Next Article in Journal
The Protective Effect of Antioxidants Consumption on Diabetes and Vascular Complications
Previous Article in Journal
The Role of Glucosinolate Hydrolysis Products from Brassica Vegetable Consumption in Inducing Antioxidant Activity and Reducing Cancer Incidence
Previous Article in Special Issue
Prader-Willi Syndrome: The Disease that Opened up Epigenomic-Based Preemptive Medicine
Article Menu

Export Article

Open AccessReview
Diseases 2016, 4(3), 23; doi:10.3390/diseases4030023

Disorders of Sleep and Ventilatory Control in Prader-Willi Syndrome

Division of Pediatric Pulmonology and Sleep Medicine, Children’s Hospital Los Angeles, 4650 Sunset Blvd, Mailstop #83, Los Angeles, CA 90027, USA
*
Author to whom correspondence should be addressed.
Academic Editor: Danny Camfferman
Received: 3 May 2016 / Revised: 1 July 2016 / Accepted: 4 July 2016 / Published: 8 July 2016
(This article belongs to the Special Issue Prader-Willi Syndrome)
View Full-Text   |   Download PDF [224 KB, uploaded 8 July 2016]

Abstract

Prader-Willi syndrome (PWS) is an imprinted genetic disorder conferred by loss of paternal gene expression from chromosome 15q11.2-q13. Individuals with PWS have impairments in ventilatory control and are predisposed toward sleep disordered breathing due to a combination of characteristic craniofacial features, obesity, hypotonia, and hypothalamic dysfunction. Children with PWS progress from failure to thrive during infancy to hyperphagia and morbid obesity during later childhood and onward. Similarly, the phenotype of sleep disordered breathing in PWS patients also evolves over time from predominantly central sleep apnea in infants to obstructive sleep apnea (OSA) in older children. Behavioral difficulties are common and may make establishing effective therapy with continuous positive airway pressure (CPAP) more challenging when OSA persists after adenotonsillectomy. Excessive daytime sleepiness (EDS) is also common in patients with PWS and may continue after OSA is effectively treated. We describe here the characteristic ventilatory control deficits, sleep disordered breathing, and excessive daytime sleepiness seen in individuals with PWS. We review respiratory issues that may contribute to sudden death events in PWS patients during sleep and wakefulness. We also discuss therapeutic options for treating sleep disordered breathing including adenotonsillectomy, weight loss, and CPAP. Lastly, we discuss the benefits and safety considerations related to growth hormone therapy. View Full-Text
Keywords: Prader-Willi syndrome; obstructive sleep apnea; sleep disorders; ventilatory control; obesity; growth hormone; adenotonsillectomy; non-invasive ventilation; bariatric surgery; hypocretin Prader-Willi syndrome; obstructive sleep apnea; sleep disorders; ventilatory control; obesity; growth hormone; adenotonsillectomy; non-invasive ventilation; bariatric surgery; hypocretin
This is an open access article distributed under the Creative Commons Attribution License which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. (CC BY 4.0).

Scifeed alert for new publications

Never miss any articles matching your research from any publisher
  • Get alerts for new papers matching your research
  • Find out the new papers from selected authors
  • Updated daily for 49'000+ journals and 6000+ publishers
  • Define your Scifeed now

SciFeed Share & Cite This Article

MDPI and ACS Style

Gillett, E.S.; Perez, I.A. Disorders of Sleep and Ventilatory Control in Prader-Willi Syndrome. Diseases 2016, 4, 23.

Show more citation formats Show less citations formats

Note that from the first issue of 2016, MDPI journals use article numbers instead of page numbers. See further details here.

Related Articles

Article Metrics

Article Access Statistics

1

Comments

[Return to top]
Diseases EISSN 2079-9721 Published by MDPI AG, Basel, Switzerland RSS E-Mail Table of Contents Alert
Back to Top