4 results match your criteria: "Branche "Adultes" du Centre de Référence du Syndrome d'Ondine[Affiliation]"
Current Perspectives for the use of Gonane Progesteronergic Drugs in the Treatment of Central Hypoventilation Syndromes.
Curr Neuropharmacol
November 2018
Sorbonne Universites, UPMC Univ Paris 06, INSERM, UMR_S1158 Neurophysiologie Respiratoire Experimentale et Clinique, F-75013, Paris, France.
Article Synopsis
- Central alveolar hypoventilation syndromes (CHS) are serious neurorespiratory disorders that lead to dangerous levels of carbon dioxide and low oxygen, requiring patients to rely on assisted ventilation for their entire lives.
- Recent research suggests that gonane synthetic progestins, such as desogestrel, may improve respiratory function in CHS patients by restoring CO2 chemosensitivity, although results have been inconsistent.
- Further studies are needed to clarify how these progestins affect breathing mechanisms in CHS and determine their practical clinical applications.
Normal sleep on mechanical ventilation in adult patients with congenital central alveolar hypoventilation (Ondine's curse syndrome).
Orphanet J Rare Dis
January 2017
Sorbonne Universités, UPMC Université Paris 06, INSERM, UMRS1158 "Neurophysiologie Respiratoire Expérimentale et Clinique", Paris, France.
Background: The purpose of this study was to describe the sleep structure (especially slow wave sleep) in adults with congenital central hypoventilation syndrome (CCHS), a rare genetic disease due to mutations in the PHOX2B gene. Fourteen patients aged 23 (19.0; 24.
View Article and Find Full Text PDFDesogestrel enhances ventilation in ondine patients: Animal data involving serotoninergic systems.
Neuropharmacology
August 2016
Sorbonne Universités, UPMC Univ Paris 06, INSERM, UMR_S1158 Neurophysiologie respiratoire expérimentale et clinique, F-75013, Paris, France. Electronic address:
Congenital central hypoventilation syndrome (CCHS) is a neurorespiratory disease characterized by life-threatening sleep-related hypoventilation involving an alteration of CO2/H(+) chemosensitivity. Incidental findings have suggested that desogestrel may allow recovery of the ventilatory response to CO2. The effects of desogestrel on resting ventilation have not been reported.
View Article and Find Full Text PDFHealth-related quality of life in young adults with congenital central hypoventilation syndrome due to PHOX2B mutations: a cross-sectional study.
Respir Res
June 2015
AP-HP, Groupe Hospitalier Pitié-Salpêtrière Charles Foix, Branche "Adultes" du Centre de Référence du Syndrome d'Ondine, F-75013, Paris, France.
Background: Congenital central hypoventilation syndrome (CCHS) is a rare genetic disease due to PHOX2B mutations. CCHS patients suffer from many autonomic disorders, dominated clinically by defective ventilatory automatisms. From birth, the life of CCHS patients depends on ventilatory support during sleep, involving a high burden of care.
View Article and Find Full Text PDF