Department of Endocrinology, The Children's Hospital of Zhejiang University School of Medicine, National Clinical Research Center for Child Health, No 3333 Binsheng Road, Hangzhou, 310051, China.
Department of Genetics, Yale University School of Medicine, New Haven, USA.
Orphanet J Rare Dis. 2022 May 7;17(1):187. doi: 10.1186/s13023-022-02344-3.
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.
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".
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.
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.
近年来,越来越多的研究观察到普拉德-威利综合征患者的胰岛素水平和胰岛素抵抗低于体重指数匹配的对照组,这可能表明葡萄糖代谢受到保护。
在 PubMed 和 Web of Science 在线数据库中,使用“Prader-Willi syndrome”和“glucose”、“insulin”、“diabetes mellitus”、“fat”、“adipo*”、“ghrelin”、“oxytocin”、“irisin”或“autonomic nervous system”等术语搜索了以英文发表的相关研究。
与一般肥胖相比,普拉德-威利综合征患者的葡萄糖耐量受损、2 型糖尿病和其他一些肥胖相关并发症的患病率较低,这与假设的葡萄糖代谢受到保护一致。脂肪组织、脂联素、生长激素释放肽、催产素、鸢尾素、生长激素和自主神经系统等因素可能调节普拉德-威利综合征患者的胰岛素敏感性。
尽管 PWS 患者普遍存在胰岛素水平较低、IR 较低和葡萄糖代谢受到保护的情况,但原因仍不清楚。基于现有知识,我们无法确定哪个因素最重要,也无法确定潜在机制,因此急需进一步研究。