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Prader-Willi Syndrome (Syndrome, Prader-Willi)

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An autosomal dominant disorder caused by deletion of the proximal long arm of the paternal chromosome 15 (15q11-q13) or by inheritance of both of the pair of chromosomes 15 from the mother (UNIPARENTAL DISOMY) which are imprinted (GENETIC IMPRINTING) and hence silenced. Clinical manifestations include MENTAL RETARDATION; MUSCULAR HYPOTONIA; HYPERPHAGIA; OBESITY; short stature; HYPOGONADISM; STRABISMUS; and HYPERSOMNOLENCE. (Menkes, Textbook of Child Neurology, 5th ed, p229)

Also Known As:

Syndrome, Prader-Willi; Labhart Willi Syndrome; Labhart-Willi-Prader-Fanconi Syndrome; Prader Labhart Willi Syndrome; Royer's Syndrome; Willi-Prader Syndrome; Labhart Willi Prader Fanconi Syndrome; Prader Willi Syndrome; Royers Syndrome; Syndrome, Labhart-Willi; Syndrome, Labhart-Willi-Prader-Fanconi; Syndrome, Royer; Syndrome, Royer's; Syndrome, Willi-Prader; Willi Prader Syndrome; Labhart-Willi Syndrome; Royer Syndrome

Networked: 747 relevant articles (21 outcomes, 64 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Morbid Obesity
2.	Obesity
3.	Hyperphagia
4.	Insulin Resistance
5.	Sleep Apnea Syndromes (Sleep Apnea)

Experts

1.	Butler, Merlin G: 18 articles (08/2015 - 06/2003)
2.	Grugni, Graziano: 16 articles (05/2015 - 10/2005)
3.	Wevrick, Rachel: 14 articles (08/2015 - 03/2003)
4.	Hokken-Koelega, Anita C S: 13 articles (09/2015 - 04/2009)
5.	Driscoll, Daniel J: 12 articles (08/2015 - 06/2002)
6.	Höybye, Charlotte: 12 articles (04/2015 - 05/2003)
7.	Jin, Dong-Kyu: 12 articles (07/2012 - 08/2004)
8.	Hokken-Koelega, A C S: 10 articles (12/2015 - 12/2006)
9.	Haqq, Andrea M: 10 articles (10/2015 - 01/2003)
10.	Grugni, G: 10 articles (08/2015 - 04/2000)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Prader-Willi Syndrome:

