Airway obstruction in two children with congenital central hypoventilation syndrome and review of the literature

Arch Pediatr. 2024 Apr;31(3):205-208. doi: 10.1016/j.arcped.2023.12.002. Epub 2024 Mar 27.ABSTRACTCongenital central hypoventilation syndrome (CCHS) is an autosomal dominant disease that is caused by heterozygous mutations in the paired-like homeobox 2B gene (PHOX2B). Madani et al. described an abnormally high degree of not only central apnea but also obstructive and mixed apnea in Phox2b27Ala/+newborn mice. Newborns with CCHS must undergo polysomnography for obstructive respiratory events in order to guide the optimal ventilation strategy if oxygen desaturation, bradycardia, and malaise persist under noninvasive ventilation. Newborns and infants with CCHS must be systematically tested for obstructive apnea, especially in cases of inefficient noninvasive ventilation.PMID:38538464 | DOI:10.1016/j.arcped.2023.12.002

Source: Archives de Pediatrie - March 27, 2024 Category: Pediatrics Authors: Richard Wolff Benjamin Dudoignon J érôme Naudin Am élia Madani Christophe Delclaux Plamen Bokov St éphane Dauger Source Type: research