Résumé :
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Objective : To assess the utility of sniff nasal inspiratory pressure (SNIP) in the assessment of respiratory function in aymptomatic patients with late-onset inherited muscle disorders (IMD). Methods : 27 patients with no respiratory symptoms (and no symptoms of nocturnal hypoventilation) were assessed by vital capacity (VC), maximal inspiratory mouth pressures (MIP), maximal expiratory mouth pressures (MEP) and sniff inspiratory nasal pressures (SNIP). Results : SNIP showed a better correlation than mouth pressures with VC, particularly in patients with facioscapulohumeral dystrophy. All patients with a VC<1.5 litres or <50% predicted had a SNIP<40cmH2O. Conclusion : Despite being asymptomatic for respiratory muscle weakness, patients with IMD may still have significant respiratory impairment. SNIP is a simple test to perform in patients with IMD, and should form part of their routine assessment; a value >40cmH2O excludes a significant reduction in VC.
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