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Table 6 Summary of patients undergoing desensitization for drug hypersensitivity

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

Sex/age Phenotype/CRIM status Symptoms Immune reaction Premedication Desensitization: initial concentration Desensitization: total dose Desensitization: adverse reactions
M, 11 months [79] IOPD CP Urticarial rash Elevated serum tryptase, activated complement, IgE and eosinophil count normal Tranexamic acid, deflazacort, cetirizine, ranitidine 0.5 μg/ml 10 mg/kg (only for the first infusion) minimal skin reactions
M, 4 months [80] IOPD CP Anaphylaxis IgE and eosinophil count normal Tranexamic acid, deflazacort, cetirizine, ranitidine 15 μg/ml Full dose 20 mg/kg EOW NO
F, 4 months [82] IOPD, CRIM ND Urticarial rash intradermal test positive, SPT negative Diphenhydramine, methylprednisolone 0.1 μg/ml 20 mg/kg EOW NO
M, 3 years 8 months [69] Juvenile onset PD CP Anaphylaxis IgG 1:51200, negative IgE titers, complement activation, normal serum tryptase Diphenhydramine, acetaminophen 0.07 μg/ml 10 mg/kg/week severe reactions (requiring epinephrine)
M, 10 months [69] IOPD CP Urticarial rash Elevated IgE and serum tryptase, negative IgG and no complement activation No 0.05 μg/ml 10 mg/kg/weekly minor skin reaction
F, 6 years 9 months [74] IOPD CP Urticarial rash SPT negative, IgG negative Prednisolone 0.01 μg/ml 10 mg/kg/weekly Urticarial rash
F, 15 months [75] IOPD CP Anaphylaxis IgG 1:6400, normal IgE, tryptase and complement No 1:100 0.2 mg/kg. The dose was doubled every week No
F 7 months [82] IOPD CP Urticarial rash, intractable cough and stridor Intradermal test positive, SPT negative No 0.05 μg/ml 10 mg/kg/week Urticarial rash
F 28 y [56] LOPD Anaphylaxis IgE positive, IgG 1600, SPT negative, intradermal testing positive, complement testing negative, elevated tryptase levels Diphenhydramine and prednisone 0.1 μg/ml 10 mg/kg/week severe reactions (requiring epinephrine)
F, 46 years [56] LOPD Anaphylaxis SPT and intradermal tests negative, normal IgE, eosinophil counts, complement, tryptase Antihistamines H1 (cetirizine), antileukotriene (montelukast) Drop by drop of ERT solution 2 mg/ml with increasing flow + saline solution with continuous flow by another perfusion pocket Full dose Minor reaction: anxiety, crying, abdominal pain
  1. IOPD infantile onset Pompe disease, LOPD late onset Pompe disease, CP CRIM positive, CN CRIM negative, ND not determined, SPT skin prick test, EOW every other week