Airway obstruction in two children with congenital central hypoventilation syndrome and review of the literature - 04/04/24
, Jérôme Naudin a, Amélia Madani c, Christophe Delclaux b, c, Plamen Bokov b, c, Stéphane Dauger a, cAbstract |
Congenital 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.
Le texte complet de cet article est disponible en PDF.Keywords : Congenital central hypoventilation syndrome, Obstructive sleep apnea, Mechanical ventilation
Abbreviations : CPAP, CHS, EEG, ENT, MV, NICU, NIV, NPARM, ARM, PHOX2B, PICU, PSG
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Vol 31 - N° 3
P. 205-208 - avril 2024 Retour au numéro
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