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Respiratory treatment in a patient with nemaline myopathy

Massimiliano Polastri1, Gioacchino Schifino2, Eva Tonveronachi3

  • 1Department of Continuity of Care and Disability, Physical Medicine and Rehabilitation, St Orsola University Hospital.

Clinics and Practice
|January 14, 2020
PubMed

Respiratory involvement of nemaline myopathy is evident on the diaphragm and intercostal muscles function. The present case study aimed to describe the characteristics of the respiratory physiotherapeutic treatment in a patient with nemaline myopathy. Here, we describe a case of a 43-yearold woman with nemaline myopathy undergoing pulmonary rehabilitation during short hospital admission for an acute exacerbation. At the time of hospitalisation, the patient was already under oxygen therapy (1 L/min) and noninvasive home ventilation using bi-level positive airway pressure with a nasal interface. Supplementary therapeutic exercises were implemented in addition to daily nasal noninvasive ventilation. At discharge, ultrasound examination revealed increased diaphragm trophism if compared with data obtained at hospital admission.

Keywords:
Nemaline myopathynoninvasive ventilationphysiotherapy techniquesrespiratory therapyscoliosis

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