{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,1,23]],"date-time":"2026-01-23T20:35:10Z","timestamp":1769200510807,"version":"3.49.0"},"reference-count":23,"publisher":"Wiley","issue":"7","license":[{"start":{"date-parts":[[2024,4,17]],"date-time":"2024-04-17T00:00:00Z","timestamp":1713312000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Pediatric Pulmonology"],"published-print":{"date-parts":[[2024,7]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Study Objectives<\/jats:title><jats:p>Congenital central hypoventilation syndrome (CCHS) is a rare disease predisposing children to respiratory failure due to abnormal ventilatory drive. Variability in hypoventilation and respiratory support need have been reported. We aim to identify clinical variables associated with incident tracheostomy and common etiologies of hospitalization among children with CCHS.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Hospital discharge records were obtained for children (&lt;21 years) with CCHS hospitalized between 2006 and 2019 from the Kid's Inpatient Database. Primary diagnostic categories for hospitalizations with CCHS were summarized. Multivariable logistic regression models were used to explore risk factors associated with incident tracheostomy.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Among 2404 hospitalizations with CCHS, 133 (5.5%) had incident tracheostomy, 1230 (51.2%) had established tracheostomy, and 1041 (43.3%) had no tracheostomy. Compared with children without tracheostomy, those with incident tracheostomy were younger, had a history of prematurity, congenital heart disease, laryngeal, glottic, and subglottic stenosis (LGSS), congenital airway anomalies, neuromuscular weakness, gastroesophageal reflux disease. Children without tracheostomy had higher mortality than those with tracheostomy status (2.19% vs. 0.66%). Multivariable\u2010adjusted analyses showed that incident tracheostomy was associated with infancy (0\u20131 years), neuromuscular weakness, and congenital heart disease. Most common diagnostic categories include (1) diseases of the respiratory system (30.23%), (2) injury and poisoning (9.35%), and (3) diseases of the nervous system and sense organs (6.71%).<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>Children with CCHS who received incident tracheostomy are more likely to be younger and with LGSS, neuromuscular weakness and congenital heart disease. Clinicians should be aware of these risk factors representing more severe CCHS with earlier manifestation needing tracheostomy. Higher mortality among nontracheostomy group highlights the need for considering tracheostomy in caring for children with CCHS.<\/jats:p><\/jats:sec>","DOI":"10.1002\/ppul.27015","type":"journal-article","created":{"date-parts":[[2024,4,17]],"date-time":"2024-04-17T10:05:47Z","timestamp":1713348347000},"page":"1932-1943","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":3,"title":["Tracheostomy and inpatient outcomes among children with congenital central hypoventilation syndrome: A kids' inpatient database study"],"prefix":"10.1002","volume":"59","author":[{"given":"Po\u2010Yang","family":"Tsou","sequence":"first","affiliation":[{"name":"Boston Children's Hospital, Harvard Medical School Division of Pulmonary Medicine Boston Massachusetts USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1400-6852","authenticated-orcid":false,"given":"Ignacio E.","family":"Tapia","sequence":"additional","affiliation":[{"name":"Division of Pediatric Pulmonology University of Miami Health System Miami Florida USA"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2024,4,17]]},"reference":[{"key":"e_1_2_15_2_1","doi-asserted-by":"publisher","DOI":"10.1378\/chest.127.1.72"},{"key":"e_1_2_15_3_1","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.200807-1069ST"},{"key":"e_1_2_15_4_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00431-019-03568-5"},{"key":"e_1_2_15_5_1","volume-title":"Congenital Central Hypoventilation Syndrome","author":"Weese\u2010Mayer DE","year":"1993"},{"key":"e_1_2_15_6_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00431-020-03922-y"},{"key":"e_1_2_15_7_1","doi-asserted-by":"publisher","DOI":"10.1002\/ppul.10438"},{"key":"e_1_2_15_8_1","doi-asserted-by":"publisher","DOI":"10.5664\/jcsm.6706"},{"key":"e_1_2_15_9_1","doi-asserted-by":"publisher","DOI":"10.5664\/jcsm.9370"},{"key":"e_1_2_15_10_1","doi-asserted-by":"publisher","DOI":"10.1002\/ajmg.a.38726"},{"key":"e_1_2_15_11_1","volume-title":"Healthcare Cost and Utilization Project (HCUP). 2000, 2003, 2006, 2009, and 2012","author":"HCUP Kids' Inpatient Database (KID)","year":"2024"},{"key":"e_1_2_15_12_1","volume-title":"Healthcare Cost and Utilization Project Data Use Agreement Course","author":"Agency for Healthcare Research and Quality","year":"2024"},{"key":"e_1_2_15_13_1","doi-asserted-by":"publisher","DOI":"10.1001\/archotol.129.5.523"},{"key":"e_1_2_15_14_1","doi-asserted-by":"publisher","DOI":"10.1097\/PCC.0000000000003002"},{"key":"e_1_2_15_15_1","doi-asserted-by":"publisher","DOI":"10.1111\/jce.15347"},{"key":"e_1_2_15_16_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.sleep.2023.06.030"},{"key":"e_1_2_15_17_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jpedsurg.2020.01.038"},{"issue":"95","key":"e_1_2_15_18_1","first-page":"64","article-title":"Cancer registration: principles and methods. 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