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 μm 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 ≥50 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

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