Prader-Willi syndrome–associated obesity treated by biliopancreatic diversion with duodenal switch. Case report and literature review

Papavramidis, Spiros; Kotidis, Efstathios; Gamvros, O.

doi:10.1016/j.jpedsurg.2006.01.065

Cited by 30 publications

(19 citation statements)

References 35 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…Yet, in these patients, a drastic body weight reduction is mandatory to reduce the risk of cardio-respiratory and metabolic complications and to improve life expectancy. In the last three decades, several bariatric surgical techniques were applied to treat morbid obesity in PWS patients, such as trunkal vagotomy [13], jejunoileal bypass [14,15], vertical banded gastroplasty [16,17], adjustable silicon gastric banding [18], gastric bypass [19], and biliopancreatic diversion (BPD) [20][21][22][23][24][25][26]. However, a higher morbidity and mortality rate in these patients, compared to those with simple obesity, has been reported [27].…”

Section: Discussionmentioning

confidence: 99%

BioEnterics Intragastric Balloon for Treatment of Morbid Obesity in Prader–Willi Syndrome: Specific Risks and Benefits

et al. 2008

View full text Add to dashboard Cite

show abstract

Section: Discussionmentioning

confidence: 99%

BioEnterics Intragastric Balloon for Treatment of Morbid Obesity in Prader–Willi Syndrome: Specific Risks and Benefits

et al. 2008

View full text Add to dashboard Cite

show abstract

“…Appetite-reducing drugs used in simple obesity have not shown wide-spread improvements, 102 and bariatric procedures have been associated with severe complications. 103,104 Growth hormone treatment has been shown to improve the fat-muscle mass ratio, 105 and may help to avoid the obesity for the younger generations of people with PWS, but has no effect on eating behaviour. As parents are now aware of the implications of PWS from very early on in their child's life, eating management advice and discipline may help delay problems in future.…”

Section: Treatment Implicationsmentioning

confidence: 99%

Development of the eating behaviour in Prader–Willi Syndrome: advances in our understanding

2010

View full text Add to dashboard Cite

Prader-Willi Syndrome (PWS) is a genetically determined neurodevelopmental disorder associated with mild to moderate intellectual disability, growth and sex-hormone deficiencies and a propensity to overeat that leads to severe obesity. The PWS phenotype changes from an early disinterest in food to an increasing pre-occupation with eating and a failure of the normal satiety response to food intake. The prevention of severe obesity is primarily through strict control of access to food and it is this aspect that most limits the independence of those with PWS. This review considers the eating disorder in PWS, specifically how the as yet uncertain genetics of the syndrome and the transition from the early to the later phenotype might account for the later hyperphagia. On the basis of behavioural and imaging studies, a failure of satiety and excessive activation of neural reward pathways have both been suggested. We speculate that the overeating behaviour, consequent upon one or other of the above, could either be due to a direct effect of the PWS genotype on the feeding pathways of the hypothalamus or a consequence of prenatal changes in the regulation of genes responsible for energy balance that sets a high satiation threshold. Understanding the overeating in PWS will lead to more focused and successful management and ultimately, treatment of this life-threatening behaviour. International Journal of Obesity (2011) IntroductionWe review the present state of knowledge on the eating disorder and potential resultant life-threatening obesity that is associated with Prader-Willi Syndrome (PWS), a genetically determined neurodevelopmental disorder affecting 1 in 22-25 000 live births.1-3 The aims of the review are: first, to consider our understanding of the eating disorder associated with PWS primarily from a behavioural and neuroscience perspective; second, to speculate as to the possible mechanisms that might directly or indirectly link the PWS genotype to the 'eating' phenotype; and third, to reflect on the implications for future research and for treatment. We discuss the major theories that could account for eating behaviour and whether any of these adequately explain the complex array of characteristics that exist in PWS and fit with what we know about the pathophysiology and the genetics of the disorder. The main discussion items include considering PWS as a disorder of satiety, as well as theories of hyper-responsive rewards systems and over-sensitivity to food stimuli, as has been proposed in general obesity. Further, we consider the importance of the prenatal environment and examine the possibility that people with PWS have an altered inner physical awareness, as shown by abnormal pain responses, and how this may alter the eating behaviour. MethodThis review has been carried out through a search of relevant articles primarily on the PubMed search engine (www. pubmed.gov) using 'eating behaviour Prader-Willi syndrome OR Prader Willi syndrome' as the search term. As the eating behaviour is such a key feature of t...

show abstract

“…In our series we have shown feasibility and success in a male patient with Bardet-Biedl syndrome with application of RNYGB (Daskalakis et al 2010), whereas several reports are published on PWS patients treated with either BPD (Papavramidis et al 2006) or BDS-Scopinaro technique (Marinari et al 2001;Brossy 1989). Sugerman et al (2003) reported a series of 32 children with a mean age of 16 AE 1 years (range: 12.4-17.9 years) using various operations including gastric bypass with various intestinal limb lengths.…”

Section: Restrictive Proceduresmentioning

confidence: 76%

Is There an Indication for BPD-DS/BPD? What the Potential Advantages/Disadvantages to RYNGB?

Weiner

2014

Morbid Obesity in Adolescents

View full text Add to dashboard Cite

Prader-Willi syndrome–associated obesity treated by biliopancreatic diversion with duodenal switch. Case report and literature review

Cited by 30 publications

References 35 publications

BioEnterics Intragastric Balloon for Treatment of Morbid Obesity in Prader–Willi Syndrome: Specific Risks and Benefits

BioEnterics Intragastric Balloon for Treatment of Morbid Obesity in Prader–Willi Syndrome: Specific Risks and Benefits

Development of the eating behaviour in Prader–Willi Syndrome: advances in our understanding

Is There an Indication for BPD-DS/BPD? What the Potential Advantages/Disadvantages to RYNGB?

Contact Info

Product

Resources

About