Abstract
Enzyme replacement therapy (ERT) is important for the treatment of lysosomal storage disorders. Hypersensitivity reactions with ERT have been reported, and in these cases, desensitisation with the enzyme is necessary. Here we report the cases of 3 patients with lysosomal storage disorders, including Pompe disease and mucopolysaccharidosis type I and VI, who had IgE-mediated hypersensitivity reactions and positive skin tests. Successful desensitisation protocols with the culprit enzyme solution were used for these patients. All 3 patients were able to safely receive ERT with the desensitisation protocol.
© 2016 S. Karger AG, Basel.
MeSH terms
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Allergens / immunology
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Child, Preschool
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Desensitization, Immunologic*
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Enzyme Replacement Therapy / adverse effects*
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Enzymes / administration & dosage
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Enzymes / adverse effects*
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Female
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Glycogen Storage Disease Type II / complications*
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Glycogen Storage Disease Type II / diagnosis
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Glycogen Storage Disease Type II / therapy
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Humans
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Hypersensitivity, Immediate / complications*
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Hypersensitivity, Immediate / diagnosis
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Hypersensitivity, Immediate / therapy*
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Infant
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Male
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Mucopolysaccharidosis I / complications*
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Mucopolysaccharidosis I / diagnosis
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Mucopolysaccharidosis I / therapy
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Mucopolysaccharidosis VI / complications*
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Mucopolysaccharidosis VI / diagnosis
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Mucopolysaccharidosis VI / therapy
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N-Acetylgalactosamine-4-Sulfatase / administration & dosage
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N-Acetylgalactosamine-4-Sulfatase / immunology
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Recombinant Proteins / adverse effects
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alpha-Glucosidases / administration & dosage
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alpha-Glucosidases / immunology
Substances
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Allergens
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Enzymes
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Recombinant Proteins
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N-Acetylgalactosamine-4-Sulfatase
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GAA protein, human
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alpha-Glucosidases