Soomro Faiza H, Razzaq Aneela, Siddiq Ghulam
General Surgery, The Dudley Group NHS Foundation Trust, Dudley, GBR.
General Surgery, Shifa International Hospital, Islamabad, PAK.
Cureus. 2022 Aug 12;14(8):e27955. doi: 10.7759/cureus.27955. eCollection 2022 Aug.
Hyperphagia leading to obesity is the most common cause of mortality and morbidity in Prader-Willi syndrome (PWS). It has been classified as the most common genetic cause of the development of life-threatening obesity resulting from a defect in satiety, with an onset during early childhood. Abnormalities in the feedback from gut peptides, including ghrelin, may contribute to the satiety defect; autonomic dysfunction may also play a role in impaired satiety. Usually, pharmacological treatment is ineffective in managing obesity in these patients. A 19-year-old male child with Prader-Willi syndrome presented with morbid obesity, obstructive sleep apnea, and impaired glycemic control. The patient had complained of hyperphagia since early childhood, but food intake increased aggressively in the last few years, which resulted in morbid obesity. The patient was treated with laparoscopic sleeve gastrectomy, and the residual stomach volume was 100 ml. The intervention resulted in a 37.1% weight reduction after one year of surgery with well-controlled blood sugar levels. The patient also reported improved overall quality of life, mood, and functionality. Laparoscopic sleeve gastrectomy can be offered to obese Prader-Willi syndrome patients with heightened mortality, particularly because no other effective alternative therapy is available.
导致肥胖的食欲亢进是普拉德-威利综合征（PWS）最常见的死亡和发病原因。它已被归类为由于饱腹感缺陷导致危及生命的肥胖发展的最常见遗传原因，发病于儿童早期。包括胃饥饿素在内的肠道肽反馈异常可能导致饱腹感缺陷；自主神经功能障碍也可能在饱腹感受损中起作用。通常，药物治疗对这些患者的肥胖管理无效。一名19岁的普拉德-威利综合征男性患儿出现病态肥胖、阻塞性睡眠呼吸暂停和血糖控制受损。该患者自幼儿期起就抱怨食欲亢进，但在过去几年中食物摄入量急剧增加，导致病态肥胖。该患者接受了腹腔镜袖状胃切除术，残余胃容积为100毫升。手术后一年，干预措施使体重减轻了37.1%，血糖水平得到良好控制。患者还报告整体生活质量、情绪和功能有所改善。对于死亡率较高的肥胖普拉德-威利综合征患者，可以考虑进行腹腔镜袖状胃切除术，特别是因为没有其他有效的替代疗法。
