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臀肌群组磁共振成像生物标志物与杜氏肌营养不良症患者功能能力的纵向变化:一项为期 12 个月的队列研究。

Longitudinal changes in magnetic resonance imaging biomarkers of the gluteal muscle groups and functional ability in Duchenne muscular dystrophy: a 12-month cohort study.

机构信息

Department of Radiology, Key Laboratory of Obstetric and Gynecologic and Pediatric Diseases and Birth Defects of Ministry of Education, West China Second University Hospital, Sichuan University, Chengdu, 610041, China.

Department of Rehabilitation Medicine, Key Laboratory of Obstetric and Gynecologic and Pediatric Diseases and Birth Defects of Ministry of Education, West China Second University Hospital, Sichuan University, Chengdu, 610041, China.

出版信息

Pediatr Radiol. 2023 Dec;53(13):2672-2682. doi: 10.1007/s00247-023-05791-7. Epub 2023 Oct 27.

Abstract

BACKGROUND

Quantitative magnetic resonance imaging (MRI) is considered an objective biomarker of Duchenne muscular dystrophy (DMD), but the longitudinal progression of MRI biomarkers in gluteal muscle groups and their predictive value for future motor function have not been described.

OBJECTIVE

To explore MRI biomarkers of the gluteal muscle groups as predictors of motor function decline in DMD by characterizing the progression over 12 months.

MATERIALS AND METHODS

A total of 112 participants with DMD were enrolled and underwent MRI examination of the gluteal muscles to determine fat fraction and longitudinal relaxation time (T1). Investigations were based on gluteal muscle groups including flexors, extensors, adductors, and abductors. The North Star Ambulatory Assessment and timed functional tests were performed. All participants returned for follow-up at an average of 12 months and were divided into two subgroups (functional stability/decline groups) based on changes in timed functional tests. Univariable and multivariable logistic regression methods were used to explore the risk factors associated with future motor function decline.

RESULTS

For the functional decline group, all T1 values decreased, while fat fraction values increased significantly over 12 months (P<0.05). For the functional stability group, only the fat fraction of the flexors and abductors increased significantly over 12 months (P<0.05). The baseline T1 value was positively correlated with North Star Ambulatory Assessment and negatively correlated with timed functional tests at the 12-month follow-up (P<0.001), while the baseline fat fraction value was negatively correlated with North Star Ambulatory Assessment and positively correlated with timed functional tests at the 12-month follow-up (P<0.001). Multivariate regression showed that increased fat fraction of the abductors was associated with future motor function decline (model 1: odds ratio [OR]=1.104, 95% confidence interval [CI]: 1.0261.187, P=0.008; model 2: OR=1.085, 95% CI: 1.0131.161, P=0.019), with an area under the curve of 0.874.

CONCLUSION

Fat fraction of the abductors is a powerful predictor of future motor functional decline in DMD patients at 12 months, underscoring the importance of focusing early on this parameter in patients with DMD.

摘要

背景

定量磁共振成像(MRI)被认为是杜氏肌营养不良症(DMD)的客观生物标志物,但尚未描述臀肌群组的 MRI 生物标志物的纵向进展及其对未来运动功能的预测价值。

目的

通过 12 个月的特征描述,探讨臀肌群组的 MRI 生物标志物作为 DMD 运动功能下降的预测指标。

材料与方法

共纳入 112 例 DMD 患者,进行臀肌 MRI 检查以确定脂肪分数和纵向弛豫时间(T1)。研究基于包括臀肌屈肌、伸肌、内收肌和外展肌在内的臀肌群组进行。进行了北美之星动态评估和定时功能测试。所有参与者平均 12 个月后返回进行随访,并根据定时功能测试的变化分为两个亚组(功能稳定/下降组)。使用单变量和多变量逻辑回归方法探讨与未来运动功能下降相关的危险因素。

结果

对于功能下降组,所有 T1 值在 12 个月内均降低,而脂肪分数值显著升高(P<0.05)。对于功能稳定组,仅在 12 个月时,屈肌和外展肌的脂肪分数显著增加(P<0.05)。基线 T1 值与北美之星动态评估呈正相关,与 12 个月时的定时功能测试呈负相关(P<0.001),而基线脂肪分数值与北美之星动态评估呈负相关,与 12 个月时的定时功能测试呈正相关(P<0.001)。多变量回归显示,外展肌的脂肪分数增加与未来的运动功能下降相关(模型 1:比值比[OR]=1.104,95%置信区间[CI]:1.0261.187,P=0.008;模型 2:OR=1.085,95% CI:1.0131.161,P=0.019),曲线下面积为 0.874。

结论

外展肌的脂肪分数是 DMD 患者 12 个月时未来运动功能下降的有力预测指标,这突显了在 DMD 患者中早期关注该参数的重要性。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/755d/10697878/2c9aa2ac6db5/247_2023_5791_Fig1_HTML.jpg

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