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Simplified Whole Body Plethysmography to Characterize Lung Function During Respiratory Melioidosis
Published on: February 24, 2023
[Respiratory aspects of Pompe disease: case report]
Ewandro L R Moura1, Thiago A Jabuonski, Veronica M Amado
1Faculdade de Medicina, Universidade de Brasília, Brasília, Brasil.
Abstract:
We report the case of a 38 years-old female patient with Pompe disease, diagnosed eight years ago. Respiratory complaints appeared five years ago progressing to chronic respiratory failure. Nocturnal CPAP (continuous positive airway pressure) was prescribed last year, presenting progressive clinical worsening. She was referred to Hospital Universitário de Brasília, on account of respiratory failure and cor pulmonale. Thus, she began non-invasive ventilation (NIV) with bi-level positive airways pressure, and a polysomnography showed the need of spontaneous/timed mode associated with adjustments in breathing pressures to improve ventilation during sleep. There was significant clinical improvement after NIV support was established.
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