Metabolic and Adipose Tissue Signatures in Adults With Prader-Willi Syndrome: A Model of Extreme Adiposity

Lacroix, Dan; Moutel, Sandrine; Coupaye, Muriel; Huvenne, Hélène; Faucher, Pauline; Pelloux, Véronique; Rouault, Christine; Bastard, Jean‐Philippe; Cagnard, Nicolas; Dubern, B.; Clément, Karine; Poitou, Christine

doi:10.1210/jc.2014-3127

Cited by 41 publications

(50 citation statements)

References 42 publications

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“…PWS individuals may be less likely to develop T2DM than non-syndromic subjects with comparable obesity. In fact, the lower fasting insulin and HOMA-IR were shown in not only PWS children but also PWS adults 5,6) . However, recent study suggested further investigation with hyperinsulinemic-euglycemic clamp due to failure to reprove this identification 7) .…”

Section: Altered Glucose Metabolism In Pwsmentioning

confidence: 93%

An Update on Prader-Willi Syndrome with Diabetes Mellitus

Lee¹

2016

Journal of mucopolysaccharidosis and rare disease

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Prader-Willi syndrome (PWS) often develops type 2 diabetes mellitus (T2DM) related to severe obesity. The prevalence of T2DM in adults with PWS (7-20%) exceeds greatly the prevalence in the general population (5-7%). It is uncommon for pre-pubertal children with PWS to develop overt diabetes or glucose intolerance. GH therapy and genotype did not influence the development of altered glucose metabolism. It has been assumed that T2DM in PWS develops as a consequence of morbid obesity and concomitant insulin resistance. However recent studies suggest the relationship between morbid obesity and T2DM development is more complex and appears to differ in PWS subjects compared to non-PWS subjects. PWS patients had relatively lower fasting insulin levels and increased adiponectin levels compared with BMI-matched obese control despite of similar levels of leptin. So PWS children may be protected to some extent form of obesity-associated insulin resistance. Although there's no data, it seems logical to approach diabetes management including weight loss and increased exercise, using similar pharmacological agents as with non-PWS obesity-related diabetes such as metformin or thiazolidinedione, with the introduction of insulin as required. On the other hand, several recent T2DM in PWS case reports suggest favorable outcomes using Glucagon-like peptide 1 (GLP-1) analog with regard to ghrelin reduction, control of glucose and appetite, weight loss and pre-prandial insulin secretion. The role of GLP-1 agonist therapy is promising, but has not yet been fully elucidated.

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Section: Altered Glucose Metabolism In Pwsmentioning

confidence: 93%

An Update on Prader-Willi Syndrome with Diabetes Mellitus

Lee¹

2016

Journal of mucopolysaccharidosis and rare disease

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“…Ainsi, un profil normal de méthylation de la région 15q11-q12 exclut à 99 % le diagnostic de syndrome de Prader-Willi. [6]. Le traitement par l'hormone de croissance permet de restaurer, au moins partiellement, certaines propriétés du tissu adipeux, avec chez l'enfant, une normalisation du nombre de précurseurs adipocytaires.…”

Section: Diagnosticunclassified

“…La publication par la Haute autorité de santé du programme national de diagnostic et de soins 6 en 2012, a permis de décrire la prise en charge multidisciplinaire dans le centre de référence et les centres de compé-tence qui sont répartis sur le territoire, en relation avec le médecin traitant. En 2011, le plan Obésité a labellisé deux établissements de soins de suite et de réadaptation pour enfants (un en Île-de-France et un près de Toulouse), en lien avec les sites des centres de référence, pour l'accueil des enfants atteints de SPW et d'autres obésités syndromiques ou apparentées.…”

Section: La Prise En Charge Une Organisation Des Soins Et Un Maillageunclassified

Le syndrome de Prader-Willi en 2015

2015

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“…The development of AT occurs early in PWS infants before the onset of weight gain 25) , so PWS can be seen as an exceptional model of early and massive AT development, notably in subcutaneous depots 7) . The mechanisms of reduced LBM in PWS patients are not fully understood.…”

Section: Adipose Tissue In Prader-willi Syndromementioning

confidence: 99%

“…However, obesity remains a critical problem in PWS teenagers and adults because it leads to severe complications, such as sleep disorder, cardiac or respiratory failure, and physical disability 6) . Obesity status also plays on a crucial role in development of metabolic syndrome (Mets) in PWS children and adults [7][8][9][10] . Several studies focused on the metabolic outcomes of PWS adults.…”

mentioning

confidence: 99%