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亨廷顿病——营养管理的原则与实践

Huntington Disease - principles and practice of nutritional management.

作者信息

Zukiewicz-Sobczak Wioletta, Król Renata, Wróblewska Paula, Piątek Jacek, Gibas-Dorna Magdalena

机构信息

Institute of Rural Health in Lublin, Lublin, Poland.

Higher School of Social Sciences in Lublin, Lublin, Poland.

出版信息

Neurol Neurochir Pol. 2014;48(6):442-8. doi: 10.1016/j.pjnns.2014.10.006. Epub 2014 Nov 7.

DOI:10.1016/j.pjnns.2014.10.006
PMID:25482256
Abstract

Huntington disease (HD) is a degenerative brain disease clinically manifested by the characteristic triad: physical symptoms including involuntary movements and poor coordination, cognitive changes with less ability to organize routine tasks, and some emotional and behavioral disturbances. For patients with HD, feeding is one of the problems they have to face. People with HD often have lower than average body weight and struggle with malnutrition. As a part of therapy, good nutrition is an intervention maintaining health and functional ability for maximally prolonged time. In the early stages of HD, small amounts of blenderized foods given orally are recommended. In more advanced stages, enteral nutrition is essential using gastric, or jejunal tubes for short term. Most severe cases require gastrostomy or gastrojejunostomy. Although enteral feeding is well tolerated by most of the patients, a number of complications may occur, including damage to the nose, pharynx, or esophagus, aspiration pneumonia, sinusitis, metabolic imbalances due to improper nutrient and fluid supply, adverse effects affecting gastrointestinal system, and refeeding syndrome.

摘要

亨廷顿病(HD)是一种退行性脑部疾病,临床表现为典型的三联征:身体症状包括不自主运动和协调性差;认知变化,组织日常任务的能力下降;以及一些情绪和行为障碍。对于HD患者来说,进食是他们必须面对的问题之一。HD患者的体重往往低于平均水平,并与营养不良作斗争。作为治疗的一部分,良好的营养是一种干预措施,可在最长时间内维持健康和功能能力。在HD的早期阶段,建议口服少量经搅拌的食物。在更晚期阶段,短期使用胃管或空肠管进行肠内营养至关重要。最严重的病例需要胃造口术或胃空肠吻合术。尽管大多数患者对肠内喂养耐受性良好,但可能会出现一些并发症,包括鼻、咽或食管损伤、吸入性肺炎、鼻窦炎、由于营养和液体供应不当导致的代谢失衡、影响胃肠道系统的不良反应以及再喂养综合征。

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