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[神经肌肉疾病中的呼吸治疗]

[Respiratory treatments in neuromuscular disease].

作者信息

Martínez Carrasco C, Cols Roig M, Salcedo Posadas A, Sardon Prado O, Asensio de la Cruz O, Torrent Vernetta A

机构信息

Sección de Neumología Pediátrica, Hospital Universitario La Paz, Madrid, España.

Sección de Neumología Pediátrica, Hospital Sant Joan de Déu, Barcelona, España.

出版信息

An Pediatr (Barc). 2014 Oct;81(4):259.e1-9. doi: 10.1016/j.anpedi.2014.04.008. Epub 2014 Jun 2.

Abstract

In a previous article, a review was presented of the respiratory pathophysiology of the patient with neuromuscular disease, as well as their clinical evaluation and the major complications causing pulmonary deterioration. This article presents the respiratory treatments required to preserve lung function in neuromuscular disease as long as possible, as well as in special situations (respiratory infections, spinal curvature surgery, etc.). Special emphasis is made on the use of non-invasive ventilation, which is changing the natural history of many of these diseases. The increase in survival and life expectancy of these children means that they can continue their clinical care in adult units. The transition from pediatric care must be an active, timely and progressive process. It may be slightly stressful for the patient before the adaptation to this new environment, with multidisciplinary care always being maintained.

摘要

在前一篇文章中,对神经肌肉疾病患者的呼吸病理生理学、临床评估以及导致肺部恶化的主要并发症进行了综述。本文介绍了为尽可能长时间维持神经肌肉疾病患者肺功能所需的呼吸治疗方法,以及在特殊情况下(呼吸道感染、脊柱侧弯手术等)的治疗方法。特别强调了无创通气的使用,它正在改变许多此类疾病的自然病程。这些儿童生存率和预期寿命的提高意味着他们可以在成人科室继续接受临床护理。从儿科护理的过渡必须是一个积极、及时且循序渐进的过程。在适应这个新环境之前,患者可能会有轻微的压力,但始终要保持多学科护理。

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