Division of Endocrinology, Diabetes and Metabolism, Department of Medicine, University of Verona and Azienda Ospedaliera Universitaria Integrata of Verona, Verona, Italy.
Inherited Metabolic Diseases Unit and Regional Centre for Newborn Screening, Diagnosis and Treatment of Inherited Metabolic Diseases and Congenital Endocrine Diseases, Azienda Ospedaliera Universitaria Integrata, Verona, Italy.
Endocr Metab Immune Disord Drug Targets. 2022;22(10):1053-1057. doi: 10.2174/1871530322666220509225637.
Prader-Willi syndrome is the most frequent genetic cause of obesity and is often complicated by glucose metabolism alterations. Conventional therapies prescribed for type 2 diabetes frequently failed to achieve adequate glycemic control in patients with Prader-Willi syndrome. Beneficial effects of glucagon like peptide-1 receptor agonists exenatide and liraglutide have been reported for the management of type 2 diabetes in Prader-Willi syndrome, but no data are currently available in this population on the use of semaglutide.
We report for the first time the use of semaglutide 1 mg per week in a 33-yearold man with Prader-Will syndrome complicated by poorly controlled diabetes and severe obesity. After 12 months of semaglutide treatment, we observed an important reduction in glycated hemoglobin levels (11.1% to 7.2%) and body weight (99.5 kg to 94.3 kg), with a notable decrease in fat mass and insulin requirements. Interestingly, our patient had already tried liraglutide therapy in adjunction to metformin and insulin therapy, reporting no substantial efficacy.
The beneficial effects of semaglutide on glycemic control and weight reduction provide a promising treatment for diabetes and obesity in Prader-Willi syndrome, even where other glucagons like peptide-1 receptor agonists have failed. Further studies are required to confirm the efficacy and safety of semaglutide in patients with Prader-Willi syndrome.
普拉德-威利综合征是肥胖症最常见的遗传原因，常伴有葡萄糖代谢改变。对于 2 型糖尿病患者，常规治疗方案常无法实现血糖的充分控制。胰高血糖素样肽-1 受体激动剂艾塞那肽和利拉鲁肽对普拉德-威利综合征 2 型糖尿病的治疗有益，但目前尚无关于在该人群中使用司美格鲁肽的数据。
我们首次报告了每周使用 1 毫克司美格鲁肽治疗一名 33 岁患有普拉德-威利综合征的男性患者，该患者患有控制不佳的糖尿病和严重肥胖症。在司美格鲁肽治疗 12 个月后，我们观察到糖化血红蛋白水平（从 11.1%降至 7.2%）和体重（从 99.5 公斤降至 94.3 公斤）显著降低，脂肪量和胰岛素需求明显减少。有趣的是，我们的患者已经尝试过在二甲双胍和胰岛素治疗的基础上加用利拉鲁肽治疗，但没有明显的疗效。
司美格鲁肽在血糖控制和体重减轻方面的有益作用为普拉德-威利综合征的糖尿病和肥胖症提供了一种有前途的治疗方法，即使在其他胰高血糖素样肽-1 受体激动剂治疗失败的情况下也是如此。需要进一步的研究来确认司美格鲁肽在普拉德-威利综合征患者中的疗效和安全性。
