{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,3]],"date-time":"2026-07-03T08:01:11Z","timestamp":1783065671375,"version":"3.54.6"},"reference-count":7,"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":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Introduction<\/jats:title><jats:p>Congenital central hypoventilation syndrome (<jats:styled-content style=\"fixed-case\">CCHS<\/jats:styled-content>) is a rare disorder characterized by alveolar hypoventilation and autonomic system dysregulation secondary to mutations of the <jats:italic><jats:styled-content style=\"fixed-case\">PHOX<\/jats:styled-content>2B<\/jats:italic> gene. Treatment consists of assisted ventilation using positive\u2010pressure ventilators via tracheostomy, bi\u2010level positive airway pressure (<jats:styled-content style=\"fixed-case\">BPAP<\/jats:styled-content>) via a noninvasive interface, negative\u2010pressure ventilators, or diaphragm pacing. The long\u2010term use of <jats:styled-content style=\"fixed-case\">BPAP<\/jats:styled-content> in younger children at home has been less frequently reported.<\/jats:p><\/jats:sec><jats:sec><jats:title>Case presentation<\/jats:title><jats:p>We present a case of a 2\u2010month\u2010old infant with <jats:styled-content style=\"fixed-case\">CCHS<\/jats:styled-content> who was successfully managed by <jats:styled-content style=\"fixed-case\">BPAP<\/jats:styled-content> without the need for tracheostomy and followed up for 7 years.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p><jats:styled-content style=\"fixed-case\">CCHS<\/jats:styled-content> is a rare disease that manifests as nocturnal desaturation and carbon dioxide retention in early life. Noninvasive ventilation can be successfully used in young infants via an appropriate mask.<\/jats:p><\/jats:sec>","DOI":"10.1002\/ped4.12167","type":"journal-article","created":{"date-parts":[[2019,12,21]],"date-time":"2019-12-21T18:38:26Z","timestamp":1576953506000},"page":"261-264","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":5,"title":["Noninvasive ventilation in a young infant with congenital central hypoventilation and 7\u2010year follow\u2010up"],"prefix":"10.1002","volume":"3","author":[{"given":"Zhifei","family":"Xu","sequence":"first","affiliation":[{"name":"Department of Respiratory Medicine Beijing Children's Hospital Capital Medical University National Center for Children's Health Beijing China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yunxiao","family":"Wu","sequence":"additional","affiliation":[{"name":"Beijing Key Laboratory of Pediatric Otolaryngology, Head and Neck Surgery Beijing Pediatric Research Institute Beijing Children's Hospital Capital Medical University National Center for Children's Health Beijing China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Bei","family":"Li","sequence":"additional","affiliation":[{"name":"Department of Otolaryngology, Head and Neck Surgery Beijing Children's Hospital Capital Medical University National Center for Children's Health Beijing China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Li","family":"Zheng","sequence":"additional","affiliation":[{"name":"Department of Otolaryngology, Head and Neck Surgery Beijing Children's Hospital Capital Medical University National Center for Children's Health Beijing China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jingyuan","family":"Liu","sequence":"additional","affiliation":[{"name":"Department of Pediatric Critical Care Medicine Beijing Children's Hospital Capital Medical University National Center for Children's Health Beijing China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kunling","family":"Shen","sequence":"additional","affiliation":[{"name":"Department of Respiratory Medicine Beijing Children's Hospital Capital Medical University National Center for Children's Health Beijing China"}],"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":"10.1016\/j.prrv.2010.10.001"},{"key":"e_1_2_5_3_1","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.200807-1069ST"},{"key":"e_1_2_5_4_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1651-2227.2003.tb02540.x"},{"key":"e_1_2_5_5_1","doi-asserted-by":"publisher","DOI":"10.1002\/ppul.10392"},{"key":"e_1_2_5_6_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.autneu.2017.11.003"},{"key":"e_1_2_5_7_1","doi-asserted-by":"publisher","DOI":"10.2147\/TACG.S140629"},{"key":"e_1_2_5_8_1","doi-asserted-by":"publisher","DOI":"10.1002\/rcr2.448"}],"container-title":["Pediatric Investigation"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1002%2Fped4.12167","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/ped4.12167","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/full-xml\/10.1002\/ped4.12167","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/ped4.12167","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,9,8]],"date-time":"2023-09-08T16:08:34Z","timestamp":1694189314000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1002\/ped4.12167"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2019,12]]},"references-count":7,"journal-issue":{"issue":"4","published-print":{"date-parts":[[2019,12]]}},"alternative-id":["10.1002\/ped4.12167"],"URL":"https:\/\/doi.org\/10.1002\/ped4.12167","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":"2019-08-13","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"}}]}}