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Updated: May 12, 2026

Normothermic Negative Pressure Ventilation Ex Situ Lung Perfusion: Evaluation of Lung Function and Metabolism
Published on: February 14, 2022
The role of respiratory management of Pompe disease
Nicolino Ambrosino1, Marco Confalonieri, Grazia Crescimanno
1U.O. Pneumologia e Terapia Intensiva Respiratoria, Dipartimento Cardio-Toraco-Vascolare, Azienda Ospedaliero Universitaria Pisana, Pisa, Italy. nico.ambrosino@gmail.com
Abstract:
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.
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