{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,29]],"date-time":"2026-08-29T01:17:55Z","timestamp":1787966275939,"version":"build-2784847793"},"reference-count":93,"publisher":"Wiley","license":[{"start":{"date-parts":[[2026,8,28]],"date-time":"2026-08-28T00:00:00Z","timestamp":1787875200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"},{"start":{"date-parts":[[2026,8,28]],"date-time":"2026-08-28T00:00:00Z","timestamp":1787875200000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["The Laryngoscope"],"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Objective<\/jats:title>\n                    <jats:p>To synthesize the prevalence and subtype\u2010specific patterns of airway manifestations in mucopolysaccharidoses (MPS) and summarize related morbidity and mortality.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Data Sources<\/jats:title>\n                    <jats:p>PubMed, CINAHL, Ovid Embase, Ovid MEDLINE, and Ovid All EBM Reviews.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Review Methods<\/jats:title>\n                    <jats:p>PRISMA\u2010ScR guided scoping review of English and French\u2010language studies reporting airway manifestations in \u2265\u20095 patients up to June 2026. Pooled prevalences with 95% confidence intervals (CI) were estimated using proportional meta\u2010analysis.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>Of 2359 records, 65 case series (3715 participants; Level 4 evidence) were included. Upper\u2010airway soft tissue hypertrophy was common: adenoid hypertrophy ranged from 52.5% (MPS IV; 95% CI, 21.0\u201382.1) to 78.5% (MPS I; 95% CI, 23.9\u201397.7); tonsillar hypertrophy ranged from 38.5% (MPS I; 95% CI, 17.5\u201364.9) to 59.5% (MPS II; 95% CI, 34.6\u201380.4). Macroglossia was frequent, with the highest prevalence in MPS II (79.7%; 95% CI, 16.5\u201398.7). Laryngotracheal involvement varied, with tracheal narrowing reaching 92.3% (95% CI, 7.0\u201399.9) in MPS II and 85.4% (95% CI, 38.3\u201398.2) in MPS IV. Obstructive sleep apnea was highly prevalent across subtypes (MPS I, 61.7%; 95% CI, 45.3\u201375.9; MPS II, 75.3%; 95% CI, 43.1\u201392.4; MPS VI, 83.7%; 95% CI, 48.0\u201396.6). Tracheotomy was required in 7.0% (95% CI, 4.9\u20139.9), and respiratory\u2010related deaths occurred in 10.4% (95% CI, 5.2\u201319.9) of patients with available data.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>Airway disease in MPS is common, multilevel, and clinically consequential, with substantial burdens of OSA and progressive laryngotracheal pathology. Early, systematic, longitudinal airway assessment is essential to guide counseling and management across MPS subtypes.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1002\/lary.70868","type":"journal-article","created":{"date-parts":[[2026,8,29]],"date-time":"2026-08-29T01:13:47Z","timestamp":1787966027000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["The Burden of Airway Disease in Mucopolysaccharidoses: Evidence Across Subtypes"],"prefix":"10.1002","author":[{"ORCID":"https:\/\/orcid.org\/0009-0005-5519-7730","authenticated-orcid":false,"given":"Julia","family":"Edward","sequence":"first","affiliation":[{"name":"Faculty of Medicine Universit\u00e9 de Montr\u00e9al  Montreal Quebec Canada"},{"name":"Department of Pediatric Otolaryngology\u2014Head &amp; Neck Surgery CHU Sainte\u2010Justine  Montreal Quebec Canada"},{"name":"CHU Sainte\u2010Justine Research Institute CHU Sainte\u2010Justine  Montreal Quebec Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alix","family":"Pincivy","sequence":"additional","affiliation":[{"name":"Medical Library CHU Sainte Justine  Montreal Quebec Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8300-3220","authenticated-orcid":false,"given":"Mathieu","family":"Bergeron","sequence":"additional","affiliation":[{"name":"Faculty of Medicine Universit\u00e9 de Montr\u00e9al  Montreal Quebec Canada"},{"name":"Department of Pediatric Otolaryngology\u2014Head &amp; Neck Surgery CHU Sainte\u2010Justine  Montreal Quebec Canada"},{"name":"CHU Sainte\u2010Justine Research Institute CHU Sainte\u2010Justine  Montreal Quebec Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2026,8,28]]},"reference":[{"key":"e_1_2_10_2_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.pedneo.2022.10.001"},{"issue":"2","key":"e_1_2_10_3_1","doi-asserted-by":"crossref","first-page":"133","DOI":"10.1016\/j.prrv.2010.10.005","article-title":"Respiratory Manifestations in Mucopolysaccharidoses","volume":"12","author":"Muhlebach M. 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