Assessment of Factors That Affected Respiratory Parameters in Mucopolysaccharidoses Patients

Mucopolysaccharidoses Clinical Trial

Official title:

Assessment of Factors That Affected Respiratory Parameters in Mucopolysaccharidoses Patients

Clinical Trial Details — Status: Completed

Administrative data

• NCT number: NCT04491747
• Other study ID #: GO 19/97
• Status: Completed
• Start date: February 1, 2019
• Est. completion date: February 1, 2020

Study information

• Verified date: July 2020
• Source: Hacettepe University
• Contact: n/a
• Is FDA regulated: No
• Study type: Observational

Clinical Trial 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.

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.

Recruitment information / eligibility

• Status: Completed
• Enrollment: 24
• Est. completion date: February 1, 2020
• Est. primary completion date: February 1, 2020
• Gender: All
• Age group: 7 Years to 30 Years

Eligibility

Inclusion Criteria:

• People who diagnosed Mucopolysaccharidoses disease in Hacettepe University.

• Cooperation

Exclusion Criteria:

• Patients who were not walking ability.

Related Conditions & MeSH terms

• Mucopolysaccharidoses

Intervention

Diagnostic Test:
• functional capacity
Six minute walk test used assessment of functional capacity.

Locations

Country	Name	City	State
Turkey	Hacettepe University	Ankara

Sponsors (1)

Lead Sponsor	Collaborator
Hacettepe University

Country where clinical trial is conducted

Turkey, 

Outcome

Type	Measure	Description	Time frame	Safety issue
Primary	functional capacity	Six minute walk test: Distance walked in six minute was recorded	15 minutes
Primary	3 minute step test	number of completed step in 3 minute was recorded	5 minutes
Primary	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
Primary	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
Primary	Pulmonary functions	FVC (Forced vital capacity) assessment via spirometry	15 minutes
Primary	Pulmonary functions	FEV 1(forced expiratory volume in first second) assessment via spirometry	15 minutes
Primary	Pulmonary functions	PEF (peak expiratory flow) assessment via spirometry	15 minutes
Primary	MVV (Maximal ventilatory assesssment)	Maximize ventilation by inhaling and exhaling as quickly and deeply as possible for 15 s via spirometry	5 minutes
Primary	cough effectiveness	cough effectiveness assessment was evaluated peak cough flow meter.	5 minutes
Secondary	heart rate	difference pre and post test heart beat number	6 minutes
Secondary	oxygen saturation	difference pre and post test percentage of oxygen saturation	6 minutes