Table 1.

Patient 1. Child with infantile PD, IgE positive and recurrent significant reactions.

Modified desensitization protocol for severe reactions to alglucosidase alpha infusions (Standard recommended dose is 20 mg/kg biweekly)	Individualized protocol based on age (clinical presentation), weight (kg), cardiac status, arrhythmia risk, fluid tolerance, and IgE status
Total dose given was 10 mg/kg weekly Micro-dilutions were used for the first 2 h of the infusion The infusion rate was increased every 30 min and every hour the concentration was increased The remainder of the infusion was given at one-half the standard ramp up step-up rate as: 0.5, 1.5, 2.5, and 3.5 mg/kg/h Infusions (19–24) were administered using this protocol.	**Initial micro-dilutions were prepared as follows at infusion 19: 0.05, 0.5, 5, and 50 mcg/ml Resulting infusions were given as: 0.045, 0.15, 0.45, and1.5 mcg/kg/h, with increments every 30 min. The remainder of the infusion was mixed at 1 mg/ml stepping up every 30 min if no IAR occurred The total volume infused was 143 ml over approximately 6 h. First micro dilution started at 6.65 mcg/ml, resulting in infusion of 6 mcg/kg/h, then 20 mcg/kg/h Second micro-dilution started at 66.5 mcg/ml resulting in infusion of 60 mcg/kg/h, then 200 mcg/kg/h The remainder was administered at half the usual step-up rate (15-minute increments) each of 0.5,1, 2, 3.3. and 5 mg/kg/h Total infusion time was 6 h and 30 min
After two successive infusions were tolerated using the initial micro dilution preparation** modifications were made as follows: The standard total dose of 20 mg/kg every other week was given in gradually increasing concentrations, administered cautiously as separate micro dilutions (the first and second seen across the table in bold) The remainder of the infusions was administered at approximately half the usual step-up rate 15–30-minute increments as tolerated The rate was increased over time until the infusion time lasted approx 4 h, 15 min