{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,8]],"date-time":"2026-05-08T00:35:45Z","timestamp":1778200545944,"version":"3.51.4"},"reference-count":24,"publisher":"Wiley","issue":"8","license":[{"start":{"date-parts":[[2024,5,31]],"date-time":"2024-05-31T00:00:00Z","timestamp":1717113600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"funder":[{"DOI":"10.13039\/100013669","name":"Ionis Pharmaceuticals","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100013669","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Pediatric Anesthesia"],"published-print":{"date-parts":[[2024,8]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>Alexander disease is a rare, progressive leukodystrophy, which predisposes patients to complications under general anesthesia due to clinical manifestations including developmental delay, seizures, dysphagia, vomiting, and sleep apnea. However, study of anesthetic outcomes is limited.<\/jats:p><\/jats:sec><jats:sec><jats:title>Aims<\/jats:title><jats:p>Our aim was to describe patient characteristics, anesthetic techniques, and anesthesia\u2010related complications for Alexander disease patients undergoing magnetic resonance imaging and\/or lumbar puncture at a quaternary\u2010care children's hospital.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>We performed a retrospective review of anesthetic outcomes in patients with Alexander disease enrolled in a prospective observational study. Included patients had diagnosed Alexander disease and underwent magnetic resonance imaging and\/or lumbar puncture at our institution. We excluded anesthetics for other procedures or at outside institutions. Collected data included patient characteristics, anesthetic techniques, medications, and complications under anesthesia and in the subsequent 24\u2009h. We performed descriptive statistics as appropriate.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Forty patients undergoing 64 procedures met inclusion criteria. Fifty\u2010six procedures (87.5%) required general anesthesia or monitored anesthesia care (MAC) and eight (12.5%) did not. The general anesthesia\/MAC group tended to be younger than nonanesthetized patients (median age 6\u2009years [IQR 3.8; 9] vs. 14.5\u2009years [IQR 12.8; 17.5]). In both groups, dysphagia (78.6% vs. 87.5%, respectively), seizures (62.5% vs. 25%), and recurrent vomiting (17.9% vs. 25%) were frequently reported preprocedure symptoms. Inhalational induction was common (<jats:italic>N<\/jats:italic>\u2009=\u200948; 85.7%), and two (3.6%) underwent rapid sequence induction. Serious complications were rare, with no aspiration or seizures. Hypotension resolving with ephedrine occurred in eight cases (14.3%). One patient each (1.8%) experienced postprocedure emergence agitation or vomiting. Fifty\u2010three (94.6%) were ambulatory procedures. No inpatients required escalation in acuity of care.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>In this single\u2010center study, patients with Alexander disease did not experience frequent or irreversible complications while undergoing general anesthesia\/MAC. Co\u2010morbid symptoms were not increased postanesthesia. Some patients may not require anesthesia to complete short procedures.<\/jats:p><\/jats:sec>","DOI":"10.1111\/pan.14937","type":"journal-article","created":{"date-parts":[[2024,5,31]],"date-time":"2024-05-31T10:42:30Z","timestamp":1717152150000},"page":"810-817","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":3,"title":["A retrospective observational cohort study of the anesthetic management and outcomes of pediatric patients with Alexander disease undergoing lumbar puncture or magnetic resonance imaging"],"prefix":"10.1111","volume":"34","author":[{"ORCID":"https:\/\/orcid.org\/0009-0008-9857-3048","authenticated-orcid":false,"given":"Jessica A.","family":"Berger","sequence":"first","affiliation":[{"name":"Department of Anesthesiology and Critical Care University of Pennsylvania Perelman School of Medicine  Philadelphia Pennsylvania USA"},{"name":"Department of Anesthesiology and Critical Care Medicine Children's Hospital of Philadelphia  Philadelphia Pennsylvania USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8871-2579","authenticated-orcid":false,"given":"Allan F.","family":"Simpao","sequence":"additional","affiliation":[{"name":"Department of Anesthesiology and Critical Care University of Pennsylvania Perelman School of Medicine  Philadelphia Pennsylvania USA"},{"name":"Department of Anesthesiology and Critical Care Medicine Children's Hospital of Philadelphia  Philadelphia Pennsylvania USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Scott R.","family":"Dubow","sequence":"additional","affiliation":[{"name":"Department of Anesthesiology and Critical Care University of Pennsylvania Perelman School of Medicine  Philadelphia Pennsylvania USA"},{"name":"Department of Anesthesiology and Critical Care Medicine Children's Hospital of Philadelphia  Philadelphia Pennsylvania USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Heather A.","family":"McClung","sequence":"additional","affiliation":[{"name":"Department of Anesthesiology and Critical Care University of Pennsylvania Perelman School of Medicine  Philadelphia Pennsylvania USA"},{"name":"Department of Anesthesiology and Critical Care Medicine Children's Hospital of Philadelphia  Philadelphia Pennsylvania USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Geraldine W.","family":"Liu","sequence":"additional","affiliation":[{"name":"Division of Neurology Children's Hospital of Philadelphia  Philadelphia Pennsylvania USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1054-0815","authenticated-orcid":false,"given":"Amy T.","family":"Waldman","sequence":"additional","affiliation":[{"name":"Division of Neurology Children's Hospital of Philadelphia  Philadelphia Pennsylvania USA"},{"name":"Department of Neurology University of Pennsylvania Perelman School of Medicine  Philadelphia Pennsylvania USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1943-3060","authenticated-orcid":false,"given":"Elizabeth T.","family":"Drum","sequence":"additional","affiliation":[{"name":"Department of Anesthesiology and Critical Care University of Pennsylvania Perelman School of Medicine  Philadelphia Pennsylvania USA"},{"name":"Department of Anesthesiology and Critical Care Medicine Children's Hospital of Philadelphia  Philadelphia Pennsylvania USA"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2024,5,31]]},"reference":[{"key":"e_1_2_10_2_1","doi-asserted-by":"publisher","DOI":"10.1177\/08830738030180090901"},{"key":"e_1_2_10_3_1","first-page":"693","volume-title":"Handbook of Clinical Neurology","author":"Messing A","year":"2018"},{"key":"e_1_2_10_4_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.braindev.2009.03.006"},{"key":"e_1_2_10_5_1","volume-title":"GeneReviews(\u00ae)","author":"Srivastava S","year":"2002"},{"key":"e_1_2_10_6_1","doi-asserted-by":"publisher","DOI":"10.1038\/83679"},{"key":"e_1_2_10_7_1","doi-asserted-by":"publisher","DOI":"10.1016\/s0736-5748(02)00019-9"},{"key":"e_1_2_10_8_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00415-011-6056-3"},{"key":"e_1_2_10_9_1","doi-asserted-by":"publisher","DOI":"10.1177\/08830738231210040"},{"key":"e_1_2_10_10_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.conb.2021.10.002"},{"key":"e_1_2_10_11_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.nurt.2010.05.013"},{"key":"e_1_2_10_12_1","doi-asserted-by":"publisher","DOI":"10.4103\/1673-5374.369097"},{"key":"e_1_2_10_13_1","unstructured":"Ionis Pharmaceuticals Inc.A study to evaluate the safety and efficacy of ION373 in patients with Alexander disease (AxD). 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