Respiratory muscle tests in neuromuscular children
Research type
Research Study
Full title
Respiratory muscle strength tests in neuromuscular paediatric patients
IRAS ID
236895
Contact name
Francesco Muntoni
Contact email
Sponsor organisation
Head of Governance, Clinical Trials and Contracts Division of Research & Innovation | Joint R&D Offi
Duration of Study in the UK
1 years, 9 months, 1 days
Research summary
Respiratory muscle involvement in neuromuscular disorders (NMD) is a common feature.
The progressive reduction in lung volumes (expressed as Vital Capacity, VC) due to the combination of respiratory muscle impairment with the rib cage stiffness correlates with the onset of sleep disordered breathing (SDB) ranging from obstructive sleep apnoea to carbon dioxide (CO2) retention (defined nocturnal hypoventilation).
Therefore, it is important that both daytime and overnight respiratory assessment are carried out regularly in neuromuscular patients as per British Thoracic Society current guidelines on the respiratory management in NMD children [Hull J et al. Thorax. 2012 Jul;67 Suppl 1:i1-40].Sniff inspiratory pressure (SNIP) is a test of inspiratory muscle strength detected through a nasal probe. SNIP has been used in monitoring the respiratory decline over time in Duchenne muscular Dystrophy patients and other adult NMD.
When it comes to children, SNIP has been proven to be feasible and reproducible.The most significant advantage of SNIP is its feasibility in young children/those with developmental delay or in patients with bulbar dysfunction/ facial weakness.
Two works have tried to correlate SNIP not only with lung volumes, but also with the presence/severity of sleep disordered breathing in children with neuromuscular disorders, with contrasting results mostly caused by the heterogeneity of the NMD diagnostic groups [Bersanini C. et al Eur Respir J 2012; 39: 1206–1212, Anderson VB. et al Neuromuscular Disorders 22 (2012) 528–533].Specific differences of SNIP values among specific NMD diagnostic groups, both in terms of repeatability, absolute numbers compared to standard values or prognostic usefulness have not been specifically addressed yet.
We hypothesized that SNIP measurements are feasible and repeatable in a paediatric NMD population and that vary significantly across specific diagnostic NMD subgroups.
REC name
West Midlands - Black Country Research Ethics Committee
REC reference
19/WM/0042
Date of REC Opinion
5 Mar 2019
REC opinion
Further Information Favourable Opinion