amyloid dysphagia

Abstract
Background: Systemic amyloidosis, a rare disorder characterized by protein misfolding and accumulation, affects various organs and can impair swallowing, leading to complications like aspiration pneumonia. This study aimed to measure swallowing function in patients with systemic amyloidosis using both objective (FEES) and subjective (EAT-10) methods of assessment to determine the specific stages and severity of dysphagia.
Methods: Twenty-six patients with amyloidosis underwent a prospective evaluation using flexible endoscopic evaluation of swallowing (FEES) and the Eating Assessment Tool (EAT-10). The measures included the Penetration-Aspiration Scale (PAS), Yale Pharyngeal Residue Severity Scale (YPRSS), Functional Oral Intake Scale (FOIS), and Functional Oral Swallowing Scale (FOSS). Swallowing impairments were determined in relation to the established normal reference values for each assessment.
ResultsThe average EAT-10 score exceeded normal population levels, reflecting higher perceived swallowing difficulties. FEES assessments revealed elevated risk of penetration and aspiration, especially with liquids (PAS: p<0.001), and significant pharyngeal residue (YPRSS: p<0.001). FOSS scores also indicated reduced swallowing efficiency compared with controls (p<0.001).
Conclusion: Patients with amyloidosis experience substantial swallowing impairment, particularly with fluid intake, increasing their risk for aspiration and potential complications. The combined use of FEES and EAT-10 provides a comprehensive assessment approach, supporting the need for targeted dietary strategies and careful dysphagia management in this patient population.
Keywords:Systemic Amyloidosis,Chronic Kidney Disease, Dysphagia, Eating Assessment Tool (EAT-10), Fiberoptic Endoscopic Evaluation of Swallowing (FEES), Swallowing Dysfunction.

摘要
背景：系统性淀粉样变性是一种以蛋白质错误折叠和积累为特征的罕见疾病，可影响多种器官，并可能导致吞咽困难，进而引发吸人性肺炎等并发症。本研究旨在采用客观（吞咽内窥镜检查，FEES）和主观（进食评估工具，EAT-10）的评估方法，对系统性淀粉样变性患者的吞咽功能进行测量，以确定吞咽障碍的具体阶段和严重程度。
方法：26名淀粉样变性患者接受了前瞻性评估，使用吞咽内窥镜检查（FEES）和进食评估工具（EAT-10）。评估措施包括穿透-吸入量表（PAS）、耶鲁咽部残留严重程度量表（YPRSS）、功能性口腔摄入量表（FOIS）和功能性口腔吞咽量表（FOSS）。吞咽障碍的评估与每种评估方法所确立的正常参考值相关联。
结果：EAT-10的平均得分超出正常人群水平，反映出更高的感知吞咽困难。FEES评估显示，尤其是液体摄入时，发生穿透和吸入的风险升高（PAS：p<0.001），并且咽部残留显著（YPRSS：p<0.001）。FOSS得分也表明，与对照相比，吞咽效率降低（p<0.001）。
结论：淀粉样变性患者存在显著的吞咽障碍，尤其是在流体摄入时，这增加了吸入和潜在并发症的风险。FEES和EAT-10的结合使用提供了一种全面的评估方法，支持对这一患者群体采取针对性的饮食策略和细致的吞咽障碍管理。
关键词：系统性淀粉样变性，慢性肾病，吞咽困难，进食评估工具（EAT-10），吞咽内窥镜检查（FEES），吞咽功能障碍。