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肌营养不良症患者呼吸并发症的管理和康复：意大利肌营养不良症协会（UILDM）第 1 次共识会议报告（2019 年 1 月 25 日至 26 日，米兰）。

Management of respiratory complications and rehabilitation in individuals with muscular dystrophies: 1st Consensus Conference report from UILDM - Italian Muscular Dystrophy Association (Milan, January 25-26, 2019).

机构信息

Respiratory Unit, NEuroMuscular OmniCentre (NeMO), Serena Onlus Foundation, Niguarda Hospital, Milan, Italy.

Pneumology Unit, Santa Maria Bianca Hospital, AUSL Modena, Italy.

出版信息

Acta Myol. 2021 Mar 31;40(1):8-42. doi: 10.36185/2532-1900-045. eCollection 2021 Mar.

DOI:10.36185/2532-1900-045
PMID:33870094
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC8033426/
Abstract

Respiratory complications are common in the patient with muscular dystrophy. The periodic clinical and instrumental respiratory evaluation is extremely important. Despite the presence in the literature of updated guidelines, patient associations often report lack of knowledge of these pathologies, particularly in peripheral hospitals. The purpose of this work, inspired by the Italian Muscular Dystrophy Association (UILDM) is to improve management of respiratory problems necessary for the management of these patients complex. To this end, the main items that the specialist can meet in the follow-up of these pathologies have been analyzed and discussed, among which the respiratory basal evaluation, the criteria of adaptation to non-invasive ventilation, management of bronchial secretions, situations of respiratory emergency, indications for tracheostomy and the subject of advance directives of treatment (DAT).

摘要

呼吸并发症在肌肉萎缩症患者中很常见。定期进行临床和仪器呼吸评估非常重要。尽管文献中有更新的指南，但患者协会经常报告缺乏对这些疾病的了解，特别是在偏远医院。这项工作的目的是受意大利肌肉萎缩症协会（UILDM）的启发，旨在改善对这些复杂患者管理所需的呼吸问题的管理。为此，分析和讨论了专家在随访这些疾病时可能遇到的主要项目，其中包括呼吸基础评估、无创通气适应标准、支气管分泌物管理、呼吸紧急情况、气管切开术适应症和治疗预先指示（DAT）问题。

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