{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,29]],"date-time":"2026-05-29T18:58:58Z","timestamp":1780081138318,"version":"3.54.0"},"reference-count":8,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[2020,10,8]],"date-time":"2020-10-08T00:00:00Z","timestamp":1602115200000},"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":["Pediatrics International"],"published-print":{"date-parts":[[2021,2]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>A definitive diagnosis of congenital central hypoventilation syndrome (CCHS) is made by genetic testing. However, there are only a few examinations that warrant genetic testing. Electrical activity of the diaphragm (Edi) reflects neural respiratory drive from respiratory center to diaphragm. We evaluated the function of the respiratory center in CCHS by Edi monitoring.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Monitoring of Edi was performed in six CCHS cases without mechanical ventilation. The monitoring time was 30 consecutive minutes from wakefulness to sleep. The TcPCO<jats:sub>2<\/jats:sub> or EtCO<jats:sub>2<\/jats:sub> and SpO<jats:sub>2<\/jats:sub> were recorded simultaneously.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>The Edi peak during wakefulness was 14.0 (10.3\u201321.0) \u00b5V and the Edi peak during sleep was 6.7 (3.8\u20138.0) \u00b5V. The Edi peak during sleep was significantly lower than the Edi peak during wakefulness, and patients were in a state of hypoventilation. Although TcPCO<jats:sub>2<\/jats:sub> or EtCO<jats:sub>2<\/jats:sub> increased due to hypoventilation, an increase in the Edi peak that reflects central respiratory drive was not observed. \u0394Edi\/\u0394CO<jats:sub>2<\/jats:sub> was \u22120.06\u03bcV\/mmHg. Maximum EtCO<jats:sub>2<\/jats:sub> or TcPco<jats:sub>2<\/jats:sub> was 51\u00a0mmHg, and the average SpO<jats:sub>2<\/jats:sub> was 91.5% during monitoring.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>We confirmed that Edi monitoring could evaluate the function of the respiratory center and reproduce the hypoventilation of CCHS. The present study suggested that Edi monitoring is a useful examination in deciding whether to perform genetic testing or not and it may lead to an early diagnosis of CCHS.<\/jats:p><\/jats:sec>","DOI":"10.1111\/ped.14359","type":"journal-article","created":{"date-parts":[[2020,6,23]],"date-time":"2020-06-23T17:14:26Z","timestamp":1592932466000},"page":"168-171","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":4,"title":["Evaluation of respiratory center function in congenital central hypoventilation syndrome by monitoring electrical activity of the diaphragm"],"prefix":"10.1111","volume":"63","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-2732-5695","authenticated-orcid":false,"given":"Yosuke","family":"Yamada","sequence":"first","affiliation":[{"name":"Department of Neonatology Tokyo Women's Medical University Medical Center East Tokyo Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hisaya","family":"Hasegawa","sequence":"additional","affiliation":[{"name":"Department of Neonatology Tokyo Women's Medical University Medical Center East Tokyo Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nobuhide","family":"Henmi","sequence":"additional","affiliation":[{"name":"Department of Neonatology Tokyo Women's Medical University Medical Center East Tokyo Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shio","family":"Tsuruta","sequence":"additional","affiliation":[{"name":"Department of Neonatology Tokyo Women's Medical University Medical Center East Tokyo Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Masanori","family":"Wasa","sequence":"additional","affiliation":[{"name":"Department of Neonatology Tokyo Women's Medical University Medical Center East Tokyo Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hirotaka","family":"Kihara","sequence":"additional","affiliation":[{"name":"Department of Neonatology Tokyo Women's Medical University Medical Center East Tokyo Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Takayuki","family":"Kodera","sequence":"additional","affiliation":[{"name":"Department of Neonatology Tokyo Women's Medical University Medical Center East Tokyo Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Toshinari","family":"Kouyama","sequence":"additional","affiliation":[{"name":"Department of Neonatology Tokyo Women's Medical University Medical Center East Tokyo Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kensuke","family":"Kumazawa","sequence":"additional","affiliation":[{"name":"Department of Neonatology Tokyo Women's Medical University Medical Center East Tokyo Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2020,10,8]]},"reference":[{"key":"e_1_2_8_2_1","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.200807-1069ST"},{"key":"e_1_2_8_3_1","doi-asserted-by":"publisher","DOI":"10.1038\/jhg.2015.65"},{"key":"e_1_2_8_4_1","first-page":"874","article-title":"Neurally\u2010adjusted ventilatory assist (NAVA) in children: A systematic review","volume":"82","author":"Beck J","year":"2016","journal-title":"Minerva Anestesiol."},{"key":"e_1_2_8_5_1","doi-asserted-by":"publisher","DOI":"10.1038\/jp.2013.89"},{"key":"e_1_2_8_6_1","doi-asserted-by":"publisher","DOI":"10.5005\/jp-journals-10071-23286"},{"key":"e_1_2_8_7_1","doi-asserted-by":"crossref","first-page":"1483","DOI":"10.4187\/respcare.01650","article-title":"Electrical activity of the diaphragm in a small cohort of term neonates","volume":"57","author":"Howard M","year":"2012","journal-title":"Resp. Care."},{"key":"e_1_2_8_8_1","first-page":"493","article-title":"Assessment of neural respiratory drive in humans","volume":"36","author":"Lio ZD","year":"2013","journal-title":"Zhonghua Jie He He Hu Xi Za Zhi."},{"key":"e_1_2_8_9_1","doi-asserted-by":"publisher","DOI":"10.1378\/chest.14-0970"}],"container-title":["Pediatrics International"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1111%2Fped.14359","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/ped.14359","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/full-xml\/10.1111\/ped.14359","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/ped.14359","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,9,2]],"date-time":"2023-09-02T08:38:23Z","timestamp":1693643903000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/ped.14359"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2020,10,8]]},"references-count":8,"journal-issue":{"issue":"2","published-print":{"date-parts":[[2021,2]]}},"alternative-id":["10.1111\/ped.14359"],"URL":"https:\/\/doi.org\/10.1111\/ped.14359","archive":["Portico"],"relation":{},"ISSN":["1328-8067","1442-200X"],"issn-type":[{"value":"1328-8067","type":"print"},{"value":"1442-200X","type":"electronic"}],"subject":[],"published":{"date-parts":[[2020,10,8]]},"assertion":[{"value":"2020-05-06","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2020-06-17","order":1,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2020-10-08","order":2,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}