Assessment of Factors That Affected Respiratory Parameters in Mucopolysaccharidoses Patients

Study Overview
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Brief Summary
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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.

Detailed Description
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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.

Official Title
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Assessment of Factors That Affected Respiratory Parameters in Mucopolysaccharidoses Patients

Conditions
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Mucopolysaccharidoses

Intervention / Treatment
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* Diagnostic Test: functional capacity


Participation Criteria
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Eligibility Criteria
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Inclusion Criteria: People who diagnosed Mucopolysaccharidoses disease in Hacettepe University. Cooperation Exclusion Criteria: Patients who were not walking ability.

Ages Eligible for Study
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Minimum Age: 7 Years
Maximum Age: 30 Years

Sexes Eligible for Study
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All

Study Plan
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How is the study designed?
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Arms and Interventions

| Intervention/Treatment |
| --- |
|Diagnostic Test: functional capacity|Six minute walk test used assessment of functional capacity.|

What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| functional capacity | Six minute walk test: Distance walked in six minute was recorded | 15 minutes | 
| 3 minute step test | number of completed step in 3 minute was recorded | 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. | 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 | 10 minutes | 
| Pulmonary functions | FVC (Forced vital capacity) assessment via spirometry | 15 minutes | 
| Pulmonary functions | FEV 1(forced expiratory volume in first second) assessment via spirometry | 15 minutes | 
| Pulmonary functions | PEF (peak expiratory flow) assessment via spirometry | 15 minutes | 
| MVV (Maximal ventilatory assesssment) | Maximize ventilation by inhaling and exhaling as quickly and deeply as possible for 15 s via spirometry | 5 minutes | 
| cough effectiveness | cough effectiveness assessment was evaluated peak cough flow meter. | 5 minutes | 

Secondary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| heart rate | difference pre and post test heart beat number | 6 minutes | 
| oxygen saturation | difference pre and post test percentage of oxygen saturation | 6 minutes | 

 Terms related to the study
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Keywords Provided by Centre Hospitalier Valida
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Respiratory muscles, exercise test