Literature DB >> 20962018

The metabolic phenotype of Prader-Willi syndrome (PWS) in childhood: heightened insulin sensitivity relative to body mass index.

Andrea M Haqq1, Michael J Muehlbauer, Christopher B Newgard, Steven Grambow, Michael Freemark.   

Abstract

CONTEXT: Insulin sensitivity is higher in patients with Prader-Willi syndrome (PWS) than in body mass index-matched obese controls (OCs). Factors contributing to the heightened insulin sensitivity of PWS remain obscure. We compared the fasting levels of various hormones, cytokines, lipids, and liver function tests in 14 PWS patients and 14 OCs with those in 14 age- and gender-matched lean children (LC). We hypothesized that metabolic profiles of children with PWS are comparable with those of LC, but different from those of OCs.
RESULTS: Leptin levels were comparable in PWS patients and OCs, suggesting comparable degrees of adiposity. Glucose levels were comparable among groups. However, fasting insulin concentrations and homeostasis model assessment insulin resistance index were lower in PWS patients than in OCs (P < 0.05) and similar to LC. Moreover, high-density lipoprotein levels were lower and triglycerides higher in OCs (P < 0.05) but not PWS patients. Total adiponectin, high-molecular-weight (HMW) adiponectin and the HMW to total adiponectin ratio were higher in PWS patients (P < 0.05) than in OCs and similar to LC. High-sensitivity C-reactive protein and IL-6 levels were higher in OCs than in PWS patients or LC (P < 0.05). Nevertheless, PAI-1 levels were elevated in both OC and PWS patients. There were no group differences in glucagon-like peptide-1, macrophage chemoattractant protein-1, TNFα, IL-2, IL-8, IL-10, IL-12p40, IL-18, resistin, total or low-density lipoprotein cholesterol, aspartate aminotransferase, or alanine aminotransferase.
CONCLUSIONS: The heightened insulin sensitivity of PWS patients relative to OCs is associated with higher levels of adiponectin and lower levels of high-sensitivity C-reactive protein and IL-6. Future studies will determine whether PWS children are protected from obesity comorbidities such as type 2 diabetes, hyperlipidemia, and nonalcoholic fatty liver disease.

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Year:  2010        PMID: 20962018      PMCID: PMC3038476          DOI: 10.1210/jc.2010-1733

Source DB:  PubMed          Journal:  J Clin Endocrinol Metab        ISSN: 0021-972X            Impact factor:   5.958


  40 in total

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2.  Prader-Willi syndrome is associated with activation of the innate immune system independently of central adiposity and insulin resistance.

Authors:  Alexander Viardot; Lisa Sze; Louise Purtell; Amanda Sainsbury; Georgina Loughnan; Ellie Smith; Herbert Herzog; Katharine Steinbeck; Lesley V Campbell
Journal:  J Clin Endocrinol Metab       Date:  2010-05-05       Impact factor: 5.958

3.  Regional variation in plasminogen activator inhibitor-1 expression in adipose tissue from obese individuals.

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4.  Growth hormone and body composition in children younger than 2 years with Prader-Willi syndrome.

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5.  Serum adiponectin levels in adults with Prader-Willi syndrome are independent of anthropometrical parameters and do not change with GH treatment.

Authors:  Charlotte Hoybye; Jens M Bruun; Bjorn Richelsen; Allan Flyvbjerg; Jan Frystyk
Journal:  Eur J Endocrinol       Date:  2004-10       Impact factor: 6.664

6.  Plasma PAI-1 levels are more strongly related to liver steatosis than to adipose tissue accumulation.

Authors:  Marie-Christine Alessi; Delphine Bastelica; Alenka Mavri; Pierre Morange; Bruno Berthet; Michel Grino; Irene Juhan-Vague
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Journal:  Thromb Haemost       Date:  2002-09       Impact factor: 5.249

8.  Adiponectin in youth: relationship to visceral adiposity, insulin sensitivity, and beta-cell function.

Authors:  Fida Bacha; Rola Saad; Neslihan Gungor; Silva A Arslanian
Journal:  Diabetes Care       Date:  2004-02       Impact factor: 19.112

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Authors:  David E Laaksonen; Leo Niskanen; Kari Punnonen; Kristiina Nyyssönen; Tomi-Pekka Tuomainen; Riitta Salonen; Rainer Rauramaa; Jukka T Salonen
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10.  Visceral adipose tissue and metabolic complications of obesity are reduced in Prader-Willi syndrome female adults: evidence for novel influences on body fat distribution.

Authors:  A P Goldstone; E L Thomas; A E Brynes; J D Bell; G Frost; N Saeed; J V Hajnal; J K Howard; A Holland; S R Bloom
Journal:  J Clin Endocrinol Metab       Date:  2001-09       Impact factor: 5.958

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3.  Impairment of adipose tissue in Prader-Willi syndrome rescued by growth hormone treatment.

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Review 4.  Update on Diabetes Mellitus and Glucose Metabolism Alterations in Prader-Willi Syndrome.

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Journal:  Curr Diab Rep       Date:  2020-02-06       Impact factor: 4.810

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6.  Hormonal and metabolic effects of carbohydrate restriction in children with Prader-Willi syndrome.

Authors:  Krystal A Irizarry; Diana R Mager; Lucila Triador; Michael J Muehlbauer; Andrea M Haqq; Michael Freemark
Journal:  Clin Endocrinol (Oxf)       Date:  2019-01-31       Impact factor: 3.478

7.  Metabolic profiling in Prader-Willi syndrome and nonsyndromic obesity: sex differences and the role of growth hormone.

Authors:  Krystal A Irizarry; James Bain; Merlin G Butler; Olga Ilkayeva; Michael Muehlbauer; Andrea M Haqq; Michael Freemark
Journal:  Clin Endocrinol (Oxf)       Date:  2015-04-01       Impact factor: 3.478

8.  Effects of MetAP2 inhibition on hyperphagia and body weight in Prader-Willi syndrome: A randomized, double-blind, placebo-controlled trial.

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Journal:  Diabetes Obes Metab       Date:  2017-07-13       Impact factor: 6.577

9.  Effects of metformin in children and adolescents with Prader-Willi syndrome and early-onset morbid obesity: a pilot study.

Authors:  Jennifer L Miller; Tiffany D Linville; Elisabeth M Dykens
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10.  [Non-alcoholic steatohepatitis and cirrhosis in young adult patients with hypothalamic-pituitary dysfunction].

Authors:  D Goltz; K Schötta; H Zhou; H-P Fischer
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