862 Volume-Assured Pressure Support improves outcomes in a patient with Congenital Central Hypoventilation Syndrome

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منابع مشابه

Congenital central hypoventilation syndrome

Congenital central hypoventilation syndrome (CCHS) is a rare disorder of the automatic control of breathing. The literary misnomer "Ondine's curse" has been used in prior literatures and the disease was first described in 1970 by Mellins et al.1 The hallmark of the disease is alveolar hypoventilation with insensitivity to hypoxaemia and hypercapnia, most pronounced during sleep, but the clinica...

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Congenital central hypoventilation syndrome

3 Nelson D A, Weiner A, Yanoff M, DePeralta J. Retinal lesions in subacute sclerosing panencephalitis. Arch Ophthalmol 1970; 84: 613-21. 4 Andriola M, Karlsberg R 0. Maculopathy in subacute sclerosing panencephalitis. Am J Dis Child 1972; 124: 187-9. 5 Cape C A, Martinez J, Robertson J T, Hamilton R, Jabbour J T. Adult onset of subacute sclerosing panencephalitis. Arch Neurol 1973; 28: 124-7. 6...

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Congenital Central Hypoventilation Syndrome

Congenital central hypoventilation syndrome (CCHS) is a rare neurocristopathy with disordered respiratory control and autonomic nervous system regulation. CCHS is caused by mutations in the PHOX2B gene, and the PHOX2B genotype/mutation anticipates the CCHS phenotype, including the severity of hypoventilation, risk of sinus pauses, and risk of associated disorders including Hirschsprung disease ...

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Hippocampal Volume Reduction in Congenital Central Hypoventilation Syndrome

Children with congenital central hypoventilation syndrome (CCHS), a genetic disorder characterized by diminished drive to breathe during sleep and impaired CO(2) sensitivity, show brain structural and functional changes on magnetic resonance imaging (MRI) scans, with impaired responses in specific hippocampal regions, suggesting localized injury.We assessed total volume and regional variation i...

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Average volume-assured pressure support in obesity hypoventilation: A randomized crossover trial.

BACKGROUND Average volume-assured pressure support (AVAPS) has been introduced as a new additional mode for a bilevel pressure ventilation (BPV) device (BiPAP; Respironics; Murrysville, PA), but studies on the physiologic and clinical effects have not yet been performed. There is a particular need to better define the most efficient ventilatory treatment modality for patients with obesity hypov...

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ژورنال

عنوان ژورنال: Sleep

سال: 2021

ISSN: 0161-8105,1550-9109

DOI: 10.1093/sleep/zsab072.859