捷克共和国重症肌无力的负担：全国患者登记的分析。

IF 3.9 3区医学 Q1 CLINICAL NEUROLOGY Neurology and Therapy Pub Date : 2025-02-01 Epub Date: 2024-12-04 DOI:10.1007/s40120-024-00682-x

Stanislav Voháňka, Aleš Tichopád, Magda Horáková, Jana Junkerová, Michala Jakubíková, Jiří Piťha, Michaela Týblová, Daniela Vlažná, Katarína Breciková, Jacek Cudny, Petr Hájek

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引用次数: 0

摘要

本研究的主要目的是描述捷克MG患者的人口统计学、疾病特征、管理方法和治疗趋势。方法：我们选择了2015年8月24日至2021年11月19日期间在捷克MyReg注册中心登记的所有患者，包括发病和流行患者。对于描述性分析，所有入组的患者，无论其诊断日期或入组日期，均被纳入。我们分析了以下疾病相关终点：重症肌无力复合（MGC）评分、用力肺活量（FVC）和美国重症肌无力基金会（MGFA）临床分型。结果：发病率从0.62上升到3.13，呈持续上升趋势。平均MGC评分为5.0（中位数4.0,95% CI 4.7, 5.3），代表轻度MG。有肌无力危像和无肌无力危像患者的FVC与预测值的差异分别为58.93% （95% CI 37.27, 80.59）和75.93% （95% CI 74.87, 77.00）。我们发现70例难治性MG患者（5.0%），其中58.6%为女性。难治性和非难治性疾病的MGFA分类如下：IIa 21.8% vs 23.2%, IIb 45.3% vs 33.6%, IIIb 14.1% vs 4.6%。结论：我们的分析表明，MG在捷克共和国的发病率正在增加，难治性疾病患者（其中高达58%为女性）的疾病负担高于非难治性患者。

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Burden of Myasthenia Gravis in the Czech Republic: Analysis of the Nationwide Patient Registry.

Introduction: The main goal of this study was to describe the Czech population of patients with MG in terms of demographics, disease characteristics, management approaches, and treatment trends.

Methods: We selected all patients, both incident and prevalent, who were enrolled in the Czech MyReg registry between August 24, 2015 and November 19, 2021. For the descriptive analysis, all patients enrolled in the registry, regardless of their date of diagnosis or date of enrolment, were included. We analyzed the following disease-related endpoints: myasthenia gravis composite (MGC) score, forced vital capacity (FVC), and Myasthenia Gravis Foundation of America (MGFA) clinical classification.

Results: The incidence showed a consistent increasing trend from 0.62 to 3.13. The mean MGC score was 5.0 (median 4.0, 95% CI 4.7, 5.3) representing mild form of MG. The difference in FVC from the predicted value in patients during and without myasthenic crisis was 58.93% (95% CI 37.27, 80.59) and 75.93% (95% CI 74.87, 77.00), respectively. We identified 70 patients (5.0%) with refractory MG, of whom 58.6% were female. The MGFA classifications in those with refractory vs. non-refractory disease was as follows: IIa 21.8% vs 23.2%, IIb 45.3% vs 33.6%, and IIIb 14.1% vs 4.6%, respectively.

Conclusion: Our analysis shows that the incidence of MG is increasing in the Czech Republic and that patients with refractory disease, of whom up to 58% are female, have a higher burden of disease than non-refractory patients.

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来源期刊

Neurology and Therapy CLINICAL NEUROLOGY-

CiteScore

5.40

自引率

8.10%

发文量

103

审稿时长

6 weeks

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