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dc.contributor.authorTamay, Zeynep
dc.contributor.authorGokcay, Gulden
dc.contributor.authorDilek, Fatih
dc.contributor.authorBalci, Mehmet Cihan
dc.contributor.authorOzceker, Deniz
dc.contributor.authorDemirkol, Mubeccel
dc.contributor.authorGuler, Nermin
dc.contributor.editorBaumgartner, M
dc.contributor.editorPatterson, M
dc.contributor.editorRahman, S
dc.contributor.editorZschocke, J
dc.contributor.editorMorava, E
dc.contributor.editorPeters, V
dc.date.accessioned2019-08-13T12:10:23Z
dc.date.accessioned2019-08-13T15:57:36Z
dc.date.available2019-08-13T12:10:23Z
dc.date.available2019-08-13T15:57:36Z
dc.date.issued2016
dc.identifier.isbn978-3-662-53681-0 -- 978-3-662-53680-3
dc.identifier.issn2192-8304
dc.identifier.urihttps://dx.doi.org/10.1007/8904_2016_542
dc.identifier.urihttp://hdl.handle.net/11446/2548
dc.descriptionWOS: 000405119300008en_US
dc.descriptionPubMed ID: 26951141en_US
dc.description.abstractMucopolysaccharidosis type VI (MPS VI) is a progressive, chronic, and multisystem lysosomal storage disease. Enzyme replacement therapy (ERT) with the recombinant human arylsulfatase B enzyme (galsulfase [Naglazyme]) is recommended as first-line therapy. It is generally reported as safe and well tolerated. Frequently observed mild to moderate infusion-related reactions which can be easily handled by reducing or interrupting the infusion and/or administering additional antihistamines, antipyretics, and corticosteroids are mostly mediated by non-IgE mechanisms. Here we report two children with MPS VI who experienced IgE-mediated reactions with galsulfase at the second year of the therapy. One child had anaphylaxis and the other had urticarial eruptions. They could receive ERT after successful rapid desensitization. To our knowledge, this is the second report on galsulfase allergy with IgE-mediated reaction. It is important to recognize IgE-mediated reactions since they can be life-threatening and do not respond to the standard therapies. We recommend allergy skin tests in the evaluation of infusion-related reactions unresponsive to standard therapies, so that continuation of ERT will be feasible after successful desensitization.en_US
dc.language.isoengen_US
dc.publisherSPRINGER-VERLAG BERLINen_US
dc.relation.ispartofseriesJIMD Reports
dc.relation.isversionof10.1007/8904_2016_542en_US
dc.rightsinfo:eu-repo/semantics/openAccessen_US
dc.titleRapid Desensitization for Immediate Hypersensitivity to Galsulfase Therapy in Patients with MPS VIen_US
dc.typearticleen_US
dc.relation.journalJIMD REPORTS, VOL 30en_US
dc.contributor.departmentDBÜen_US
dc.identifier.volume30en_US
dc.identifier.startpage53en_US
dc.identifier.endpage57en_US
dc.contributor.authorID0000-0002-6817-4715en_US
dc.relation.publicationcategoryMakale - Uluslararası Hakemli Dergi - Kurum Öğretim Elemanıen_US


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