The role of respiratory management of Pompe disease

详细信息    查看全文

• 作者：Nicolino Ambrosino ; Marco Confalonieri ; Grazia Crescimanno ; Andrea Vianello ; Michele Vitacca
• 关键词：Neuromuscular diseases ; Pompe disease ; Respiratory failure ; Mechanical ventilation ; Dyspnoea
• 刊名：Respiratory Medicine
• 出版年：2013
• 出版时间：August, 2013
• 年：2013
• 卷：107
• 期：8
• 页码：1124-1132
• 全文大小：544 K

文摘

| Figures/TablesFigures/Tables | ReferencesReferences

Summary

Respiratory failure is an unavoidable event in the natural history of some neuromuscular diseases, while appearing very infrequently in others. In some cases, such as Pompe disease, respiratory failure progresses more rapidly than motor impairment, sometimes being the onset event. Home mechanical ventilation improves survival and quality of life of these patients, with a reduction in healthcare costs. Therefore, pulmonologists must improve their skills in order to play a more relevant role in the care of these patients. The aim of this statement is to provide pulmonologists with some simple information in order for them to fulfil their role of primary caregiver, enabling appropriate and rapid diagnosis and treatment.