Electrical impedance tomography for the improved assessment of pulmonary function in neuromuscular disease

项目总结 呼吸无力是许多神经肌肉疾病的常见表现，包括 肌萎缩侧索硬化症、重症肌无力、格林-巴利综合征和某些肌肉疾病 营养不良。标准的肺功能测试(PFT)有许多局限性。这些措施包括：1. 无法获得伴随球软弱患者的准确数据2.依赖于患者 能够有效地合作；3.无法获得持续的评估，特别是当患者处于 重症监护病房；4。它不能提供对肺部状况的洞察，包括肺不张的存在。一 一种为监测肺功能提供潜在更有效手段的方法是通过应用 电阻抗断层扫描(EIT)。在EIT中，放置了嵌入多个表面电极的皮带 在胸部周围，通过通过非常微弱的非电离电流获得低分辨率图像 在不同的电极组之间进行测量，并测量产生的电压。实时的呼吸视频可以 在患者休息、呼吸舒适、充分吸气和呼气时获得。从这些 图像，可以得出肺功能的简单指标。这项技术已经在关键的 护理患有各种疾病的患者是非常安全的，它甚至被用来帮助监测早产儿 肺部状况和肺成熟。在这份R21提案中，我们寻求建立以下具体应用 这项技术适用于ALS有肺功能障碍风险或有ALS患者，因为他们代表了 这项技术可能对他们特别有用。在具体目标1中，我们计划评估该技术的可靠性 在ALS患者和年龄和性别匹配的健康对照组中，并确定其相对敏感性 肺功能障碍。在具体目标2中，我们将确定企业所得税价值与标准之间的关系 大约3个月的横断面和纵向肺功能参数 在有和没有伴随的球无力的患者中，时间的长短。在这项研究结束时，我们希望有 展示了EIT在ALS肺功能评估中的潜在价值，打开了EIT的大门 广泛应用于各种神经肌肉疾病患者的急慢性护理。

PROJECT SUMMARY Respiratory weakness is a common manifestation of many neuromuscular diseases, including amyotrophic lateral sclerosis (ALS), myasthenia gravis, Guillain-Barre syndrome, and certain muscular dystrophies. Standard pulmonary function tests (PFTs) suffer from a number of limitations. These include: 1. inability to obtain accurate data in patients with concomitant bulbar weakness 2. Reliance on the patient being able to cooperate effectively; 3. Inability to obtain continuous assessment, especially when the patient is in the intensive care unit; 4. It provides no insight into pulmonary condition, including the presence of atelectasis. One approach that offers a potential more effective means for monitoring pulmonary function is via the application of electrical impedance tomography (EIT). In EIT, a belt embedded with multiple surface electrodes is placed around the chest and a low-resolution image is obtained by passing a very weak, non-ionizing electrical current between varying sets of electrodes and measuring the resulting voltages. Real-time videos of breathing can be obtained with the patient at rest breathing comfortably and also with full inspiration and expiration. From these images, simple metrics of pulmonary function can be derived. The technology has already found use in critical care patients with a variety of disorders and is very safe, it is even being used in premature infants to help monitor pulmonary condition and lung maturation. In this R21 proposal, we seek to establish the specific application of this technology in patients at risk for or with pulmonary dysfunction with ALS since they represent a group in whom the technology may be especially useful. In specific aim 1, we plan to assess the reliability of the technique in both ALS patients and age- and sex-matched healthy controls and to determine its relative sensitivity to pulmonary dysfunction. In specific aim 2, we will determine the relationship between EIT values and standard pulmonary function parameters both cross-sectionally and longitudinally over an approximately 3-month period of time, in patients both with and without concomitant bulbar weakness. At the end of this study we hope to have demonstrated the potential value for EIT the assessment of pulmonary function in ALS, opening the door to its wide application in the acute and chronic care of patients across a range of neuromuscular diseases.