Natural history of respiratory muscle strength in spinal muscular atrophy: a prospective national cohort study.

ABSTRACT:

Background

Respiratory complications are the most important cause of morbidity and mortality in spinal muscular atrophy (SMA). Respiratory muscle weakness results in impaired cough, recurrent respiratory tract infections and eventually can cause respiratory failure. We assessed longitudinal patterns of respiratory muscle strength in a national cohort of treatment-naïve children and adults with SMA, hypothesizing a continued decline throughout life.

Methods

We measured maximal expiratory and inspiratory pressure (PE_max and PI_max), Sniff Nasal inspiratory pressure (SNIP), peak expiratory flow (PEF), and peak cough flow (PCF) in treatment-naïve patients with SMA. We used mixed-models to analyze natural history patterns.

Results

We included 2172 meas

SUBMITTER: Veldhoen ES 

PROVIDER: S-EPMC8862532 | biostudies-literature | 2022 Feb

REPOSITORIES: biostudies-literature