Respiratory system affects in mucopolysaccharidosis (MPS) disease. Respiratory system symptoms are seen in almost every MPS patients and respiratory failure is one of the most common causes of death in this population. The aim of the study was to evaluate respiratory muscle strength and endurance, cough effectiveness and functional capacity in MPS patients and to defined the factors that affected the respiratory problem in this.
This study was participated MPS patients. Patients' pulmonary function test, functional capacity, respiratory muscle strength and endurance, peak cough flow was assessed and recorded. Pulmonary function test was assessed with spirometry, functional capacity was evaluated with 3-minute step test and six minute walk test (MWT). Respiratory muscle strength was evaluated mouth pressure measurement (MIP- maximal inspiratory pressure and MEP- maximal expiratory pressure) and Sniff nasal inspiratory pressure (SNIP). Respiratory muscle endurance was assessed Powerbreathe. And cough effectiveness was evaluated peak cough flow.
Study Type
OBSERVATIONAL
Enrollment
24
Six minute walk test used assessment of functional capacity.
Hacettepe University
Ankara, Turkey (Türkiye)
functional capacity
Six minute walk test: Distance walked in six minute was recorded
Time frame: 15 minutes
3 minute step test
number of completed step in 3 minute was recorded
Time frame: 5 minutes
Respiratory muscle strength assessment
Respiratory muscle strength was evaluated by measuring maximal inspiratory pressure (MIP) and maximal expiratory pressure (MEP) and sniff nasal inspiratory pressure(SNIP). Also dynamic inspiratory muscle strength was evaluated Powerbreathe K5 device.
Time frame: 15 minutes
Respiratory muscle endurance
Pressure time unit: A constant load test was used to evaluate respiratory muscle endurance. Using a PowerBreathe device (HaB International Ltd., Southam, UK), the participants were asked to breathe maintaining a constant threshold (60% of MIP) until task failure, and the test duration was recorded. Results were obtained by multiplying the set pressure by the test duration
Time frame: 10 minutes
Pulmonary functions
FVC (Forced vital capacity) assessment via spirometry
Time frame: 15 minutes
Pulmonary functions
FEV 1(forced expiratory volume in first second) assessment via spirometry
Time frame: 15 minutes
Pulmonary functions
PEF (peak expiratory flow) assessment via spirometry
Time frame: 15 minutes
MVV (Maximal ventilatory assesssment)
Maximize ventilation by inhaling and exhaling as quickly and deeply as possible for 15 s via spirometry
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Time frame: 5 minutes
cough effectiveness
cough effectiveness assessment was evaluated peak cough flow meter.
Time frame: 5 minutes
heart rate
difference pre and post test heart beat number
Time frame: 6 minutes
oxygen saturation
difference pre and post test percentage of oxygen saturation
Time frame: 6 minutes