关键词: Huntington's disease autonomic cardiovascular gastric intestinal respiratory sexual sweating thermoregulatory urinary

Mesh : Huntington Disease / physiopathology genetics Humans Autonomic Nervous System Diseases / physiopathology etiology Cardiovascular Diseases / epidemiology Gastrointestinal Diseases / etiology physiopathology

来  源:   DOI:10.1002/mdc3.14029   PDF(Pubmed)

Abstract:
BACKGROUND: Although Huntington\'s disease (HD) is usually thought of as a triad of motor, cognitive, and psychiatric symptoms, there is growing appreciation of HD as a systemic illness affecting the entire body.
OBJECTIVE: This review aims to draw attention to these systemic non-motor symptoms in HD.
METHODS: We identified relevant studies published in English by searching MEDLINE (from 1966 to September 2023), using the following subject headings: Huntington disease, autonomic, systemic, cardiovascular, respiratory, gastrointestinal, urinary, sexual and cutaneous, and additional specific symptoms.
RESULTS: Data from 123 articles were critically reviewed with focus on systemic features associated with HD, such as cardiovascular, respiratory, gastrointestinal, urinary, sexual and sweating.
CONCLUSIONS: This systematic review draws attention to a variety of systemic and autonomic co-morbidities in patients with HD. Not all of them correlate with the severity of the primary HD symptoms or CAG repeats. More research is needed to better understand the pathophysiology and treatment of systemic and autonomic dysfunction in HD.
摘要:
背景:尽管亨廷顿病(HD)通常被认为是运动的三联症,认知,和精神症状,人们越来越认识到HD是一种影响整个身体的系统性疾病。
目的:这篇综述旨在引起人们对HD患者这些系统性非运动症状的关注。
方法:我们通过搜索MEDLINE(从1966年到2023年9月)确定了以英文发表的相关研究,使用以下主题词:亨廷顿病,自主性,系统性,心血管,呼吸,胃肠,泌尿,性和皮肤,和其他特定症状。
结果:对123篇文章的数据进行了严格审查,重点关注与HD相关的系统特征,比如心血管,呼吸,胃肠,泌尿,性和出汗
结论:本系统综述提请注意HD患者的各种系统性和自主神经并发症。并非所有这些都与原发性HD症状或CAG重复的严重程度相关。需要更多的研究来更好地了解HD的全身和自主神经功能障碍的病理生理学和治疗。
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