{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,3]],"date-time":"2026-07-03T08:01:10Z","timestamp":1783065670065,"version":"3.54.6"},"reference-count":10,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2019,12,21]],"date-time":"2019-12-21T00:00:00Z","timestamp":1576886400000},"content-version":"vor","delay-in-days":20,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Pediatric Investigation"],"published-print":{"date-parts":[[2019,12]]},"abstract":"Abstract<\/jats:title>Introduction<\/jats:title>Rapid\u2010onset obesity with hypothalamic dysfunction, hypoventilation, and autonomic dysregulation (ROHHAD<\/jats:styled-content>) syndrome is an exceptionally rare clinical entity with significant morbidity and high mortality with challenging\u2010to\u2010treat hypoventilation.<\/jats:p><\/jats:sec>Case presentation<\/jats:title>An 11\u2010year\u2010old morbidly obese Chinese female presented with a putative diagnosis of ROHHAD<\/jats:styled-content> associated with a left psoas ganglioneuroma. Initial polysomnography showed severe obstructive sleep apnea and hypoventilation. She was not adherent to prescribed non\u2010invasive positive pressure ventilation (NIPPV<\/jats:styled-content>). Echocardiography demonstrated evidence of pulmonary hypertension, likely secondary to chronic hypoventilation. With behavioral modification and trial of average volume\u2010assured pressure support (AVAPS<\/jats:styled-content>), adherence improved with eventual improvement of her pulmonary hypertension.<\/jats:p><\/jats:sec>Conclusion<\/jats:title>AVAPS<\/jats:styled-content> may improve ventilation and NIPPV<\/jats:styled-content> adherence in central hypoventilation disorders such as ROHHAD<\/jats:styled-content>, reducing risk of morbidity and mortality.<\/jats:p><\/jats:sec>","DOI":"10.1002\/ped4.12168","type":"journal-article","created":{"date-parts":[[2019,12,21]],"date-time":"2019-12-21T18:38:26Z","timestamp":1576953506000},"page":"253-256","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":12,"title":["Pulmonary hypertension and chronic hypoventilation in ROHHAD<\/scp> syndrome treated with average\u2010volume assured pressure support"],"prefix":"10.1002","volume":"3","author":[{"given":"Robert C.","family":"Stowe","sequence":"first","affiliation":[{"name":"Division of Neurology Children's Hospital of Philadelphia Philadelphia PA USA"},{"name":"Children's Hospital of Philadelphia Sleep Center Philadelphia PA USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Olufunke","family":"Afolabi\u2010Brown","sequence":"additional","affiliation":[{"name":"Children's Hospital of Philadelphia Sleep Center Philadelphia PA USA"},{"name":"Division of Pulmonary and Sleep Medicine Children's Hospital of Philadelphia Philadelphia PA USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2019,12,21]]},"reference":[{"key":"e_1_2_5_2_1","doi-asserted-by":"publisher","DOI":"10.1542\/peds.2006-3324"},{"key":"e_1_2_5_3_1","doi-asserted-by":"publisher","DOI":"10.1002\/ppul.23164"},{"key":"e_1_2_5_4_1","doi-asserted-by":"publisher","DOI":"10.1186\/s13023-015-0314-x"},{"key":"e_1_2_5_5_1","doi-asserted-by":"publisher","DOI":"10.1155\/2018\/1250721"},{"key":"e_1_2_5_6_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jsmc.2013.10.005"},{"key":"e_1_2_5_7_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.spen.2017.08.011"},{"key":"e_1_2_5_8_1","doi-asserted-by":"publisher","DOI":"10.1542\/peds.2015-1080"},{"key":"e_1_2_5_9_1","first-page":"1257","article-title":"Ventilatory responses to hypercapnia during wakefulness and sleep in obese adolescents with and without obstructive sleep apnea syndrome","volume":"35","author":"Yuan H","year":"2012","journal-title":"Sleep"},{"key":"e_1_2_5_10_1","doi-asserted-by":"publisher","DOI":"10.5664\/jcsm.27997"},{"key":"e_1_2_5_11_1","doi-asserted-by":"publisher","DOI":"10.1378\/chest.117.3.870"}],"container-title":["Pediatric Investigation"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1002%2Fped4.12168","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/ped4.12168","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/full-xml\/10.1002\/ped4.12168","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/ped4.12168","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,9,8]],"date-time":"2023-09-08T16:08:37Z","timestamp":1694189317000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1002\/ped4.12168"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2019,12]]},"references-count":10,"journal-issue":{"issue":"4","published-print":{"date-parts":[[2019,12]]}},"alternative-id":["10.1002\/ped4.12168"],"URL":"https:\/\/doi.org\/10.1002\/ped4.12168","archive":["Portico"],"relation":{},"ISSN":["2574-2272","2574-2272"],"issn-type":[{"value":"2574-2272","type":"print"},{"value":"2574-2272","type":"electronic"}],"subject":[],"published":{"date-parts":[[2019,12]]},"assertion":[{"value":"2019-09-16","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2019-10-20","order":1,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2019-12-21","order":2,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}