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XENPOZYME (olipudase alfa-rpcp)
Treatment of non–central nervous system manifestations of acid sphingomyelinase deficiency (ASMD) in adult and pediatric patients.
Recommended Dosage in Adult Patients (18 years and older):
Dose Escalation Phase:
The recommended starting dose of XENPOZYME in adults is 0.1 mg/kg.In order to reduce the risk of hypersensitivity and infusion-associated reactions or elevated transaminase levels, follow the dose escalation regimen in Table 1.
Administer XENPOZYME via intravenous infusion every 2 weeks.
Table 1: XENPOZYME Dose Escalation Regimen for Adult Patients*:
|
First dose (Day 1/Week 0 ) |
0.1 mg/kg |
|
Second dose (Week 2) |
0.3 mg/kg |
|
Third dose (Week 4) |
0.3 mg/kg |
|
Fourth dose (Week 6) |
0.6 mg/kg |
|
Fifth dose (Week 8) |
0.6 mg/kg |
|
Sixth dose (Week 10) |
1 mg/kg |
|
Seventh dose (Week 12) |
2 mg/kg |
|
Eighth dose (Week 14) † |
3 mg/kg (recommended maintenance dose) |
* Use actual body weight for patients with a BMI less than or equal to 30. For patients with a BMI greater than 30, calculate adjusted body weight (kg) = (actual height in m)2 x 30.
† The dose escalation phase includes the first 3 mg/kg dose.
Recommended Dosage in Pediatric Patients:
Dose Escalation Phase:
The recommended starting dose of XENPOZYME in pediatric patients is 0.03 mg/kg. In order to reduce the risk of hypersensitivity and infusion-associated reactions or elevated liver enzyme elevations, follow the dose escalation regimen in Table 2.
Administer XENPOZYME via intravenous infusion every 2 weeks.
Table 2: XENPOZYME Dose Escalation Regimen for Pediatric Patients*:
|
First dose (Day 1/Week 0 ) |
0.03 mg/kg |
|
Second dose (Week 2) |
0.1 mg/kg |
|
Third dose (Week 4) |
0.3 mg/kg |
|
Fourth dose (Week 6) |
0.3 mg/kg |
|
Fifth dose (Week 8) |
0.6 mg/kg |
|
Sixth dose (Week 10) |
0.6 mg/kg |
|
Seventh dose (Week 12) |
1 mg/kg |
|
Eighth dose (Week 14) |
2 mg/kg |
|
Ninth dose (Week 16)† |
3 mg/kg (recommended maintenance dose) |
* Use actual body weight for patients with a BMI less than or equal to 30. For patients with a BMI greater than 30, calculate adjusted body weight (kg) = (actual height in m)2 x 30
† The dose escalation phase includes the first 3 mg/kg dose.
Maintinance dose:
3 mg/ kg IV every 2 weeks.(197)
For injection: 20 mg of olipudase alfa-rpcp as a lyophilized powder in a single-dose vial for reconstitution.
IV
Headache, cough, diarrhea, hypotension, ocular hyperemia, pyrexia, cough, diarrhea, rhinitis, abdominal pain, vomiting, urticaria, nausea, rash, arthralgia, pruritus, fatigue and pharyngitis.
1b
197. Melissa Wasserstein, Robin Lachmann, Carla Hollak, Laila Arash-Kaps, Antonio Barbato, Renata C Gallagher, Roberto Giugliani, et al., A randomized, placebo-controlled clinical trial evaluating olipudase alfa enzyme replacement therapy for chronic acid sphingomyelinase deficiency (ASMD) in adults: One-year results. Clinical Trial Genet Med . 2022 Jul;24(7):1425-1436. doi: 10.1016/j.gim.2022.03.021.
