{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,16]],"date-time":"2026-10-16T06:40:31Z","timestamp":1792132831930,"version":"4.4.0"},"reference-count":31,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2017,2,27]],"date-time":"2017-02-27T00:00:00Z","timestamp":1488153600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["American J of Med Genetics Pt A"],"published-print":{"date-parts":[[2017,4]]},"abstract":"<jats:sec><jats:label\/><jats:p>Sleep\u2010disordered breathing is a common feature in children with achondroplasia. The aim of our study was to review the poly(somno)graphic (P(S)G) findings and consequent treatments in children with achondroplasia followed in the national reference center for skeletal dysplasia. A retrospective review of the clinical charts and P(S)G of 43 consecutive children (mean age 3.9\u2009\u00b1\u20093.5 years) with achondroplasia seen over a period of 2 years was performed. Twenty four (59%) children had obstructive sleep apnea (OSA). Thirteen children had an obstructive apnea\u2010hypopnea index (OAHI)\u2009&lt;\u20095\/hr, four had an OAHI between 5 and 10\/hr, and seven had an OAHI\u2009\u2265\u200910\/hr. Ten of the 15 children who had previous upper airway surgery still had an abnormal P(S)G. All the patients with an AHI\u2009\u2265\u200910\/hr were under 7 years of age and none had a prior tonsillectomy. The children who underwent adeno\u2010tonsillectomy, coupled in most cases with turbinectomy, were significantly older (mean age 7.5\u2009\u00b1\u20093.5 vs. 3.5\u2009\u00b1\u20091.7 years old, <jats:italic>P\u2009<\/jats:italic>=\u20090.015) and had significantly better P(S)G results than those who underwent only adeno\u2010turbinectomy. No correlation was observed between the mean AHI value at the baseline P(S)G and the type of academic course (standard, supported or specialized). In conclusion, OSA is common in children with achondroplasia. The observation of a reduced prevalence of OSA after (adeno\u2010)tonsillectomy is in favor of this type of surgery when possible.<\/jats:p><\/jats:sec>","DOI":"10.1002\/ajmg.a.38130","type":"journal-article","created":{"date-parts":[[2017,2,27]],"date-time":"2017-02-27T05:28:41Z","timestamp":1488173321000},"page":"868-878","source":"Crossref","is-referenced-by-count":72,"title":["Sleep\u2010disordered breathing and its management in children with achondroplasia"],"prefix":"10.1002","volume":"173","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-3137-5756","authenticated-orcid":false,"given":"Rossana","family":"Tenconi","sequence":"first","affiliation":[{"name":"Pediatric Highly Intensive Care Unit Department of Pathophysiology and Transplantation, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico Universit\u00e0 degli Studi di Milano Milan Italy"},{"name":"AP\u2010HP, H\u00f4pital Necker\u2010Enfants malades Pediatric Noninvasive Ventilation and Sleep Unit Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sonia","family":"Khirani","sequence":"additional","affiliation":[{"name":"AP\u2010HP, H\u00f4pital Necker\u2010Enfants malades Pediatric Noninvasive Ventilation and Sleep Unit Paris France"},{"name":"ASV Sant\u00e9 Gennevilliers France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alessandro","family":"Amaddeo","sequence":"additional","affiliation":[{"name":"AP\u2010HP, H\u00f4pital Necker\u2010Enfants malades Pediatric Noninvasive Ventilation and Sleep Unit Paris France"},{"name":"Paris Descartes University Paris France"},{"name":"Inserm U955 Equipe 13 Cr\u00e9teil France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Caroline","family":"Michot","sequence":"additional","affiliation":[{"name":"AP\u2010HP, H\u00f4pital Necker\u2010Enfants malades, Genetics Departement, Centre of Reference for Skeletal Dysplasia, INSERM UMR 1163, Institut Imagine University Paris Descartes\u2010Sorbonne Paris Cit\u00e9 Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Genevi\u00e8ve","family":"Baujat","sequence":"additional","affiliation":[{"name":"AP\u2010HP, H\u00f4pital Necker\u2010Enfants malades, Genetics Departement, Centre of Reference for Skeletal Dysplasia, INSERM UMR 1163, Institut Imagine University Paris Descartes\u2010Sorbonne Paris Cit\u00e9 Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Vincent","family":"Couloigner","sequence":"additional","affiliation":[{"name":"Head and Neck Surgery and Otorhinolaryngology Department, AP\u2010HP H\u00f4pital Necker\u2010Enfants malades Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Livio","family":"De Sanctis","sequence":"additional","affiliation":[{"name":"AP\u2010HP, H\u00f4pital Necker\u2010Enfants malades Pediatric Noninvasive Ventilation and Sleep Unit Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Syril","family":"James","sequence":"additional","affiliation":[{"name":"Pediatric Neurosurgery Department, AP\u2010HP H\u00f4pital Necker\u2010Enfants malades Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michel","family":"Zerah","sequence":"additional","affiliation":[{"name":"Paris Descartes University Paris France"},{"name":"Pediatric Neurosurgery Department, AP\u2010HP H\u00f4pital Necker\u2010Enfants malades Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Val\u00e9rie","family":"Cormier\u2010Daire","sequence":"additional","affiliation":[{"name":"AP\u2010HP, H\u00f4pital Necker\u2010Enfants malades, Genetics Departement, Centre of Reference for Skeletal Dysplasia, INSERM UMR 1163, Institut Imagine University Paris Descartes\u2010Sorbonne Paris Cit\u00e9 Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Brigitte","family":"Fauroux","sequence":"additional","affiliation":[{"name":"AP\u2010HP, H\u00f4pital Necker\u2010Enfants malades Pediatric Noninvasive Ventilation and Sleep Unit Paris France"},{"name":"Paris Descartes University Paris France"},{"name":"Inserm U955 Equipe 13 Cr\u00e9teil France"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2017,2,27]]},"reference":[{"key":"e_1_2_6_2_1","doi-asserted-by":"publisher","DOI":"10.1007\/s11325-010-0432-6"},{"key":"e_1_2_6_3_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.berh.2007.12.008"},{"key":"e_1_2_6_4_1","first-page":"368","article-title":"Achondroplasia is defined by recurrent G380R mutations of FGFR3","volume":"56","author":"Bellus G. 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