University of Cambridge School of Clinical Medicine, Box 111, Cambridge Biomedical Campus, Hills Road, Cambridge CB2 0SP, UK.
Medical Library, University of Cambridge School of Clinical Medicine, Box 111, Cambridge Biomedical Campus, Hills Road, Cambridge CB2 0SP, UK.
Int J Law Psychiatry. 2021 Sep-Oct;78:101733. doi: 10.1016/j.ijlp.2021.101733. Epub 2021 Sep 1.
Prader-Willi Syndrome (PWS) is a complex multisystem genetic disorder associated with several challenges for people with PWS themselves, and for their families and care givers. Support around access to food is a particular issue due to impaired satiety and, because of this, people with PWS eat excessive amounts of food (hyperphagia). Together with other aspects of the PWS phenotype including, in many cases, a reduced sensitivity to pain, hyperphagia results in life-threatening obesity and life-shortening complications for some people with PWS. Restrictions to liberty and/or access to food raise important legal and ethical considerations in the clinical management of children and adults with PWS. Particularly where disagreements arise and, in the absence of comprehensive guidance for care providers, the courts may be called upon to resolve these difficult issues.
Westlaw, Westlaw AU, and Lexis Nexis were searched for case law concerning the treatment or support of a person with PWS.
Fifteen cases from jurisdictions in Australia, New Zealand, Canada, the United Kingdom and the United States of America met inclusion criteria. Areas requiring judicial decision making included a) detention in psychiatric hospital; b) support in least restrictive environments c) eligibility for support services; d) guardianship; e) access to special education. Judicial decisions are discussed in the context of the United Nations' Convention on the Rights of Persons with Disabilities.
普拉德-威利综合征（PWS）是一种复杂的多系统遗传疾病，给 PWS 患者及其家属和护理人员带来了多方面的挑战。由于饱腹感受损，获取食物方面的支持是一个特别的问题，因此 PWS 患者会摄入过量的食物（暴食症）。与 PWS 表型的其他方面（包括在许多情况下对疼痛的敏感性降低）一起，暴食症导致危及生命的肥胖症和一些 PWS 患者的寿命缩短并发症。对自由和/或食物获取的限制在 PWS 儿童和成人的临床管理中引发了重要的法律和伦理问题。特别是在出现分歧且缺乏全面的护理提供者指南的情况下，法院可能会被要求解决这些棘手的问题。
1）审查来自英语国家普通法司法管辖区的有关 PWS 患者支持安排的案例法，以确定需要法院干预的问题。2）确定与已确定问题相关的临床指南的原则，确保这些指南符合道德和人权要求。
在 Westlaw、Westlaw AU 和 Lexis Nexis 上搜索有关 PWS 患者治疗或支持的案例法。
从澳大利亚、新西兰、加拿大、英国和美国的司法管辖区中找到了符合纳入标准的 15 个案例。需要司法决策的领域包括 a）在精神病院拘留；b）在限制最少的环境中提供支持；c）有资格获得支持服务；d）监护权；e）获得特殊教育。在《联合国残疾人权利公约》的背景下讨论了司法判决。
