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关于 Phelan-McDermid 综合征中精神健康问题的共识建议。

Consensus recommendations on mental health issues in Phelan-McDermid syndrome.

机构信息

Jonx, Department of (Youth) Mental Health and Autism, Lentis Psychiatric Institute, Groningen, the Netherlands; Rob Giel Research Centre, Department of Psychiatry, University Medical Center Groningen, Groningen, the Netherlands.

Department of Child and Adolescent Psychiatry, Institute of Psychiatry and Mental Health, Hospital General Universitario Gregorio Marañón, IiSGM, Instituto de Investigación Sanitaria Gregorio Marañón, Madrid, Spain; School of Psychology, Universidad Autónoma, Madrid, Spain.

出版信息

Eur J Med Genet. 2023 Jun;66(6):104770. doi: 10.1016/j.ejmg.2023.104770. Epub 2023 Apr 20.

Abstract

Phelan-McDermid syndrome is a rare genetic condition caused by a deletion encompassing the 22q13.3 region or a pathogenic variant of the gene SHANK3. The clinical presentation is variable, but main characteristics include global developmental delay/intellectual disability (ID), marked speech impairment or delay, along with other features like hypotonia and somatic or psychiatric comorbidities. This publication delineates mental health, developmental and behavioural themes across the lifetime of individuals with PMS as informed by parents/caregivers, experts, and other key professionals involved in PMS care. We put forward several recommendations based on the available literature concerning mental health and behaviour in PMS. Additionally, this article aims to improve our awareness of the importance of considering developmental level of the individual with PMS when assessing mental health and behavioural issues. Understanding how the discrepancy between developmental level and chronological age may impact concerning behaviours offers insight into the meaning of those behaviours and informs care for individuals with PMS, enabling clinicians to address unmet (mental health) care needs and improve quality of life.

摘要

佩兰-麦克德米德综合征是一种罕见的遗传疾病,由包含 22q13.3 区域的缺失或 SHANK3 基因的致病性变异引起。临床表现多样,但主要特征包括全面发育迟缓/智力残疾 (ID)、明显的言语障碍或延迟,以及低张力和躯体或精神合并症等其他特征。本出版物通过家长/照顾者、专家和其他参与 PMS 护理的关键专业人员,描绘了 PMS 个体一生中的心理健康、发育和行为主题。我们根据有关 PMS 中的心理健康和行为的现有文献提出了一些建议。此外,本文旨在提高我们对在评估心理健康和行为问题时考虑 PMS 个体发育水平重要性的认识。了解发育水平和年龄之间的差异如何影响相关行为,可以深入了解这些行为的意义,并为 PMS 个体提供护理,使临床医生能够解决未满足的(心理健康)护理需求并提高生活质量。

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