{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,16]],"date-time":"2026-09-16T18:49:18Z","timestamp":1789584558155,"version":"build-2803163510"},"reference-count":5,"publisher":"Wiley","issue":"6","license":[{"start":{"date-parts":[[2019,6,22]],"date-time":"2019-06-22T00:00:00Z","timestamp":1561161600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Respirology Case Reports"],"published-print":{"date-parts":[[2019,8]]},"abstract":"<jats:p>Congenital central hypoventilation syndrome (CCHS) is a rare disorder characterized by alveolar hypoventilation and autonomic dysregulation secondary to mutations of the <jats:italic>PHOX 2B<\/jats:italic> genes. Treatment consists of assisted ventilation using positive pressure ventilators via tracheostomy, bi\u2010level positive airway pressure (BPAP), negative pressure ventilators, or diaphragm pacing. Previous case reports have highlighted early use of nasal non\u2010invasive BPAP use in infants with CCHS. We present a case of a 10\u2010month\u2010old infant who was successfully managed on a new feature of non\u2010invasive ventilation called average volume assured pressure support (AVAPS) without the need for tracheostomy. The AVAPS feature enables the machine to automatically adjust the inspiratory pressures to deliver a constant targeted tidal volume. This feature enabled a better control of ventilation as indicated by a more stable transcutaneous carbon dioxide profile compared to conventional nasal non\u2010invasive BPAP, making non\u2010invasive ventilation a more accessible method of managing sleep hypoventilation in CCHS.<\/jats:p>","DOI":"10.1002\/rcr2.448","type":"journal-article","created":{"date-parts":[[2019,6,22]],"date-time":"2019-06-22T18:38:51Z","timestamp":1561228731000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":19,"title":["Nasal mask average volume\u2010assured pressure support in an infant with congenital central hypoventilation syndrome"],"prefix":"10.1002","volume":"7","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-8001-524X","authenticated-orcid":false,"given":"Vishal","family":"Saddi","sequence":"first","affiliation":[{"name":"Department of Sleep Medicine Sydney Children's Hospital  Sydney New South Wales Australia"},{"name":"School of Women 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Medicine Sydney Children's Hospital  Sydney New South Wales Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sonia","family":"Pithers","sequence":"additional","affiliation":[{"name":"Department of Sleep Medicine Sydney Children's Hospital  Sydney New South Wales Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Colin","family":"Sullivan","sequence":"additional","affiliation":[{"name":"Department of Sleep Medicine Sydney Children's Hospital  Sydney New South Wales Australia"},{"name":"School of Women and Children's Health, Faculty of Medicine University of New South Wales  Sydney New South Wales Australia"},{"name":"David Read Laboratory, Discipline of Sleep Medicine University of Sydney  Sydney New South Wales 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