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

项目摘要 呼吸无力是许多神经肌肉疾病的常见表现，包括 肌萎缩性侧索硬化症（ALS）、重症肌无力、格林-巴利综合征和某些肌肉萎缩性侧索硬化症。 营养不良标准肺功能测试（PFT）受到许多限制。这些措施包括：1. 无法获得伴有球无力患者的准确数据2.对病人的依赖 能够有效合作; 3。无法获得持续评估，尤其是当患者处于 重症监护室; 4.它不能提供对肺部状况的了解，包括肺不张的存在。一 提供一种潜在的更有效的监测肺功能的方法是通过应用 电阻抗断层成像（EIT）。在EIT中，放置嵌入有多个表面电极的带 通过一个非常弱的非电离电流， 在不同的电极组之间，并测量产生的电压。呼吸的实时视频可以 患者在休息时舒适地呼吸，也在完全吸气和呼气时获得。从这些 通过使用图像，可以导出肺功能的简单度量。这项技术已经在关键领域得到了应用。 护理患有各种疾病的患者，并且非常安全，它甚至被用于早产儿，以帮助监测 肺状况和肺成熟。在这份R21建议书中，我们寻求确立以下各项的具体应用： 这项技术在有ALS肺功能障碍风险或患有ALS肺功能障碍的患者中的应用，因为他们代表了 这项技术可能特别有用。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中， 肺功能障碍在具体目标2中，我们将确定EIT值与标准之间的关系 在大约3个月的时间内，横截面和纵向的肺功能参数 的时间，在患者和不伴随球弱。在这项研究结束时，我们希望有 证明了EIT在评估ALS肺功能方面的潜在价值，为其打开了大门。 广泛应用于各种神经肌肉疾病患者的急性和慢性护理。

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.