The Prader-Willi Syndrome shortens the life of patients due to the morbid obesity which it entails. The compulsive hyperphagia associated with it makes a dietetic treatment or a gastroplasty difficult. This study presents the case histories of three patients suffering from the Prader-Willi syndrome who were operated on by means of a Scopinaro's bilio-pancreatic diversion. Following a marked reduction the first year, the weight loss stabilized and then tended to diminish. The observation of three cases which continued for two and a half to six years did not reveal any considerable metabolic problems. The deficiency of iron, vitamins D and B12 as well as folic acid had to be made up by supplementation. These results are comparable with the most favorable ones in the literature. Even if the effect on the weight loss is not spectacular, the operation manages to hold off the development of the obesity, inexorable for those with the Prader-Willi syndrome, and prevents lethal complications, without having notable side effects. Lifting coercive dietary measures improves the quality of life.
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http://dx.doi.org/10.1381/096089291765561529 | DOI Listing |
J Intellect Dev Disabil
June 2023
School of Arts and Sciences, Utah State University, Logan, UT, USA.
Background: Siblings of children with Prader-Willi syndrome (PWS) may be at elevated risk for poor psychological adjustment (Mazaheri, M. M., Rae-Seebach, R.
View Article and Find Full Text PDFJ Psychiatr Res
January 2025
Endocrinology and Nutrition Department, Parc Taulí Hospital Universitari, Institut d'Investigació i Innovació Parc Taulí I3PT, Medicine Department, Universitat Autònoma de Barcelona, 08208, Sabadell, Spain.
Individuals with Prader Willi syndrome (PWS) often exhibit behavioral difficulties characterized by deficient impulse regulation and obsessive-compulsive features resembling those observed in obsessive-compulsive disorder. The genetic configuration of PWS aligns with molecular and neurophysiological findings suggesting dysfunction in the inhibitory gamma-aminobutyric acid (GABA) interneuron system may contribute to its clinical manifestation. In the cerebral cortex, this dysfunction is expressed as desynchronization of local neural activity.
View Article and Find Full Text PDFEndocrinology
January 2025
Department of Cancer Biology & Genetics, College of Medicine, The Ohio State University, Columbus, OH 43210, USA.
Prader-Willi syndrome (PWS) is a rare genetic disease that causes developmental delays, intellectual impairment, constant hunger, obesity, endocrine dysfunction, and various behavioral and neuropsychiatric abnormalities. Standard care of PWS is limited to strict supervision of food intake and growth hormone therapy, highlighting the unmet need for new therapeutic strategies. Environmental enrichment (EE), a housing environment providing physical, social, and cognitive stimulations, exerts broad benefits on mental and physical health.
View Article and Find Full Text PDFAm J Med Genet A
January 2025
Massachusetts General Hospital, Boston, Massachusetts, USA.
Prader-Willi syndrome (PWS) is a genetic disorder associated with baseline respiratory impairment caused by multiple contributing etiologies. While this may be expected to increase the risk of severe COVID-19 infections in PWS patients, survey studies have suggested paradoxically low disease severity. To better characterize the course of COVID-19 infection in patients with PWS, this study analyses the outcomes of hospitalizations for COVID-19 among patients with and without PWS.
View Article and Find Full Text PDFEvid Based Nurs
January 2025
Nursing, Athabasca University, Athabasca, Alberta, Canada.
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