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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