. Do not heat or microwave vials. Before withdrawing the ALDURAZYME from the vial, visually inspect each vial for particulate matter and discoloration. The ALDURAZYME solution should be clear to slightly opalescent and colorless to pale yellow. Some translucency may be present in the solution. Do not use if the solution is discolored or if there is particulate matter in the solution. Withdraw and discard a volume of the 0.9% Sodium Chloride Injection, USP from the infusion bag, equal to the volume of ALDURAZYME concentrate to be added. Slowly withdraw the calculated volume of ALDURAZYME from the appropriate number of vials using caution to avoid excessive agitation. Do not use a filter needle, as this may cause agitation. Agitation may denature ALDURAZYME, rendering it biologically inactive. Slowly add the ALDURAZYME solution to the 0.9% Sodium Chloride Injection, USP using care to avoid agitation of the solutions. Do not use a filter needle. Gently rotate the infusion bag to ensure proper distribution of ALDURAZYME. Do not shake the solution. The entire infusion volume (100 mL for patients weighing 20 kg or less and 250 mL for patients weighing greater than 20 kg) should be delivered over approximately 3 to 4 hours. The initial infusion rate of 10 µg/kg/hr may be incrementally increased every 15 minutes during the first hour, as tolerated, until a maximum infusion rate of 200 µg/kg/hr is reached. The maximum rate is then maintained for the remainder of the infusion (2-3 hours), as outlined in Tables 1 and 2. Administer the diluted ALDURAZYME solution to patients using a low-protein-binding infusion set equipped with a low-protein-binding 0.2 µm in-line filter.

Off-Label Use and Dosage (Adult)

Guideline-Supported Use

There is limited information regarding Off-Label Guideline-Supported Use of Laronidase in adult patients.

Non–Guideline-Supported Use

There is limited information regarding Off-Label Non–Guideline-Supported Use of Laronidase in adult patients.

Pediatric Indications and Dosage

FDA-Labeled Indications and Dosage (Pediatric)

Indications

  • Mucopolysaccharidosis, Type I (Hurler and Hurler-Scheie forms)

Dosage

  • (6 months or older) 0.58 mg/kg of body weight as an IV infusion once a week; pretreat 60 minutes before infusion with antihistamines and/or antipyretics

Off-Label Use and Dosage (Pediatric)

Guideline-Supported Use

There is limited information regarding Off-Label Guideline-Supported Use of Laronidase in pediatric patients.

Non–Guideline-Supported Use

There is limited information regarding Off-Label Non–Guideline-Supported Use of Laronidase in pediatric patients.

Contraindications

  • Condition1

Warnings

WARNING:
See full prescribing information for complete Boxed Warning.
RISK OF ANAPHYLAXIS:
  • Life-threatening anaphylactic reactions have been observed in some patients during ALDURAZYME® infusions. Therefore, appropriate medical support should be readily available when ALDURAZYME is administered. Patients with compromised respiratory function or acute respiratory disease may be at risk of serious acute exacerbation of their respiratory compromise due to infusion reactions, and require additional monitoring.
  • Description

Precautions

  • Description

Adverse Reactions

Clinical Trials Experience

There is limited information regarding Clinical Trial Experience of Laronidase in the drug label.

Body as a Whole
Cardiovascular
Digestive
Endocrine
Hematologic and Lymphatic
Metabolic and Nutritional
Musculoskeletal
Neurologic
Respiratory
Skin and Hypersensitivy Reactions
Special Senses
Urogenital
Miscellaneous

Postmarketing Experience

There is limited information regarding Postmarketing Experience of Laronidase in the drug label.

Body as a Whole
Cardiovascular
Digestive
Endocrine
Hematologic and Lymphatic
Metabolic and Nutritional
Musculoskeletal
Neurologic
Respiratory
Skin and Hypersensitivy Reactions
Special Senses
Urogenital
Miscellaneous

Drug Interactions

  • Drug
  • Description

Use in Specific Populations

Pregnancy

Pregnancy Category (FDA):

  • Pregnancy Category


Pregnancy Category (AUS):

  • Australian Drug Evaluation Committee (ADEC) Pregnancy Category

There is no Australian Drug Evaluation Committee (ADEC) guidance on usage of Laronidase in women who are pregnant.

Labor and Delivery

There is no FDA guidance on use of Laronidase during labor and delivery.

Nursing Mothers

There is no FDA guidance on the use of Laronidase with respect to nursing mothers.

Pediatric Use

There is no FDA guidance on the use of Laronidase with respect to pediatric patients.

Geriatic Use

There is no FDA guidance on the use of Laronidase with respect to geriatric patients.

Gender

There is no FDA guidance on the use of Laronidase with respect to specific gender populations.

Race

There is no FDA guidance on the use of Laronidase with respect to specific racial populations.

Renal Impairment

There is no FDA guidance on the use of Laronidase in patients with renal impairment.

Hepatic Impairment

There is no FDA guidance on the use of Laronidase in patients with hepatic impairment.

Females of Reproductive Potential and Males

There is no FDA guidance on the use of Laronidase in women of reproductive potentials and males.

Immunocompromised Patients

There is no FDA guidance one the use of Laronidase in patients who are immunocompromised.

Administration and Monitoring

Administration

  • Oral
  • Intravenous

Monitoring

There is limited information regarding Monitoring of Laronidase in the drug label.

  • Description

IV Compatibility

There is limited information regarding IV Compatibility of Laronidase in the drug label.

Overdosage

Acute Overdose

Signs and Symptoms

  • Description

Management

  • Description

Chronic Overdose

There is limited information regarding Chronic Overdose of Laronidase in the drug label.

Pharmacology

There is limited information regarding Laronidase Pharmacology in the drug label.

Mechanism of Action

Structure

File:Laronidase01.png
This image is provided by the National Library of Medicine.

Pharmacodynamics

There is limited information regarding Pharmacodynamics of Laronidase in the drug label.

Pharmacokinetics

There is limited information regarding Pharmacokinetics of Laronidase in the drug label.

Nonclinical Toxicology

There is limited information regarding Nonclinical Toxicology of Laronidase in the drug label.

Clinical Studies

There is limited information regarding Clinical Studies of Laronidase in the drug label.

How Supplied

Storage

There is limited information regarding Laronidase Storage in the drug label.

Images

Drug Images

{{#ask: Page Name::Laronidase |?Pill Name |?Drug Name |?Pill Ingred |?Pill Imprint |?Pill Dosage |?Pill Color |?Pill Shape |?Pill Size (mm) |?Pill Scoring |?NDC |?Drug Author |format=template |template=DrugPageImages |mainlabel=- |sort=Pill Name }}

Package and Label Display Panel

{{#ask: Label Page::Laronidase |?Label Name |format=template |template=DrugLabelImages |mainlabel=- |sort=Label Page }}

Patient Counseling Information

There is limited information regarding Patient Counseling Information of Laronidase in the drug label.

Precautions with Alcohol

  • Alcohol-Laronidase interaction has not been established. Talk to your doctor about the effects of taking alcohol with this medication.

Brand Names

Look-Alike Drug Names

Drug Shortage Status

Price

References

The contents of this FDA label are provided by the National Library of Medicine.

  1. ↑ Empty citation (help)
  2. ↑ "http://www.ismp.org". External link in |title= (help)

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