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巴西感染新型冠状病毒肺炎住院患者中的亨廷顿舞蹈症:三年期最新情况

Huntington's Disease in Hospitalized Patients Infected with SARS-CoV-2 in Brazil: Three-Year Update.

作者信息

Martins Jéssica Paula, Sciani Juliana Mozer, Marson Fernando Augusto Lima

机构信息

Laboratory of Molecular Biology and Genetics, Laboratory of Clinical and Molecular Microbiology, LunGuardian Research Group - Epidemiology of Respiratory and Infectious Diseases, São Paulo, Brazil.

Multidisciplinary Research Laboratory, São Francisco University, São Paulo, Brazil.

出版信息

Neurodegener Dis. 2025;25(2):88-105. doi: 10.1159/000538170. Epub 2025 Feb 20.

Abstract

INTRODUCTION

Huntington's disease (HD) is considered a rare and fatal neurodegenerative disease; despite that, only one study has described the outcomes among individuals with HD and COVID-19. In this context, an epidemiological investigation was conducted in Brazil using data from Open-Data-SUS (https://opendatasus.saude.gov.br/), a Brazilian open dataset encompassing demographic and clinical information of hospitalized patients.

METHOD

The study comprised 2,180,403 hospitalized patients due to COVID-19 in Brazil from December 29, 2019, to April 6, 2023. From the total original database, patients with HD and three control groups were selected (control 1 [CG-1] - hospitalized patients with COVID-19 and a neurological disorder [except those with HD], control 2 [CG-2] - hospitalized patients with COVID-19 and no comorbidities, and control 3 [CG-3] - hospitalized patients with COVID-19 except for those with HD). The study described the following characteristics of these patients: sex, age, race, place of residence, presence of nosocomial infection, clinical signs, comorbidities, use of antiviral medication for the flu, need for intensive care unit, need for mechanical ventilation support, discharge criteria, and outcome.

RESULTS

A total of 60 patients with HD were enrolled, representing a prevalence of 2.8 per 100,000 inhabitants. Of these, 23 were men, 30 were aged between 25 and 60 years, 38 were White, 58 lived in urban areas, and two had a nosocomial infection. The main clinical signs presented were fever (N = 45), dyspnea (N = 45), peripheral oxygen saturation <95% (N = 45), cough (N = 38), and respiratory discomfort (N = 37). The main comorbidities were cardiopathy (N = 13), asthma (N = 2), and chronic lung disease (N = 2). Four patients received antiviral medication for the flu. During hospitalization, 18 patients required admission to an intensive care unit, 10 used invasive mechanical ventilation, 42 used noninvasive mechanical ventilation, and eight did not use ventilatory support. Regarding outcomes, 36 patients were cured, 23 died due to COVID-19, and 1 died from a cause not associated with COVID-19. In the comparison of markers between patients with HD and the controls, the following significant associations were described: (CG-1) patients with HD were younger and presented fewer clinical signs and comorbidities; (CG-2) patients with HD were commonly female, older, presented fewer clinical signs, and had a higher chance of death (OR = 2.354 [95% CI = 1.395-3.973]); and (CG-3) patients with HD were more commonly female and presented fewer clinical signs and comorbidities.

CONCLUSIONS

Patients with HD can be considered an at-risk population due to the poor clinical outcomes compared to patients without comorbidities. Even then, this group of patients presented fewer clinical signs and comorbidities.

摘要

引言

亨廷顿舞蹈症(HD)被认为是一种罕见的致命性神经退行性疾病;尽管如此,仅有一项研究描述了HD患者和感染新冠病毒(COVID-19)患者的结局。在此背景下,巴西利用来自Open-Data-SUS(https://opendatasus.saude.gov.br/)的数据进行了一项流行病学调查,这是一个包含住院患者人口统计学和临床信息的巴西开放数据集。

方法

该研究纳入了2019年12月29日至2023年4月6日期间因COVID-19在巴西住院的2,180,403名患者。从整个原始数据库中,选取了HD患者和三个对照组(对照组1 [CG-1]——因COVID-19住院且患有神经系统疾病[HD患者除外]的患者,对照组2 [CG-2]——因COVID-19住院且无合并症的患者,以及对照组3 [CG-3]——因COVID-19住院但HD患者除外的患者)。该研究描述了这些患者的以下特征:性别、年龄、种族、居住地点、医院感染情况、临床症状、合并症、使用抗流感病毒药物情况、入住重症监护病房的需求、机械通气支持的需求、出院标准以及结局。

结果

共纳入60例HD患者,患病率为每10万居民中有2.8例。其中,男性23例,30例年龄在25至60岁之间,38例为白人,58例居住在城市地区,2例发生医院感染。主要临床症状为发热(N = 45)、呼吸困难(N = 45)、外周血氧饱和度<95%(N = 45)、咳嗽(N = 38)和呼吸不适(N = 37)。主要合并症为心脏病(N = 13)、哮喘(N = 2)和慢性肺病(N = 2)。4例患者使用了抗流感病毒药物。住院期间,18例患者需要入住重症监护病房,10例使用有创机械通气,42例使用无创机械通气,8例未使用通气支持。关于结局,36例患者治愈,23例因COVID-19死亡,1例死于与COVID-19无关的原因。在HD患者与对照组之间的标志物比较中,发现了以下显著关联:(CG-1)HD患者更年轻,临床症状和合并症更少;(CG-2)HD患者通常为女性,年龄更大,临床症状更少,死亡几率更高(OR = 2.354 [95% CI = 1.395 - 3.973]);(CG-3)HD患者更常见为女性,临床症状和合并症更少。

结论

与无合并症患者相比,HD患者临床结局较差,可被视为高危人群。即便如此,该组患者的临床症状和合并症较少。

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