{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,29]],"date-time":"2026-08-29T12:17:07Z","timestamp":1788005827751,"version":"build-2784847793"},"reference-count":32,"publisher":"Wiley","issue":"12","license":[{"start":{"date-parts":[[2018,11,18]],"date-time":"2018-11-18T00:00:00Z","timestamp":1542499200000},"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":[[2018,12]]},"abstract":"<jats:p>Respiratory problems are common in Morquio\u2010A syndrome (MPS IVA) but objective data on sleep\u2010disordered breathing are scarce. The aim of our study was to review polygraphic (PG) findings and the need for noninvasive continuous positive airway pressure (CPAP) or noninvasive ventilation (NIV) in children with MPS IVA. A retrospective review of the clinical charts and PG of 16 consecutive children (7 boys, mean age 10.5\u2009\u00b1\u20094.2 years) with MPS IVA seen over a period of 3 years was performed. The prevalence of obstructive sleep apnea (OSA) was 69% with only five patients, all younger than 10 years old, having a normal PG. Four patients had mild OSA (apnea\u2010hypopnea index [AHI] \u22651.5 and &lt;5 events\/hr), three patients had moderate OSA (AHI \u22655 and &lt;10 events\/hr), and three patients had severe OSA (AHI\u2009\u2265\u200910 events\/hr). Among the 10 patients with OSA, 3 had prior adenoidectomy \u00b1 tonsillectomy and 6 were on enzyme replacement therapy. Only one patient had a central apnea index &gt;5 events\/hr despite prior cervico\u2010occipital decompression. Six patients, all older than 11\u2009years old, were started on CPAP or NIV because of severe OSA (<jats:italic>n<\/jats:italic>\u2009=\u20094), nocturnal hypoventilation (<jats:italic>n<\/jats:italic>\u2009=\u20091), or impossibility to be weaned from NIV after an acute respiratory failure (<jats:italic>n<\/jats:italic>\u2009=\u20091). Prevalence of OSA is high in patients with MPS IVA, underlying the importance of a systematic screening for sleep\u2010disordered breathing. CPAP and NIV are efficient and well accepted for treating sleep\u2010disordered breathing.<\/jats:p>","DOI":"10.1002\/ajmg.a.40642","type":"journal-article","created":{"date-parts":[[2018,11,19]],"date-time":"2018-11-19T05:20:13Z","timestamp":1542604813000},"page":"2595-2603","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":5,"title":["A retrospective study on sleep\u2010disordered breathing in Morquio\u2010A syndrome"],"prefix":"10.1002","volume":"176","author":[{"given":"Giulia","family":"Facchina","sequence":"first","affiliation":[{"name":"Pediatric Pulmonology Department University of Trieste  Trieste Italy"},{"name":"Pediatric Noninvasive Ventilation and Sleep Unit AP\u2010HP, H\u00f4pital Necker\u2010Enfants Malades  Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alessandro","family":"Amaddeo","sequence":"additional","affiliation":[{"name":"Pediatric Noninvasive Ventilation and Sleep Unit 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