Mild to moderate alpha-mannosidosis

Active Ingredient: Velmanase alfa

Indication for Velmanase alfa

Population group: only children (1 year - 12 years old) , adolescents (12 years - 18 years old) , adults (18 - 65 years old)

Enzyme replacement therapy for the treatment of non-neurological manifestations in patients with mild to moderate alpha-mannosidosis.

For this indication, competent medicine agencies globally authorize below treatments:

1 mg/kg once a week

Route of admnistration

Intravenous

Defined daily dose

1 - 1 mg

Dosage regimen

From 1 To 1 mg once every 7 day(s)

Detailed description

The recommended dose regimen is 1 mg/kg of body weight administered once every week by intravenous infusion at a controlled speed.

Dosage considerations

The reconstituted solution should be administered using an infusion set equipped with a pump and an in-line low protein-binding 0.22 Ξm filter. The total volume of infusion is determined by the patient’s weight and should be administrated over a minimum of 50 minutes. For patients weighing less than 18 kg, and receiving less than 9 mL reconstituted solution, the infusion rate should be calculated so that the infusion time is â‰Ĩ50 minutes. The maximum infusion rate is 25 mL/hour.

The infusion time can be calculated from the following table:

Patient weight (kg) Dose (ml) Maximum infusion rate (ml/h) Minimum infusion time (min) Patient weight (kg) Dose (ml) Maximum infusion rate (ml/h) Minimum infusion time (min)
5 2.5 3 50 53 26.5 25 64
6 3 3.6 50 54 27 25 65
7 3.5 4.2 50 55 27.5 25 67
8 4 4.8 50 56 28 25 67
9 4.5 5.4 50 57 28.5 25 68
10 5 6 50 58 29 25 70
11 5.5 6.6 50 59 29.5 25 71
12 6 7.2 50 60 30 25 72
13 6.5 7.8 50 61 30.5 25 73
14 7 8.4 50 62 31 25 74
15 7.5 9 50 63 31.5 25 76
16 8 9.6 50 64 32 25 77
17 8.5 10.2 50 65 32.5 25 78
18 9 10.8 50 66 33 25 79
19 9.5 11.4 50 67 33.5 25 80
20 10 12 50 68 34 25 82
21 10.5 12.6 50 69 34.5 25 83
22 11 13.2 50 70 35 25 84
23 11.5 13.8 50 71 35.5 25 85
24 12 14.4 50 72 36 25 86
25 12.5 15 50 73 36.5 25 88
26 13 15.6 50 74 37 25 89
27 13.5 16.2 50 75 37.5 25 90
28 14 16.8 50 76 38 25 91
29 14.5 17.4 50 77 38.5 25 92
30 15 18 50 78 39 25 94
31 15.5 18.6 50 79 39.5 25 95
32 16 19.2 50 80 40 25 96
33 16.5 19.8 50 81 40.5 25 97
34 17 20.4 50 82 41 25 98
35 17.5 21 50 83 41.5 25 100
36 18 21.6 50 84 42 25 101
37 18.5 22.2 50 85 42.5 25 102
38 19 22.8 50 86 43 25 103
39 19.5 23.4 50 87 43.5 25 104
40 20 24 50 88 44 25 106
41 20.5 24.6 50 89 44.5 25 107
42 21 25 50 90 45 25 108
43 21.5 25 52 91 45.5 25 109
44 22 25 53 92 46 25 110
45 22.5 25 54 93 46.5 25 112
46 23 25 55 94 47 25 113
47 23.5 25 56 95 47.5 25 114
48 24 25 58 96 48 25 115
49 24.5 25 59 97 48.5 25 116
50 25 25 60 98 49 25 118
51 25.5 25 61 99 49.5 25 119
52 26 25 62    

Active ingredient

Velmanase alfa

Velmanase alfa is a recombinant form of human alpha-mannosidase. Velmanase alfa is intended to supplement or replace natural alpha-mannosidase, an enzyme that catalyses the sequential degradation of hybrid and complex high-mannose oligosaccharides in the lysosome, reducing the amount of accumulated mannose-rich oligosaccharides.

Read more about Velmanase alfa

Related medicines

Summary of Product Characteristics (SPC) 2022: LAMZEDE Powder for solution for infusion

Develop a tailored medication plan for your case, considering factors such as age, gender, and health history

Ask the Reasoner

Liability Disclaimer : RxReasoner has utilized reasonable care in providing content and services that are accurate, complete and up to date. However, RxReasoner does not accept any responsibility or liability about it. The content and services of RxReasoner are for informational purposes only and they are not intended to be a substitute for the knowledge, expertise, skill, and judgment of physicians, pharmacists, nurses, or other healthcare professionals involved in patient care. RxReasoner offers no medical advice. Users are responsible for the use of the provided content. A shown indication or treatment should not be construed to indicate that the medication is safe, appropriate, or effective in any given patient or under any particular circumstances. The absence of an indication or treatment should not roule out the existence of other appropriate medications. Always seek the advice of a physician or other qualified health provider with any questions you may have regarding a medical condition or medicament. RxReasoner is not liable for any damages allegedly sustained arising out of the use of its content and services.

⇈