{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2023,10,3]],"date-time":"2023-10-03T07:45:28Z","timestamp":1696319128430},"reference-count":27,"publisher":"Wiley","issue":"5","license":[{"start":{"date-parts":[[2016,3,9]],"date-time":"2016-03-09T00:00:00Z","timestamp":1457481600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"DOI":"10.13039\/100007343","name":"Shire","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100007343","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Pediatric Anesthesia"],"published-print":{"date-parts":[[2016,5]]},"abstract":"<jats:title>Summary<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>Patients with mucopolysaccharidoses (<jats:styled-content style=\"fixed-case\">MPS<\/jats:styled-content>) are generally considered high risk for anesthesia care, owing to disease\u2010related factors. Sanfilippo syndrome type A (<jats:styled-content style=\"fixed-case\">MPS IIIA<\/jats:styled-content>) is the most frequently occurring <jats:styled-content style=\"fixed-case\">MPS<\/jats:styled-content>. Anesthesia\u2010specific information for <jats:styled-content style=\"fixed-case\">MPS IIIA<\/jats:styled-content> is not readily available in the literature.<\/jats:p><\/jats:sec><jats:sec><jats:title>Objectives<\/jats:title><jats:p>To report <jats:italic>post hoc<\/jats:italic> analyses on anesthesia care and outcomes from a 2\u2010year study of the natural history of patients with untreated <jats:styled-content style=\"fixed-case\">MPS IIIA<\/jats:styled-content> (<jats:styled-content style=\"fixed-case\">NCT<\/jats:styled-content>01047306).<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Subjects were \u22651 year of age, developmental age \u22651 year, and without significant central nervous system impairment (other than that due to <jats:styled-content style=\"fixed-case\">MPS IIIA<\/jats:styled-content>) or issues that would preclude study procedures. Procedures requiring general anesthesia included brain\/abdominal magnetic resonance imaging, lumbar puncture, and echocardiography. Sedation, intubation, and extubation procedures as well as postoperative airway problems were recorded at baseline and 6, 12, and 24 months of age.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Twenty\u2010five patients (baseline age, 13\u2013220 months) received a total of 94 general anesthetics. Patients successfully received oral sedation prior to 76 of 94 anesthetics. No patients required airway intervention or oxygen supplementation during sedation. All anesthesia providers described facemask ventilation and endotracheal intubations as \u2018easy\u2019. All subjects were successfully extubated after completion of the procedures. No patients required reintubation. Six (24%) patients had episodes of postoperative airway problems: wheezing (7\/94, 7.4%), croup (6\/94, 6.4%), and laryngospasm (2\/94, 2.1%).<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>We found no change in the modified Cormack\u2013Lehane intubation grades in 25 Sanfilippo syndrome type A children over the 2\u2010year study period.<\/jats:p><\/jats:sec>","DOI":"10.1111\/pan.12876","type":"journal-article","created":{"date-parts":[[2016,3,9]],"date-time":"2016-03-09T07:20:49Z","timestamp":1457508049000},"page":"531-538","source":"Crossref","is-referenced-by-count":7,"title":["Anesthetic care and perioperative complications in children with Sanfilipo Syndrome Type A"],"prefix":"10.1111","volume":"26","author":[{"given":"Elif C.","family":"Cingi","sequence":"first","affiliation":[{"name":"Department of Anesthesiology University of Minnesota Minneapolis MN USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"David 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