Dosing Calculator
As your patients with MPS I grow, so should their Aldurazyme® treatment1
Calculating the recommended dose of Aldurazyme® for children and adults with MPS I
Recommended dosing regimen:
• 100 U/kg of body weight administered once a week*1
• No dose adjustment is required for pediatric patients1
How to calculate dose:
To calculate the recommended dose of Aldurazyme® in U and mg per week, and the corresponding number of vials required, enter the patient’s body weight (1–120 kg) in the box.
Please refer to the Summary of Product Characteristics for full information on the use of Aldurazyme® for the treatment of MPS I.
* The activity unit (U) is defined as the hydrolysis of one micromole of substrate (4-MUI) per minute.1
Administration
Administering the Aldurazyme® infusion
Recommended Aldurazyme® infusion rate and speed:
The initial infusion rate of 2 U/kg/h may be incrementally increased every fifteen minutes, if tolerated, to a maximum of 43 U/kg/h.1
If body weight ≤20 kg1
Total infusion volume of 100 ml to be delivered in approximately 3–4 hours.
| Infusion rate (U/kg/h) | Infusion speed (ml/h) |
| 2 | 2 |
| 4 | 4 |
| 8 | 8 |
| 16 | 16 |
| 32 | 32 |
| 43 | 43 |
If body weight >20 kg1
Total infusion volume of 250 ml to be delivered in approximately 3–4 hours.
| Infusion rate (U/kg/h) | Infusion speed (ml/h) |
| 2 | 5 |
| 4 | 10 |
| 8 | 20 |
| 16 | 40 |
| 32 | 80 |
| 43 | ≈107 |
For the initial administration of Aldurazyme® or upon re-administration following interruption of treatment, it is recommended that patients be administered pre-treatment medicines (antihistamines and/or antipyretics) approximately 60 minutes prior to the start of the infusion, to minimize the potential occurrence of infusion-associated reactions.1
If clinically indicated, administration of pre-treatment medications with subsequent infusions of Aldurazyme® should be considered.1
Aldurazyme® treatment should be supervised by a physician experienced in the management of patients with MPS I or other inherited metabolic diseases. Administration of Aldurazyme® should be carried out in an appropriate clinical setting where resuscitation equipment to manage medical emergencies would be readily available.1
Safety
Safety information
Infusion associated reactions (IAR)
In case of a:1
 |
Mild or moderate IAR, treatment with antihistamines and paracetamol/ibuprofen should be considered and/or a reduction in the infusion rate to half the infusion rate at which the reaction occurred. |
 |
Recurrent moderate IAR or re-challenge after a single severe IAR, pre-treatment should be considered (antihistamines and paracetamol/ ibuprofen and/or corticosteroids) and a reduction of the infusion rate to 1/2 – 1/4 the rate of the infusion at which the previous reaction occurred. |
 |
Single severe IAR, the infusion should be stopped until the symptoms are resolved and treatment with antihistamines and paracetamol/ ibuprofen should be considered. The infusion can be restarted with a reduction of the infusion rate to 1/2 – 1/4 the rate of the infusion at which the reaction occurred. |
Most frequent adverse drug reactions (ADRs)
The most frequent ADRs in clinical trials were: headache, nausea, abdominal pain, rash, arthralgia, backpain, pain at extremity, flushing, pyrexia, infusion site reactions, blood pressure increased, oxygen saturation decreased, tachycardia and chills.1
Post-marketing experience of infusion-associated reactions revealed reporting of cyanosis, hypoxia, tachypnoea, pyrexia, vomiting, chills and erythema, in which some of these reactions were severe.1
MAT-KW-2300049-V1-Feb-23