Conde Bebiana, Martins Natália, Rodrigues Inês, Pimenta Ana Claúdia, Winck João Carlos
Pulmonology Department, Centro Hospitalar Trás-os-Montes e Alto Douro, Avenida da Noruega, Vila Real 5000-508, Portugal.
Faculty of Medicine, University of Porto, Alameda Prof. Hernâni Monteiro, Porto 4200-319, Portugal.
J Clin Med. 2018 Oct 14;7(10):352. doi: 10.3390/jcm7100352.
(1) Background: Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative condition, whose bulbar involvement compromises language, swallowing, and airway protection. When oral nutrition is no longer adequate, percutaneous endoscopic gastroscopy (PEG) may be indicated. However, as exact timing is still debatable, we tried to find it. (2) Methods: A prospective cohort study was performed using fiber-optic endoscopic evaluation of swallowing (FEES), functional evaluation scales (ALS Functional Rating Scale-Revised (ALSFRS-R) and bulbar sub-score (ALSFRS-R-B)), lung function tests (like Forced Vital Capacity (FVC), Cough Peak Flow (CPF)) and anthropometric data. (3) Results: Twenty-three patients were enrolled (mean 65.4 ± 9.1 years, 60.9% males), 12 with spinal-onset. During the study period, 58 FEES were performed (1⁻4/patients). Even before formal the PEG indication, suggestions were given to correct the alterations found. PEG was placed in 12 patients, on average 21.8 months after diagnosis (FVC = 69.9% ± 26.7%, ALSFRS-R-B = 7.7 ± 3.7, ALSFRS-R = 28.9 ± 12.3), and being 91.7% under ventilatory support. ALSFRS-R-B, CPF, FVC, and ALSFRS-R showed significant discriminant ability for PEG placement. Sensitivity and specificity were, respectively, ALSFRS-R-B ≤ 8 (100/90.9), CPF ≤ 205 (83.3), FVC ≤ 74 (83.3/74.2), and ALSFRS-R < 29 (83.3/65.1). (4) Conclusions: FEES provide additional information beyond formal PEG indication. ALSFRS-R-B score ≤ 8 was found as a best functional and noninvasive indicator for PEG performance in ALS patients.
(1) 背景：肌萎缩侧索硬化症（ALS）是一种进行性神经退行性疾病，其延髓受累会影响语言、吞咽及气道保护功能。当经口营养摄入不再充足时，可能需要行经皮内镜下胃造瘘术（PEG）。然而，由于确切的时机仍存在争议，我们试图找出答案。(2) 方法：进行一项前瞻性队列研究，采用纤维内镜吞咽功能评估（FEES）、功能评估量表（修订的ALS功能评定量表（ALSFRS-R）及延髓亚评分（ALSFRS-R-B））、肺功能测试（如用力肺活量（FVC）、咳嗽峰值流速（CPF））及人体测量数据。(3) 结果：共纳入23例患者（平均年龄65.4±9.1岁，男性占60.9%），其中12例为脊髓起病型。在研究期间，共进行了58次FEES检查（每位患者1至4次）。甚至在正式符合PEG指征之前，就针对所发现的异常情况给出了纠正建议。12例患者接受了PEG置管，平均在诊断后21.8个月（FVC = 69.9%±26.7%，ALSFRS-R-B = 7.7±3.7，ALSFRS-R = 28.9±12.3），且91.7%的患者接受通气支持。ALSFRS-R-B、CPF、FVC及ALSFRS-R对PEG置管显示出显著的判别能力。敏感性和特异性分别为：ALSFRS-R-B≤8（100/90.9）、CPF≤205（83.3）、FVC≤74（83.3/74.2）、ALSFRS-R<29（83.3/65.1）。(4) 结论：FEES能提供超出正式PEG指征之外的额外信息。发现ALSFRS-R-B评分≤8是ALS患者行PEG操作的最佳功能及非侵入性指标。
