Alglucosidase-α
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Reactions 1517, p21 - 6 Sep 2014 S Alglucosidase-α Anaphylactic shock, with subsequent desensitisation: case report A female infant [age not stated] developed anaphylaxis while receiving alglucosidase-α; she later underwent desensitisation. The female infant, who was diagnosed with Pompe disease (PD), started receiving enzyme replacement therapy (ERT) with alglucosidase-α at a standard dose of 20 mg/kg alternate weekly in the first month of the postnatal period. An echocardiograph study revealed hypertrophy in the interventricular septum. She experienced anaphylaxis within 15 minutes of initiating the 12 th ERT infusion. An intradermal test with alglucosidase-α at a concentration of 1:1000 was positive. The female infant underwent desensitization therapy without serious complications. She received ERT therapy without any further allergic reactions. Author comment: "We propose a new desensitization protocol, which follows general desensitization rules, used for the youngest infantile Pompe patient, who developed anaphylaxis to [alglucosidase-α]." Kucukcongar A, et al. Desensitization of an infant with pompe disease and a history of anaphylaxis to alglucosidase alfa. Journal of Inherited Metabolic Disease 35 (Suppl. 1): S108 abstr. A-024, Sep 2012. Available from: URL: http:// doi.org/10.1007/s10545-012-9512-z [abstract] - Turkey 803107727 1 Reactions 6 Sep 2014 No. 1517 0114-9954/14/1517-0001/$14.95 Adis © 2014 Springer International Publishing AG. All rights reserved
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Transcript of Alglucosidase-α
Reactions 1517, p21 - 6 Sep 2014
SAlglucosidase-αAnaphylactic shock, with subsequentdesensitisation: case report
A female infant [age not stated] developed anaphylaxis whilereceiving alglucosidase-α; she later underwentdesensitisation.
The female infant, who was diagnosed with Pompe disease(PD), started receiving enzyme replacement therapy (ERT)with alglucosidase-α at a standard dose of 20 mg/kg alternateweekly in the first month of the postnatal period. Anechocardiograph study revealed hypertrophy in theinterventricular septum. She experienced anaphylaxis within15 minutes of initiating the 12th ERT infusion. An intradermaltest with alglucosidase-α at a concentration of 1:1000 waspositive.
The female infant underwent desensitization therapywithout serious complications. She received ERT therapywithout any further allergic reactions.
Author comment: "We propose a new desensitizationprotocol, which follows general desensitization rules, used forthe youngest infantile Pompe patient, who developedanaphylaxis to [alglucosidase-α]."Kucukcongar A, et al. Desensitization of an infant with pompe disease and ahistory of anaphylaxis to alglucosidase alfa. Journal of Inherited Metabolic Disease35 (Suppl. 1): S108 abstr. A-024, Sep 2012. Available from: URL: http://doi.org/10.1007/s10545-012-9512-z [abstract] - Turkey 803107727
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Reactions 6 Sep 2014 No. 15170114-9954/14/1517-0001/$14.95 Adis © 2014 Springer International Publishing AG. All rights reserved
