Nutrition and Obesity Management in Prader-Willi Syndrome and Other Forms of Genetic Obesity
A special issue of Nutrients (ISSN 2072-6643). This special issue belongs to the section "Nutrition and Public Health".
Deadline for manuscript submissions: closed (30 September 2021) | Viewed by 3850
Special Issue Editor
Special Issue Information
Dear Colleagues,
It is with pleasure I announce the Special Issue for Nutrients entitled “Nutrition and Obesity Management in Prader–Willi syndrome and Other Forms of Genetic Obesity” that aims to cover the most recent understandings and discoveries on the prevention of obesity and treatment of hyperphagia in these patients.
Genetic obesity includes severe and rare forms of early-onset obesity caused by mutations in a single gene (monogenic obesity) and by dysregulated hunger satiety circuits in the hypothalamus (the mutation in the melanocortin-4 receptor gene (MC4R) is the most common form). In this context, there are “syndromic obesities” where obesity occurs in the presence of mental delays, dysmorphic features, short stature, organ-specific abnormalities, hyperphagia, and/or other signs of hypothalamic dysfunction (Prader–Willi syndrome, Bardet-Biedl syndrome, Alström syndrome, Rohhadnet syndrome, etc.).
Prader–Willi syndrome (PWS) is the most common form of syndromic obesity occurring in about 1:25,000 live births due to the loss of expression of paternal alleles in the PWS region of chromosome 15. These genetic anomalies lead to a well-described clinical phenotype that includes hypotonia, hypothalamic dysfunction, behavior and psychiatric problems, and the development of life-threatening obesity. Constant and obsessive food-seeking behavior can make life very difficult both for the family and caretakers. Moreover, PWS subjects die prematurely from complications conventionally related to obesity.
Consequently, dietary restriction, strict control over access to food, continuous lifelong supervision, and regular physical activity are still the only available options in all genetic obesity.
Up to now, no drugs have proven to be effective in controlling appetite but current ongoing clinical trials with new drugs seem to be encouraging. The long-term safety and effectiveness of bariatric surgery in PWS patients and syndromic obesity with life-threatening comorbidities remain unknown.
This Special Issue welcomes original research articles and reviews of the literature by researchers and study groups who work every day in this field.
I look forward to receiving your contributions.
Dr. Antonino Crinò
Guest Editor
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Keywords
- Prader–Willi syndrome
- genetic obesity
- obesity
- nutrition
- dietary intake
- hyperphagia
- food management
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