{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2023,10,6]],"date-time":"2023-10-06T06:12:23Z","timestamp":1696572743651},"reference-count":5,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2014,8,18]],"date-time":"2014-08-18T00:00:00Z","timestamp":1408320000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/3.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Respirology Case Reports"],"published-print":{"date-parts":[[2014,9]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Our patient with <jats:styled-content style=\"fixed-case\">P<\/jats:styled-content>rader\u2010<jats:styled-content style=\"fixed-case\">W<\/jats:styled-content>illi syndrome (<jats:styled-content style=\"fixed-case\">PWS<\/jats:styled-content>) not only displayed many typical syndromic features but also presented several unique challenges, with gross velopharyngeal insufficiency necessitating repair and severe obstructive sleep apnea developing thereafter, requiring ongoing non\u2010invasive ventilation. This coincided with development of a narcolepsy\u2010like syndrome, treated with dexamphetamine. Cataplexy, hypnogogic\/hypnopompic hallucinations, sleep paralysis were absent and <jats:styled-content style=\"fixed-case\">HLA<\/jats:styled-content>\u2010<jats:styled-content style=\"fixed-case\">DQB1<\/jats:styled-content>*06:02 was negative. Growth hormone (<jats:styled-content style=\"fixed-case\">GH<\/jats:styled-content>) therapy was commenced at 8 months of age and, as recommended, regular polysomnograms were conducted. Adenotonsillar growth on <jats:styled-content style=\"fixed-case\">GH<\/jats:styled-content> therapy is reported as well as several reports of sudden death in <jats:styled-content style=\"fixed-case\">PWS<\/jats:styled-content> patients on <jats:styled-content style=\"fixed-case\">GH<\/jats:styled-content>. Despite <jats:styled-content style=\"fixed-case\">GH<\/jats:styled-content>, lifestyle measures with regular dietician review, and an exercise program, there was progressive excessive weight gain. Our patient also developed moderate tonsil hypertrophy. To our knowledge, this is the first case report of severe obstructive sleep apnea secondary to sphincter pharyngoplasty coinciding with rapidly evolving narcolepsy\u2010like syndrome.<\/jats:p>","DOI":"10.1002\/rcr2.65","type":"journal-article","created":{"date-parts":[[2014,8,18]],"date-time":"2014-08-18T06:43:05Z","timestamp":1408344185000},"page":"111-112","source":"Crossref","is-referenced-by-count":1,"title":["Rapidly evolving narcolepsy\u2010like syndrome coinciding with severe <scp>OSA<\/scp> following pharyngoplasty in <scp>P<\/scp>rader\u2010<scp>W<\/scp>illi syndrome"],"prefix":"10.1002","volume":"2","author":[{"given":"Gregory","family":"Blecher","sequence":"first","affiliation":[{"name":"Department of Sleep Medicine Sydney Children's Hospital  Randwick New South Wales Australia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Natalie","family":"Wainbergas","sequence":"additional","affiliation":[{"name":"Department of Sleep Medicine Sydney Children's Hospital  Randwick New South Wales Australia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Michael","family":"McGlynn","sequence":"additional","affiliation":[{"name":"Department of Sleep Medicine Sydney Children's Hospital  Randwick New South Wales Australia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Arthur","family":"Teng","sequence":"additional","affiliation":[{"name":"Department of Sleep Medicine Sydney Children's Hospital  Randwick New South Wales Australia"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2014,8,18]]},"reference":[{"key":"e_1_2_4_2_1","doi-asserted-by":"publisher","DOI":"10.1002\/ppul.10152"},{"key":"e_1_2_4_3_1","doi-asserted-by":"publisher","DOI":"10.1097\/SCS.0b013e31825b3ba9"},{"key":"e_1_2_4_4_1","doi-asserted-by":"publisher","DOI":"10.1002\/ajmg.a.30777"},{"key":"e_1_2_4_5_1","doi-asserted-by":"publisher","DOI":"10.1164\/ajrccm.162.2.9908058"},{"key":"e_1_2_4_6_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1468-1331.2004.00969.x"}],"container-title":["Respirology Case Reports"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1002%2Frcr2.65","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/rcr2.65","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/full-xml\/10.1002\/rcr2.65","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/rcr2.65","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,10,5]],"date-time":"2023-10-05T08:11:06Z","timestamp":1696493466000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1002\/rcr2.65"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2014,8,18]]},"references-count":5,"journal-issue":{"issue":"3","published-print":{"date-parts":[[2014,9]]}},"alternative-id":["10.1002\/rcr2.65"],"URL":"https:\/\/doi.org\/10.1002\/rcr2.65","archive":["Portico"],"relation":{},"ISSN":["2051-3380","2051-3380"],"issn-type":[{"value":"2051-3380","type":"print"},{"value":"2051-3380","type":"electronic"}],"subject":[],"published":{"date-parts":[[2014,8,18]]}}}