Assessment of Factors That Affected Respiratory Parameters in Mucopolysaccharidoses Patients

Hacettepe University

Status: Completed

Conditions: Mucopolysaccharidoses

Treatments: Diagnostic Test: functional capacity

Study type: Observational

Funder types: Other

Identifiers

NCT04491747

GO 19/97

Details and patient eligibility

About

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.

Full 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.

Enrollment: 24 patients

Sex: All

Ages: 7 to 30 years old

Volunteers: No Healthy Volunteers

Inclusion criteria

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

Exclusion criteria

• Patients who were not walking ability.