肠内营养与气道干预在多系统萎缩中的生存获益

文献信息

中文摘要

引言： 多系统萎缩（MSA）中的吞咽困难可导致肺炎和营养不良，影响生存。本研究评估了肠内营养（EN）与生存之间的关联，并描述了喉气管分离术（LTS）的结局。
方法： 我们回顾性分析了158例很可能/确诊MSA患者（2000-2025年）。使用Simon-Makuch曲线、以EN为时间依赖变量的Mantel-Byar检验，以及采用倾向评分匹配（PSM）和限制性平均生存时间（RMST）的Kaplan-Meier分析，评估自起病以来的生存情况。竞争风险分析（Fine-Gray模型）评估了死亡和呼吸干预（气管造口术或气管造口有创通气[TIV]）的累积发生率。回顾了LTS（n = 11）的临床结局。
结果： 87例患者接受了EN；EN开始后的中位生存期为4.3年。Simon-Makuch分析显示EN组生存率较低，反映了晚期才引入。经过PSM后，EN与7年时适度的生存优势相关（RMST差异0.68年，P = 0.005）。EN组猝死显著减少（15% vs. 30%，P = 0.001）。竞争风险模型显示EN组死亡较低而TIV发生率较高，反映了积极的呼吸管理。在LTS系列中，平均生存期为14.5年，无肺炎相关死亡，并保留了有限的口服进食，支持生活质量获益。
结论： EN与具有统计学显著性但临床上适度的生存优势独立相关，这主要是通过促进包括呼吸管理在内的综合照护路径实现的。在一个小型LTS系列中，肺炎相关死亡的预防和潜在的生活质量获益支持在晚期MSA中进行早期结构化预先照护规划。

英文摘要

INTRODUCTION: Dysphagia in multiple system atrophy (MSA) leads to pneumonia and malnutrition, impacting survival. This study evaluated the association between enteral nutrition (EN) and survival and described outcomes of laryngotracheal separation (LTS).
METHODS: We retrospectively analyzed 158 patients with probable/definite MSA (2000-2025). Survival from onset was evaluated using Simon-Makuch curves, Mantel-Byar tests with time-dependent EN, and Kaplan-Meier analysis with propensity score matching (PSM) and restricted mean survival time (RMST). Competing risk analyses (Fine-Gray models) assessed the cumulative incidence of death and respiratory interventions (tracheostomy or tracheostomy invasive ventilation [TIV]). Clinical outcomes of LTS (n = 11) were reviewed.
RESULTS: Eighty-seven patients received EN; median survival after EN initiation was 4.3 years. Simon-Makuch analysis showed lower survival in the EN group, reflecting late-stage introduction. After PSM, EN was associated with a modest survival advantage at 7 years (RMST difference 0.68 years, P = 0.005). Significant reduction in sudden death occurred in the EN group (15% vs. 30%, P = 0.001). Competing-risk models showed lower death and higher TIV incidence in the EN group, reflecting proactive respiratory management. In the LTS series, mean survival was 14.5 years with no pneumonia-related deaths and preserved limited oral intake, supporting quality-of-life benefits.
CONCLUSION: EN was independently associated with a statistically significant but clinically modest survival advantage, largely by facilitating a comprehensive care pathway including respiratory management. In a small LTS series, the prevention of pneumonia-related death and potential quality-of-life benefits support early structured advance care planning in advanced MSA.