{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,7,30]],"date-time":"2025-07-30T07:50:01Z","timestamp":1753861801684,"version":"3.41.2"},"reference-count":9,"publisher":"Wiley","issue":"8","license":[{"start":{"date-parts":[[2025,4,12]],"date-time":"2025-04-12T00:00:00Z","timestamp":1744416000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["American J of Med Genetics Pt A"],"published-print":{"date-parts":[[2025,8]]},"abstract":"<jats:title>ABSTRACT<\/jats:title><jats:p>Idursulfase is the first\u2010line and only available enzyme replacement therapy (ERT) for Mucopolysaccharidosis type II (MPS II), or Hunter Syndrome. Deficiency in the lysosomal enzyme iduronate\u20102\u2010sulfatase leads to progressive skeletal deformities, neurologic deterioration, airway obstruction, and cardiomyopathy. In severe cases, these deformities can lead to death during teenage years (Stapleton et\u00a0al. 2017). Continuous treatment with ERT is essential to prevent irreversible changes. However, 16 out of 108 (15%) patients had hypersensitivity reactions to idursulfase during clinical trials. Hypersensitivity reactions have also been reported several years into treatment (Elaprase 2018). Therefore, it is critical to evaluate for hypersensitivity reactions and desensitize patients to idursulfase. We report a fourteen\u2010year\u2010old male who was evaluated using a nonirritating skin test concentration and underwent a novel desensitization protocol for Lysosomal Storage Disease ERT.<\/jats:p>","DOI":"10.1002\/ajmg.a.64089","type":"journal-article","created":{"date-parts":[[2025,4,12]],"date-time":"2025-04-12T11:02:22Z","timestamp":1744455742000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Hypersensitivity Reaction and a Single\u2010Bag Rapid Desensitization to Idursulfase"],"prefix":"10.1002","volume":"197","author":[{"ORCID":"https:\/\/orcid.org\/0009-0004-9464-2254","authenticated-orcid":false,"given":"Jenna H.","family":"Jung","sequence":"first","affiliation":[{"name":"Work Conducted While Affiliated With Northwell Health, Division of Allergy and Immunology, Department of Pediatrics Donald and Barbara Zucker School of Medicine at Hofstra\/Northwell  Hempstead New York USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Berrin","family":"Monteleone","sequence":"additional","affiliation":[{"name":"Clinical Genetics. NYU Langone Hospital\u2013Long Island  Long Island New York USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Meghan","family":"McGath","sequence":"additional","affiliation":[{"name":"Clinical Genetics. NYU Langone Hospital\u2013Long Island  Long Island New York USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Blanka","family":"Kaplan","sequence":"additional","affiliation":[{"name":"Northwell Health, Division of Allergy and Immunology, Department of Pediatrics Donald and Barbara Zucker School of Medicine at Hofstra\/Northwell  Hempstead New York USA"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2025,4,12]]},"reference":[{"key":"e_1_2_5_2_1","unstructured":"Elaprase.2018.\u201cLexington MA: Shire Human Genetic Therapies Inc.\u201d"},{"key":"e_1_2_5_3_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ymgmr.2022.100878"},{"key":"e_1_2_5_4_1","doi-asserted-by":"publisher","DOI":"10.1111\/all.12155"},{"key":"e_1_2_5_5_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jaip.2017.10.031"},{"key":"e_1_2_5_6_1","doi-asserted-by":"publisher","DOI":"10.1007\/s40629\u2010021\u201000173\u20107"},{"issue":"7","key":"e_1_2_5_7_1","first-page":"571","article-title":"Successful Desensitization to Idursulfase in a Patient With Type II Mucopolysaccharidosis (Hunter Syndrome)","volume":"21","author":"Serrano C. D.","year":"2011","journal-title":"Journal of Investigational Allergology & Clinical Immunology"},{"key":"e_1_2_5_8_1","doi-asserted-by":"publisher","DOI":"10.1186\/s13023\u2010024\u201003516\u2010z"},{"key":"e_1_2_5_9_1","doi-asserted-by":"publisher","DOI":"10.1186\/s13023\u2010022\u201002556\u20107"},{"key":"e_1_2_5_10_1","doi-asserted-by":"publisher","DOI":"10.1080\/21678707.2017.1296761"}],"container-title":["American Journal of Medical Genetics Part A"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/ajmg.a.64089","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,7,3]],"date-time":"2025-07-03T06:48:53Z","timestamp":1751525333000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1002\/ajmg.a.64089"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2025,4,12]]},"references-count":9,"journal-issue":{"issue":"8","published-print":{"date-parts":[[2025,8]]}},"alternative-id":["10.1002\/ajmg.a.64089"],"URL":"https:\/\/doi.org\/10.1002\/ajmg.a.64089","archive":["Portico"],"relation":{},"ISSN":["1552-4825","1552-4833"],"issn-type":[{"type":"print","value":"1552-4825"},{"type":"electronic","value":"1552-4833"}],"subject":[],"published":{"date-parts":[[2025,4,12]]},"assertion":[{"value":"2025-01-27","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2025-04-04","order":2,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2025-04-12","order":3,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}],"article-number":"e64089"}}