关键词: Enteral nutrition aspiration congenital dysphagia inclusion body malnutrition muscular dystrophies myopathies myositis pneumonia structural weight loss

Mesh : Humans Adult Enteral Nutrition / adverse effects Deglutition Disorders / therapy complications Quality of Life Muscular Dystrophy, Facioscapulohumeral Retrospective Studies Malnutrition / etiology prevention & control Muscular Diseases / complications

来  源:   DOI:10.3233/JND-230014   PDF(Pubmed)

Abstract:
BACKGROUND: Eating an adequate diet and maintaining a healthy body weight can be challenging for patients with muscular disorders (MD). Starting tube feeding can have a positive impact on nutritional status, functioning and quality of life. Guidelines on when to start tube feeding in adults with MD are lacking.
OBJECTIVE: We aim to review the scientific literature on indications to start tube feeding in adults with facioscapulohumeral dystrophy (FSHD), inclusion body myositis (IBM), muscular dystrophy type 1 (DM1), oculopharyngeal muscular dystrophy (OPMD) and congenital myopathies.
METHODS: This scoping review was conducted according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses extension for scoping reviews (PRISMA-ScR) guidelines. Relevant studies were identified in Pubmed, Embase and Cinahl (April 2022). The medical subject headings (MeSH) and text words used were related to FSHD, IBM, DM1, OPMD or congenital myopathies and dysphagia, enteral nutrition or malnutrition.
RESULTS: Of 1046 unique articles, 9 case reports and 2 retrospective case series were included. Indications to start tube feeding were dysphagia, malnutrition/weight loss and respiratory infections (due to aspiration). Percutaneous endoscopic gastrostomy (PEG) tubes were used most often and complications were respiratory failure, problems with the tube itself, accidental tube removal, cutaneous symptoms, digestive symptoms, and peritonitis.
CONCLUSIONS: Data on tube feeding in MD is scarce. Indications to start tube feeding were similar across the various MD. We call for more research in this field and suggest to include screening for dysphagia, aspiration and malnutrition in for the treatment of various MD.
摘要:
背景:对于患有肌肉疾病(MD)的患者来说,吃足够的饮食和保持健康的体重可能是具有挑战性的。开始管饲可以对营养状况产生积极影响,功能和生活质量。缺乏关于何时开始成人MD管饲的指南。
目的:我们的目的是回顾有关成人面肩肱骨营养不良(FSHD)开始管饲适应症的科学文献,包涵体肌炎(IBM),肌营养不良1型(DM1),眼咽肌营养不良(OPMD)和先天性肌病。
方法:此范围审查是根据系统审查的首选报告项目和范围审查的荟萃分析扩展(PRISMA-ScR)指南进行的。相关研究在Pubmed,Embase和Cinahl(2022年4月)。使用的医学主题词(MeSH)和文本词与FSHD相关,IBM,DM1,OPMD或先天性肌病和吞咽困难,肠内营养或营养不良。
结果:在1046篇独特文章中,包括9例病例报告和2例回顾性病例系列。开始管饲的适应症是吞咽困难,营养不良/体重减轻和呼吸道感染(由于误吸)。经皮内镜胃造瘘术(PEG)最常用,并发症为呼吸衰竭,管子本身有问题,意外拆卸管,皮肤症状,消化症状,和腹膜炎.
结论:关于MD管饲的数据很少。在各种MD中,开始管饲的指示相似。我们呼吁在这一领域进行更多的研究,并建议包括筛查吞咽困难,用于治疗各种MD的误吸和营养不良。
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