应用于马来西亚 ALS 登记的多步骤模型。

David Paul Capelle, Wafa Sabirin, Nurul Angelyn Zulhairy-Liong, Suzanna Edgar, Khean-Jin Goh, Azlina Ahmad-Annuar, Nortina Shahrizaila
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引用次数: 0

摘要

目的:应用肌萎缩性脊髓侧索硬化症(ALS)发病机制的多步骤模型:将肌萎缩性脊髓侧索硬化症(ALS)发病机制的多步骤模型应用于马来西亚多种族登记数据:从 2016 年到 2024 年,我们收集了 289 名到我们多学科诊所就诊的患者的临床数据,包括发病年龄。根据近似发病率的对数和年龄的对数构建了最小二乘法线性回归模型。人口发病率的近似值是根据相应年龄组的总人口规模调整 5 年组中患者的绝对人数得出的:结果:发病率对数与年龄对数之间呈线性关系,斜率为 4.57(95% CI,3.3-5.8),r2 值为 0.93,表明有 6 个步骤:结论:马来西亚肌萎缩性脊髓侧索硬化症患者的发病过程似乎与其他队列中观察到的多步骤疾病模型一致。
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Multistep modeling applied to a Malaysian ALS registry.

Objective: To apply the multistep model of pathogenesis in amyotrophic lateral sclerosis (ALS) to data from a multiethnic Malaysian registry.

Methods: Clinical data, including age at symptom onset, was collected from 289 patients who presented to our multidisciplinary clinic from 2016 until 2024. A least squares linear regression model was constructed from the logarithm of approximated incidence and the logarithm of age. Population incidence was approximated by adjusting the absolute numbers of patients in 5 year groups by the size of the general population in the respective age group.

Results: A linear relationship between log of incidence versus log of age was observed, with a slope of 4.57 (95% CI, 3.3-5.8) and an r2 value of 0.93, suggesting a 6-step process.

Conclusion: Progression toward symptom onset in Malaysian ALS patients appears consistent with a multistep model of disease as observed in other cohorts.

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Estimating the minimum important difference in the ALSFRS-R-instrument in people living with MND. Quantitative susceptibility mapping in amyotrophic lateral sclerosis: automatic quantification of the magnetic susceptibility in the subcortical nuclei. Treatment continuity of amyotrophic lateral sclerosis with available riluzole formulations: state of the art and current challenges in a 'real-world' setting. Occupational lead exposure and amyotrophic lateral sclerosis survival in the Danish National Patient Registry. Jean Marie Baptiste Vianney de Jong (1937-2024).
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