Next Article in Journal
Preventive Effects of Cocoa and Cocoa Antioxidants in Colon Cancer
Next Article in Special Issue
The Developmental Trajectory of Self-Injurious Behaviours in Individuals with Prader Willi Syndrome, Autism Spectrum Disorder and Intellectual Disability
Previous Article in Journal / Special Issue
Obesity and Prader-Willi Syndrome Affect Heart Rate Recovery from Dynamic Resistance Exercise in Youth
Article Menu

Export Article

Open AccessReview
Diseases 2016, 4(1), 5; doi:10.3390/diseases4010005

Pituitary-Adrenal Axis in Prader Willi Syndrome

Developmental Endocrinology Research Group, Royal Hospital for Children, University of Glasgow, 1345 Govan Road, Glasgow G41 4TF, UK
*
Author to whom correspondence should be addressed.
Academic Editor: Danny Camfferman
Received: 17 December 2015 / Revised: 10 January 2016 / Accepted: 12 January 2016 / Published: 19 January 2016
(This article belongs to the Special Issue Prader-Willi Syndrome)
View Full-Text   |   Download PDF [186 KB, uploaded 19 January 2016]

Abstract

Purpose: Prader Willi syndrome (PWS) is a rare genetic condition that has concurrent endocrinological insufficiencies. The presence of growth hormone deficiency has been well documented, but adrenal insufficiency (AI) is not widely reported. A review was conducted to investigate its prevalence and relevance in PWS in both adults and children. Methodology: A literature review was performed with the search terms “Prader-Willi syndrome” and “adrenal insufficiency”. Results: The review found studies disagree on the prevalence and method of investigation of AI in PWS. Case studies demonstrate that patients with PWS are at risk of premature death, often secondary to respiratory infections. The possibility that this may be the result of the inability to mount an effective cortisol response has been studied, with some evidence confirming AI in PWS patients. Most reports agreed AI is present in PWS, however, Farholt et al. showed no HPA axis dysfunction in adults, suggesting that perhaps it is rare in adults, and children should be the focus of further studies. Conclusion: AI is present in some patients with PWS. Further research is required to ensure optimal treatment can be implemented and to prevent premature deaths related to adrenal insufficiency. Clinicians should have a low threshold for testing the adrenal axis and considering treatment for adrenal insufficiency in PWS patients. View Full-Text
Keywords: adrenal; steroid; hydrocortisone; PWS adrenal; steroid; hydrocortisone; PWS
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

Edgar, O.S.; Lucas-Herald, A.K.; Shaikh, M.G. Pituitary-Adrenal Axis in Prader Willi Syndrome. Diseases 2016, 4, 5.

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.

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