和佐　正紀
   Department   School of Medicine(Tokyo Women's Medical University Adachi Medical Center), School of Medicine
   Position   Assistant Professor
Article types Original article
Language English
Peer review Peer reviewed
Title Evaluation of respiratory center function in congenital central hypoventilation syndrome by monitoring electrical activity of the diaphragm
Journal Formal name：Pediatrics international : official journal of the Japan Pediatric Society
Abbreviation：Pediatr Int
ISSN code：1442200X/13288067
Domestic / ForeginForegin
Volume, Issue, Page pp.10.1111/ped.14359
Author and coauthor Yamada Yosuke, Hasegawa Hisaya, Henmi Nobuhide, Tsuruta Shio, Wasa Masanori, Kihara Hirotaka, Kodera Takayuki, Kouyama Toshinari, Kumazawa Kensuke
Publication date 2020/06
Summary BACKGROUND:Definitive diagnosis of congenital central hypoventilation syndrome (CCHS) is made by genetic testing. However, there are only a few examinations that supports to perform genetic testing. Electrical activity of the diaphragm (Edi) is a myogenic potential of the diaphragm and it reflects central respiratory drive in the respiratory center. We evaluated the function of the respiratory center in CCHS by Edi monitoring.METHODS:Edi monitoring was performed in 6 CCHS cases without mechanical ventilation. The monitoring time was 30 consecutive minutes from wakefulness to sleep. The TcPCO2 or EtCO2 and SpO2 were recorded simultaneously.RESULTS:Edi peak during wakefulness was 14.0 (10.3-21.0) µV and Edi peak during sleep was 6.7 (3.8-8.0) µV. Edi peak during sleep was significantly lower from Edi peak during wakefulness, and patients represented hypoventilation. Although TcPCO2 or EtCO2 increased due to hypoventilation, an increase in Edi peak that reflects central respiratory drive was not observed. ΔEdi/ΔCO2 was -0.06μV/mmHg. Maximum EtCO2 or TcPco2 was 51mmHg, and the average SpO2 was 91.5% during monitoring.CONCLUSIONS:We confirmed that Edi monitoring could evaluate the function of respiratory center and reproduce hypoventilation of CCHS. The present study suggested that Edi monitoring is a valuable examination for proceeding genetic testing and it may lead to an early diagnosis of CCHS.
DOI 10.1111/ped.14359
PMID 32573869