Orphanet Journal of Rare Diseases (May 2022)

Do patients with Prader–Willi syndrome have favorable glucose metabolism?

  • Yanjie Qian,
  • Fangling Xia,
  • Yiming Zuo,
  • Mianling Zhong,
  • Lili Yang,
  • Yonghui Jiang,
  • Chaochun Zou

DOI
https://doi.org/10.1186/s13023-022-02344-3
Journal volume & issue
Vol. 17, no. 1
pp. 1 – 15

Abstract

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Abstract Background In recent years, more studies have observed that patients with Prader–Willi syndrome have lower insulin levels and lower insulin resistance than body mass index-matched controls, which may suggest protected glucose metabolism. Method The PubMed and Web of Science online databases were searched to identify relevant studies published in the English language using the terms “Prader–Willi syndrome” with “glucose”, “insulin”, “diabetes mellitus”, “fat”, “adipo*”, “ghrelin”, “oxytocin”, “irisin” or “autonomic nervous system”. Results The prevalence of impaired glucose intolerance, type 2 diabetes mellitus and some other obesity-associated complications in patients with Prader–Willi syndrome tends to be lower when compared to that in general obesity, which is consistent with the hypothetically protected glucose metabolism. Factors including adipose tissue, adiponectin, ghrelin, oxytocin, irisin, growth hormone and the autonomic nervous system possibly modulate insulin sensitivity in patients with Prader–Willi syndrome. Conclusion Although lower insulin levels, lower IR and protected glucose metabolism are widely reported in PWS patients, the causes are still mysterious. Based on existing knowledge, we cannot determine which factor is of utmost importance and what are the underlying mechanisms, and further research is in urgent need.

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