Sniff nasal inspiratory pressure and sleep disordered breathing in childhood neuromuscular disorders

Anderson, Vincent B.; McKenzie, Janice M.; Seton, Chris; Fitzgerald, Dominic A.; Webster, Richard; North, Klaus; Joffe, David; Young, Helen

doi:10.1016/j.nmd.2012.02.002

Cited by 25 publications

(23 citation statements)

References 38 publications

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“…No correlation was observed among SNIP, FVC, FEV 1 , P i max, and polysomnography variables and nocturnal gas exchange in 23 children with various NMDs. 51 We observed no clinically relevant correlation between a large number of lung function and respiratory muscle function parameters and nocturnal gas exchange in a larger group of children. 64 Future studies should focus on homogeneous groups of patients, which may be challenging, considering the wide range of congenital myopathies and muscular dystrophies.…”

Section: Improving Patient Carecontrasting

confidence: 65%

“…Cross-sectional studies have compared respiratory parameters in patients with NMD and healthy control subjects or in patients with diff erent NMD. Such studies have analyzed mainly patients with DMD 18,36,50,51 or SMA. 13,21 By using Pes and Pgas measurements, we showed that DMD was characterized by an early and progressive weakness of the diaphragm and the expiratory muscles, whereas these muscles were relatively spared in children with SMA, and children with congenital myopathies harbored an intermediate weakness of the expiratory muscles.…”

Section: Understanding the Natural History Of A Diseasementioning

confidence: 99%

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The Value of Respiratory Muscle Testing in Children With Neuromuscular Disease

Fauroux

Quijano‐Roy

Desguerre

et al. 2015

Chest

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Section: Improving Patient Carecontrasting

confidence: 65%

Section: Understanding the Natural History Of A Diseasementioning

confidence: 99%

The Value of Respiratory Muscle Testing in Children With Neuromuscular Disease

Fauroux

Quijano‐Roy

Desguerre

et al. 2015

Chest

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“…FVC was found to correlate with presence of nocturnal hypoventilation in children with progressive neuromuscular diseases, but the threshold value for clinical significance varied with age and diagnosis . Others have found that pulmonary function does not predict sleep problems . Reductions of both FVC and forced expiratory volume in 1 s reflect a restrictive disease pattern.…”

Section: Respiratory Muscle Assessments In Patients With Cnmmentioning

confidence: 98%

Respiratory assessment in centronuclear myopathies

2014

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The centronuclear myopathies (CNMs) are a group of inherited neuromuscular disorders classified as congenital myopathies. While several causative genes have been identified, some patients do not harbor any of the currently known mutations. These diverse disorders have common histological features, which include a high proportion of centrally-nucleated muscle fibers, and clinical attributes of muscle weakness and respiratory insufficiency. Respiratory problems in CNMs may manifest initially during sleep, but daytime symptoms, ineffective airway clearance, and hypoventilation predominate as more severe respiratory muscle dysfunction evolves. Respiratory muscle capacity can be evaluated using a variety of clinical tests selected with consideration for the age and baseline motor function of the patient. Similar clinical tests of respiratory function can also be incorporated into preclinical CNM canine models to offer insight for clinical trials. Since respiratory problems account for significant morbidity in patients, routine assessments of respiratory muscle function are discussed.

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“…Maximal respiratory muscle testing including Maximal Inspiratory Pressures (MIP), Maximal Expiratory Pressures (MEP), and Sniff Nasal Inspiratory have a weaker correlation than Vital Capacity for nocturnal hypoventilation. [34][35][36]40,42,43 At present, there is no international consensus as to when to screen for SDB in children with neuromuscular disease. The published recommendations in the field include the following.…”

Section: Key Evidencementioning

confidence: 99%