Assessment of Factors That Affected Respiratory Parameters in Mucopolysaccharidoses Patients

Completed

• Conditions: Mucopolysaccharidoses

• Registration Number: NCT04491747
• Lead Sponsor: Hacettepe University

Brief Summary

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

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.

Basic Information
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Recruitment & Eligibility
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Study & Design
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Trial Locations

Study Timeline

• Study Start: 2019-02-01
• Primary Completion: 2020-02-01
• Study Completion: 2020-02-01
• Status Verified: 2020-07
• Study First Submitted: 2020-07-19
• Study First Submitted QC: 2020-07-27
• Last Update Submitted: 2020-07-27
• Study First Posted: 2020-07-29
• Last Update Posted: 2020-07-29

Recruitment & Eligibility

• Status: COMPLETED
• Sex: All
• Target Recruitment: 24

Inclusion Criteria

• People who diagnosed Mucopolysaccharidoses disease in Hacettepe University.
• Cooperation

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Exclusion Criteria

• Patients who were not walking ability.

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Study & Design

• Study Type: OBSERVATIONAL
• Study Design: Not specified

Primary Outcome Measures

Name	Time	Method
functional capacity	15 minutes	Six minute walk test: Distance walked in six minute was recorded
3 minute step test	5 minutes	number of completed step in 3 minute was recorded
Respiratory muscle strength assessment	15 minutes	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.
Respiratory muscle endurance	10 minutes	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
Pulmonary functions	15 minutes	PEF (peak expiratory flow) assessment via spirometry
MVV (Maximal ventilatory assesssment)	5 minutes	Maximize ventilation by inhaling and exhaling as quickly and deeply as possible for 15 s via spirometry
cough effectiveness	5 minutes	cough effectiveness assessment was evaluated peak cough flow meter.

Secondary Outcome Measures

Name	Time	Method
heart rate	6 minutes	difference pre and post test heart beat number
oxygen saturation	6 minutes	difference pre and post test percentage of oxygen saturation

Trial Locations

Locations (1)

Hacettepe University; 🇹🇷 Ankara, Turkey