Skip to main content

Table 7 Proposal of desensitisation protocol

From: Immune responses to alglucosidase in infantile Pompe disease: recommendations from an Italian pediatric expert panel

Initial dose 10 mg/kg weekly
Dose increase [69] ¾ of the dose every 10 days (after 2 successive successfully infusions) → full dose at a modified rate scheduled every 2 weeks → standard recommended rate
Initial concentration [72] 1/10,000–1/100 of the full therapeutic dose. If anaphylaxis is severe: 1/1,000,000–1/10,000 of the full therapeutic dose. If positive skin test, the starting dose can be determined based on the endpoint titration
Concentration increase [74] Doubling or three-fold every 15–20 min until the therapeutic dose is achieved