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普拉德-威利综合征患者的减重手术

Bariatric Surgery in Patients With Prader-Willi Syndrome.

作者信息

Dressler Maximilian Herbert, Choi Jong-Ho, Park Kyoyoung, Kong Seong-Ho, Park Do Joong, Lee Hyuk-Joon

机构信息

Division of Gastrointestinal Surgery, Department of Surgery, Seoul National University Hospital, Seoul, Korea.

Medical Faculty Mannheim, Heidelberg University, Mannheim, Germany.

出版信息

J Metab Bariatr Surg. 2025 Aug;14(2):85-96. doi: 10.17476/jmbs.2025.14.2.85. Epub 2025 Aug 12.

DOI:10.17476/jmbs.2025.14.2.85
PMID:40917201
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC12411141/
Abstract

Prader-Willi Syndrome (PWS) is a genetic disorder characterized by insatiable hyperphagia, resulting in severe, early-onset obesity that is often refractory to conventional management. The associated comorbidities and reduced life expectancy in PWS present a significant therapeutic challenge. This review synthesizes the existing literature on the controversial role, outcomes, and complexities of bariatric surgery in patients with PWS. In recent decades, bariatric surgical techniques have evolved from malabsorptive or restrictive operations to modern procedures such as sleeve gastrectomy (SG) and Roux-en-Y gastric bypass (RYGB). We present a 21-year-old male's SG case to illustrate key themes. Published series from specialized, high-volume centers demonstrate that SG and RYGB yield significant short- to mid-term weight loss and metabolic improvement with an acceptable perioperative risk profile. However, the fundamental challenge in PWS remains the persistent, inherent hyperphagia. As exemplified by the presented case and supported by the broader literature, this insatiable hunger severely limits long-term surgical success and frequently leads to substantial weight regain. Maintaining durable success requires rigorous, lifelong external dietary supervision and intensive multidisciplinary support. Moreover, ethical considerations, particularly regarding informed consent and procedural choice, complicate the application of bariatric surgery in this population. Bariatric surgery should therefore be considered an adjunctive tool rather than a definitive cure for PWS-related obesity. Its use must be reserved for carefully selected individuals who have severe comorbidities and robust, long-term support structures in place. This perspective highlights the critical need for ongoing research into optimal patient selection, procedural approaches, and adjuvant therapies to improve durable outcomes.

摘要

普拉德-威利综合征(PWS)是一种遗传性疾病,其特征为无法满足的食欲亢进,导致严重的早发性肥胖,且这种肥胖通常对传统治疗方法难治。PWS相关的合并症和预期寿命缩短带来了重大的治疗挑战。本综述综合了关于减肥手术在PWS患者中的争议性作用、结果和复杂性的现有文献。近几十年来,减肥手术技术已从吸收不良或限制性手术发展为现代手术,如袖状胃切除术(SG)和 Roux-en-Y 胃旁路术(RYGB)。我们呈现一名21岁男性的SG病例以阐述关键主题。来自专业、高容量中心的已发表系列研究表明,SG和RYGB在围手术期风险可接受的情况下,能带来显著的短期至中期体重减轻和代谢改善。然而,PWS的根本挑战仍然是持续存在的内在食欲亢进。如所呈现的病例所示并得到更广泛文献的支持,这种无法满足的饥饿严重限制了手术的长期成功,并经常导致大量体重反弹。维持持久的成功需要严格的、终身的外部饮食监督和强化的多学科支持。此外,伦理考量,特别是关于知情同意和手术选择的问题,使减肥手术在该人群中的应用变得复杂。因此,减肥手术应被视为一种辅助工具,而非治疗PWS相关肥胖的决定性方法。其使用必须仅限于经过精心挑选、患有严重合并症且具备强大长期支持结构的个体。这一观点凸显了持续研究最佳患者选择、手术方法和辅助治疗以改善持久疗效的迫切需求。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d0db/12411141/d71a0323a8d7/jmbs-14-85-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d0db/12411141/3f00f6fa4022/jmbs-14-85-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d0db/12411141/d71a0323a8d7/jmbs-14-85-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d0db/12411141/3f00f6fa4022/jmbs-14-85-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d0db/12411141/d71a0323a8d7/jmbs-14-85-g002.jpg

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本文引用的文献

1
Anesthesia management for patients with Prader-Willi syndrome undergoing bariatric surgery: a single-center retrospective case series study.普拉德-威利综合征患者接受减重手术的麻醉管理:一项单中心回顾性病例系列研究。
BMC Anesthesiol. 2025 Apr 17;25(1):188. doi: 10.1186/s12871-025-03013-1.
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Prader-Willi Syndrome and Weight Gain Control: From Prevention to Surgery-A Narrative Review.普拉德-威利综合征与体重控制:从预防到手术——一篇叙述性综述
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Metabolic and bariatric surgery for obesity in Prader Willi syndrome: systematic review and meta-analysis.
普拉德-威利综合征肥胖症的代谢与减重手术:系统评价与荟萃分析
Surg Obes Relat Dis. 2023 Aug;19(8):907-915. doi: 10.1016/j.soard.2023.01.017. Epub 2023 Feb 1.
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Clinical Trials in Prader-Willi Syndrome: A Review.普拉德-威利综合征的临床试验:综述。
Int J Mol Sci. 2023 Jan 21;24(3):2150. doi: 10.3390/ijms24032150.
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Prader-Willi Syndrome in Adults: An Update On Nutritional Treatment and Pharmacological Approach.成人普拉德-威利综合征:营养治疗和药物治疗方法的最新进展。
Curr Obes Rep. 2022 Dec;11(4):263-276. doi: 10.1007/s13679-022-00478-w. Epub 2022 Sep 5.
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Effects of Restrictive Bariatric Surgery on Congenital Prader-Willi Syndrome: A Case Report.限制性减肥手术对先天性普拉德-威利综合征的影响:一例报告
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Patients with Prader-Willi Syndrome (PWS) Underwent Bariatric Surgery Benefit more from High-Intensity Home Care.患有普拉德-威利综合征(PWS)的患者接受减重手术,从高强度家庭护理中获益更多。
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Metabolic and Bariatric Surgery in Adolescents: For Whom, When, and How?青少年代谢和减重手术:适合哪些人、何时以及如何进行?
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