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Desensitization of an adult patient with Pompe disease and a history of anaphylaxis to alglucosidase alfa.

Author(s): Lipinski SE, Lipinski MJ, Burnette A, Platts-Mills TA, Wilson WG

Affiliation(s): Division of Medical Genetics, Department of Pediatrics, University of Virginia Health System, Charlottesville, VA 22908-0386, USA. slipinski@virginia.edu

Publication date & source: 2009-11, Mol Genet Metab., 98(3):319-21. Epub 2009 Jul 7.

Publication type: Case Reports; Research Support, Non-U.S. Gov't

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.

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