1.	Growth Hormone (Somatotropin)IBA 01/01/2015 - "Growth hormone (GH) treatment is beneficial for children with Prader-Willi syndrome (PWS), but data about health-related quality of life (HRQOL) and effects of GH treatment are scarce. " 08/01/2002 - "In Prader-Willi syndrome, 2 years of growth hormone therapy also induced a sustained decrease in body fat, improvement in strength and physical skills, and increased lean body mass. " 08/01/2011 - "Growth hormone treatment for two years is safe and effective in adults with Prader-Willi syndrome." 03/01/2013 - "Are sleep studies helpful in children with Prader-Willi syndrome prior to commencement of growth hormone therapy?" 02/01/2013 - "Growth hormone therapy for children and adolescents with Prader-Willi syndrome is associated with improved body composition and metabolic status in adulthood."
2.	Oxytocin (Pitocin)FDA LinkGeneric 09/01/2014 - "Individuals with Prader-Willi syndrome (PWS) have a significant reduction in the number of oxytocin-producing neurons (42%) in the hypothalamic paraventricular nucleus. " 01/01/2011 - "Oxytocin may be useful to increase trust in others and decrease disruptive behaviours in patients with Prader-Willi syndrome: a randomised placebo-controlled trial in 24 patients." 02/01/1995 - "Alterations in the hypothalamic paraventricular nucleus and its oxytocin neurons (putative satiety cells) in Prader-Willi syndrome: a study of five cases." 07/15/2015 - "MAGEL2 and Oxytocin-Implications in Prader-Willi Syndrome and Beyond." 09/01/2014 - "A double-blind randomized controlled trial of oxytocin nasal spray in Prader Willi syndrome."
3.	Human Growth Hormone (Saizen)FDA LinkGeneric 01/01/2004 - "Results of several somatropin (GH therapy) studies in children with Prader-Willi syndrome have shown improvement in growth, body composition, physical strength, and agility. " 01/01/2004 - "However, further studies are required to establish the definite role and optimal dosage of somatropin, as well as long-term effects, in adults with Prader-Willi syndrome." 01/01/2004 - "In conclusion, the one published study of somatropin therapy in adults with Prader-Willi syndrome showed beneficial effects on body composition without pronounced adverse effects. " 08/01/2013 - "Recombinant human growth hormone (hGH) therapy in children with Prader-Willi syndrome (PWS) improves linear growth, body composition, physical strength and agility, and other metabolic parameters. " 08/01/2013 - "Long-term effects of recombinant human growth hormone therapy in children with Prader-Willi syndrome."
4.	modafinil (armodafinil)FDA Link 07/01/2011 - "Efficacy of modafinil on excessive daytime sleepiness in Prader-Willi syndrome." 01/01/2014 - "Long-term modafinil therapy may ameliorate the sleep disturbances of Prader-Willi syndrome and should be the focus of future clinical trials." 01/01/2014 - "In this case report we present a longitudinal perspective of sleep abnormalities in a nine-year-old Caucasian girl with Prader-Willi syndrome from age two to age nine, and detail the response to treatment with the central nervous system stimulant modafinil. "
5.	Gonadal Steroid Hormones (Sex Hormones)IBA 12/01/2001 - "Replacement of GH and/or sex hormones may therefore be beneficial in Prader-Willi syndrome, and several clinical trials have now evaluated GH replacement therapy in affected children. "
6.	Mazindol (Mazanor)FDA Link 11/01/1995 - "Mazindol may prove to be useful in the treatment of patients with Prader-Willi syndrome." 02/24/1997 - "A case of Prader-Willi syndrome with long-term mazindol treatment." 11/01/1995 - "Effects of mazindol in two patients with Prader-Willi syndrome." 11/01/1995 - "Two of 3 patients with Prader-Willi syndrome inhibited their body weight gain with mazindol. " 11/01/1995 - "We examined the effects of mazindol on 1) body weight, appetite, and abnormalities of obesity-related diseases in long-term use 2) maintenance of the reduced body weight after very-low-calorie diet (VLCD) therapy 3) combined use with VLCD therapy and, 4) inhibition of body weight gain in Prader-Willi syndrome. "
7.	Guanfacine (Tenex)FDA LinkGeneric 05/01/2015 - "Significant reduction of aggression with guanfacine extended release in an adolescent with Prader-Willi syndrome."
8.	Tryptophan (L-Tryptophan)FDA Link 04/01/2011 - "An 8-year old girl with Prader-Willi Syndrome presenting with excessive daytime sleepiness improved following treatment with tryptophan; possibly by consolidation of her fragmented sleep. " 04/01/2011 - "We conclude that tryptophan may be an useful medication for excessive sleepiness in children with Prader-Willi Syndrome." 04/01/2011 - "Tryptophan for the treatment of excessive daytime sleepiness in Prader-Willi syndrome."
9.	DeuteriumIBA 06/01/2004 - "To assess body composition of infants with Prader-Willi syndrome (PWS) by using deuterium dilution and investigating the efficacy of early institution of growth hormone (GH) therapy in increasing lean mass (LM) and preventing massive obesity. "
10.	Appetite Depressants (Anorectics)IBA 01/01/2003 - "To date, neither CNS stimulants nor anorectic agents have been effective in treating hyperphagia, in part because hyperphagia in Prader-Willi syndrome is attributed to decreased satiation as opposed to increased hunger. "

Therapies and Procedures

1.	Gastric Bypass (Roux-en-Y Gastric Bypass) 04/01/2014 - "To test the efficacy of the mini-gastric bypass (MGB) in the treatment of morbid obesity related to the Prader-Willi Syndrome (PWS). " 04/01/2014 - "The mini-gastric bypass in the management of morbid obesity in Prader-Willi syndrome: a viable option?" 10/01/2003 - "Gastric bypass in a Japanese man with Prader-Willi syndrome and morbid obesity." 08/30/2006 - "Ghrelin levels are high in Prader-Willi syndrome and low after gastric bypass surgery, possibly contributing to body-weight alterations in these settings. "
2.	Gastrectomy 03/01/2013 - "Laparoscopic sleeve gastrectomy in Chinese female patient with Prader-Willi syndrome and diabetes." 05/01/2009 - ""Efficacy of laparoscopic sleeve gastrectomy as a stand-alone technique for children with morbid obesity" and "BioEnterics intragastric balloon for treatment of morbid obesity in Prader-Willi syndrome: specific risks and benefits"." 11/01/2012 - "Ghrelin level and weight loss after laparoscopic sleeve gastrectomy and gastric mini-bypass for Prader-Willi syndrome in Chinese."
3.	Transplantation (Transplant Recipients) 12/01/1995 - "Encouraging short-term results have been reported in patients with a few other conditions, such as patients with glucocorticoid-induced growth failure, renal transplantation, and Prader-Willi syndrome, but the data are limited and no long-term studies have been reported; in many other conditions the data are either inconclusive or discouraging. "
4.	Psychophysiology (Physiological Psychology) 07/19/2002 - "Adults with Prader-Willi syndrome (PWS) and normal controls participated in a cognitive psychophysiology study in which event-related brain activity was recorded in a visual and auditory oddball task. "
5.	Language Therapy (Language Training) 03/01/2011 - "The aim of this study was to describe the trajectory and the outcomes of speech-language therapy in Prader-Willi syndrome through a longitudinal study of the case of an 8 year-old boy, along four years of speech-language therapy follow-up. " 03/01/2011 - "Trajectory and outcomes of speech language therapy in the Prader-Willi syndrome (PWS): case report."