Ultrasonography-Based Qualitative and Quantitative Evaluation Approaches for Pompe Disease

Author:

Lee Yueh-Hui,Chiou Hong-Jen,Bau Da-Tian,Niu Dau-Ming,Hsu Ting-Rong,Huang Hsuan-En,Shih Tzu-ChingORCID

Abstract

Abstract Purpose The aim of this study was to propose the qualitative and quantitative approaches to evaluate the skeletal muscle ultrasound images of 23 Pompe disease (i.e., acid maltase deficiency, AMD) patients and 14 normal subjects. Methods A cohort of 23 AMD patients and 14 normal subjects has been investigated. We compared the B-mode echo intensity of the rectus femoris muscle with that of its surrounding fat (subcutaneous fat) and proposed a qualitative grading method. Quantitative analysis of the region of interest (ROI) with the echo intensity and the segmented area was also performed. Results Qualitative results showed that AMD patients without clinical symptoms (without undergoing ERT) had the highest distribution of Grade 1, and AMD patients undergoing ERT had the widest distribution of Grade 2, and control group (n = 14) with the highest distribution of Grade 1. Using the segmented area approach, quantitative results showed that AMD patients undergoing ERT had the largest and widest distribution. Meanwhile the control subjects (normal subjects) had the lowest and the narrowest areas. The echo intensity of the segmented ROI of AMD patients undergoing ERT displayed the highest and widest (inhomogeneous) distributions. By contrast, the echo intensity of AMD patients without clinical symptoms was slightly increased and with low inhomogeneity. Conclusion The proposed ultrasonography-based qualitative and quantitative approach may be used to evaluate the severity of muscle destruction for AMD patients. Besides, the quantitative segmented area with regression analysis could help predict the incidence of onset of Pompe disease patients.

Funder

Ministry of Science and Technology, Taiwan

Publisher

Springer Science and Business Media LLC

Subject

Biomedical Engineering,General Medicine

Cited by 2 articles. 订阅此论文施引文献 订阅此论文施引文献,注册后可以免费订阅5篇论文的施引文献,订阅后可以查看论文全部施引文献

同舟云学术

1.学者识别学者识别

2.学术分析学术分析

3.人才评估人才评估

"同舟云学术"是以全球学者为主线,采集、加工和组织学术论文而形成的新型学术文献查询和分析系统,可以对全球学者进行文献检索和人才价值评估。用户可以通过关注某些学科领域的顶尖人物而持续追踪该领域的学科进展和研究前沿。经过近期的数据扩容,当前同舟云学术共收录了国内外主流学术期刊6万余种,收集的期刊论文及会议论文总量共计约1.5亿篇,并以每天添加12000余篇中外论文的速度递增。我们也可以为用户提供个性化、定制化的学者数据。欢迎来电咨询!咨询电话:010-8811{复制后删除}0370

www.globalauthorid.com

TOP

Copyright © 2019-2024 北京同舟云网络信息技术有限公司
京公网安备11010802033243号  京ICP备18003416号-3