title: Desensitization of an adult patient with Pompe disease and a history of anaphylaxis to alglucosidase alfa.
abstract: We report on the successful desensitization of an adult female with Pompe disease who had previously experienced anaphylaxis to intravenous alglucosidase alfa therapy. The starting alglucosidase alfa dose for desensitization was 10mg/kg with gradual dose escalation and desensitization via serial dilution was completed over five infusions. This methodology serves as a means to desensitize patients with prior anaphylactic response to alglucosidase alfa so that enzyme replacement therapy can be utilized.
authors.affiliation: Division of Medical Genetics, Department of Pediatrics, University of Virginia Health System, Charlottesville, VA 22908-0386, USA. slipinski@virginia.edu
authors.forename: Shawn E
authors.initials: SE
authors.lastname: Lipinski
authors.forename: Michael J
authors.initials: MJ
authors.lastname: Lipinski
authors.forename: Autumn
authors.initials: A
authors.lastname: Burnette
authors.forename: Thomas A
authors.initials: TA
authors.lastname: Platts-Mills
authors.forename: William G
authors.initials: WG
authors.lastname: Wilson
mesh_terms: D000328:Adult; D000707:Anaphylaxis / Q000188:drug therapy* / Q000276:immunology; D003888:Desensitization, Immunologic; D056947:Enzyme Replacement Therapy; D005260:Female; D006009:Glycogen Storage Disease Type II / Q000188:drug therapy*; D006801:Humans; D016896:Treatment Outcome; D000520:alpha-Glucosidases / Q000009:adverse effects / Q000276:immunology / Q000627:therapeutic use*
chemical_list: C509951:GAA protein, human; D000520:alpha-Glucosidases