{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,29]],"date-time":"2026-08-29T12:17:09Z","timestamp":1788005829168,"version":"build-2784847793"},"reference-count":47,"publisher":"Wiley","issue":"8","license":[{"start":{"date-parts":[[2021,5,7]],"date-time":"2021-05-07T00:00:00Z","timestamp":1620345600000},"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":[[2021,8]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>The aims of the study were to evaluate the prevalence of sleep\u2010disordered breathing (SDB) by using polysomnography (PSG) in children with MPS IVA and MPS VI who underwent enzyme replacement therapy (ERT) and to analyze the effect on SDB of having upper airway surgery, pulmonary functions, and exercise capacity. A retrospective cross\u2010sectional study was conducted on patients with MPS IVA (n:17) and MPS VI (n:11) aged under 19\u2009years who underwent polysomnography. Descriptive and nonparametric analyses were performed for demographic, PSG, pulmonary function and exercise capacity variables. The frequency of sleep apnea in the study sample was 85.7% (24\/28). Four patients (14.3%) had no sleep apnea, 15 (53.6%) had mild, and nine (32.1%) had moderate\u2010to\u2010severe sleep apnea. Two patients (7.1%) had central sleep apnea and 22 had obstructive sleep apnea (OSA) (78.6%). Forced expiratory volume in 1\u2009s (FEV1) and forced vital capacity (FVC) were negatively correlated to apnea\u2010hypopnea index (AHI) (<jats:italic>r<\/jats:italic>\u00a0=\u2009\u22120.594, <jats:italic>p<\/jats:italic>\u00a0=\u2009.009; <jats:italic>r<\/jats:italic>\u00a0=\u2009\u22120.636, <jats:italic>p<\/jats:italic>\u00a0=\u2009.005, respectively). Despite ERT and previous upper airway surgery, the prevalence of OSA was high in patients with MPS IVA\u2013MPS IV, emphasizing the importance of PSG screening for sleep disorders. Pulmonary function tests may be useful for predicting sleep apnea in patients with MPS IVA and MPS VI.<\/jats:p>","DOI":"10.1002\/ajmg.a.62229","type":"journal-article","created":{"date-parts":[[2021,5,8]],"date-time":"2021-05-08T18:46:04Z","timestamp":1620499564000},"page":"2306-2314","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":7,"title":["Evaluation of sleep\u2010disordered breathing and its relationship with respiratory parameters in children with mucopolysaccharidosis Type <scp>IVA<\/scp> and <scp>VI<\/scp>"],"prefix":"10.1002","volume":"185","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-0334-6336","authenticated-orcid":false,"given":"Dilber","family":"Ademhan Tural","sequence":"first","affiliation":[{"name":"Department of Pediatric Pulmonology School of Medicine, Hacettepe University, Ihsan Dogramaci Children's Hospital  Ankara 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