Abstract
Hyperphagia is one of the main problems of patients with Prader-Willi syndrome (PWS) to cope with everyday life. The underlying mechanisms are not yet well understood. Gut-brain hormones are an interrelated network that may be at least partially involved. We aimed to study the hormonal profile of PWS patients in comparison with obese and healthy controls. Thirty adult PWS patients (15 men; age 27.5 ± 8.02 years; BMI 32.4 ± 8.14 kg/m2), 30 obese and 30 healthy controls were studied before and after eating a hypercaloric liquid diet. Plasma brain-derived neurotrophic factor (BDNF), leptin, total and active ghrelin, peptide YY (PYY), pancreatic polypeptide (PP), Glucagon-like peptide-1 (GLP-1), glucose-dependent insulinotropic polypeptide (GIP) and amylin were determined at times 0′, 30′, 60′ and 120′. Cluster analysis was used. When considering all peptides together, two clusters were established according to fasting hormonal standardized concentrations. Cluster 1 encompassed most of obese (25/30) and healthy controls (28/30). By contrast, the majority of patients with PWS were located in Cluster 2 (23/27) and presented a similar fasting profile with hyperghrelinemia, high levels of leptin, PYY, GIP and GLP-1, compared to Cluster 1; that may reflect a dysfunction of these hunger/satiety hormones. When peptide behavior over the time was considered, PP concentrations were not sustained postprandially from 60 min onwards in Cluster 2. BDNF and amylin did not help to differentiate the two clusters. Thus, cluster analysis could be a good tool to distinguish and characterize the differences in hormone responses between PWS and obese or healthy controls.
Highlights
Prader-Willi syndrome (PWS) is the first genetic syndromic cause of obesity and is caused by the lack of expression of the genes contained in the 15q11-q13 region of chromosome 15 of paternal origin
We aimed to evaluate different peptides involved in the regulation of appetite in the PWS individuals and assess the possible existence of a hormonal pattern that may help to explain the exaggerated hyperphagia in this syndrome
We studied 30 adult patients with genetically confirmed PWS, 30 obese controls matched by age, sex and body mass index (BMI) and 30 lean controls matched by age and sex
Summary
Prader-Willi syndrome (PWS) is the first genetic syndromic cause of obesity and is caused by the lack of expression of the genes contained in the 15q11-q13 region of chromosome 15 of paternal origin. Given that ghrelin is a potent orexigen [23], the constant finding of high concentrations of such peptide in subjects with PWS suggests that this may be the cause of its hyperphagia. Some studies show that hyperghrelinemia is present in very young children before the onset of hyperphagia [5,24] and the reduction of ghrelin concentrations with an infusion of somatostatin or with analogues thereof does not induce a reduction in appetite or body weight or a change in eating behavior in these patients [25,26]. We aimed to evaluate different peptides involved in the regulation of appetite in the PWS individuals and assess the possible existence of a hormonal pattern that may help to explain the exaggerated hyperphagia in this syndrome. We compared the hormonal pattern to age and sex matched obese and lean controls
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