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Completed

NCT Number: NCT04491747

Assessment of Factors That Affected Respiratory Parameters in Mucopolysaccharidoses Patients

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.

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Key information

Conditions

Mucopolysaccharidoses Carbohydrate Metabolism, Inborn Errors Congenital, Hereditary, and Neonatal Diseases and Abnormalities Connective Tissue Diseases Genetic Diseases, Inborn Lysosomal Storage Diseases Metabolic Diseases Metabolism, Inborn Errors Mucinoses Nutritional and Metabolic Diseases Skin and Connective Tissue Diseases

Age range

7 year–30 year

Sex eligibility

All sexes

Study type

Observational

Primary location

Hacettepe University

Ankara, Turkey (Türkiye)

About this study

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.

Who can participate

Only the study team can determine whether someone qualifies for participation.

Inclusion criteria

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

Exclusion criteria

  • Patients who were not walking ability.

Treatment and study plan

Functional capacity

Diagnostic Test

Six minute walk test used assessment of functional capacity.

Other names: Exercise capacity

Primary outcomes

  1. functional capacity

    Time frame: 15 minutes

    Six minute walk test: Distance walked in six minute was recorded

  2. 3 minute step test

    Time frame: 5 minutes

    number of completed step in 3 minute was recorded

  3. Respiratory muscle strength assessment

    Time frame: 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.

  4. Respiratory muscle endurance

    Time frame: 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

  5. Pulmonary functions

    Time frame: 15 minutes

    FVC (Forced vital capacity) assessment via spirometry

  6. Pulmonary functions

    Time frame: 15 minutes

    FEV 1(forced expiratory volume in first second) assessment via spirometry

  7. Pulmonary functions

    Time frame: 15 minutes

    PEF (peak expiratory flow) assessment via spirometry

  8. MVV (Maximal ventilatory assesssment)

    Time frame: 5 minutes

    Maximize ventilation by inhaling and exhaling as quickly and deeply as possible for 15 s via spirometry

  9. cough effectiveness

    Time frame: 5 minutes

    cough effectiveness assessment was evaluated peak cough flow meter.

Secondary outcomes

  1. heart rate

    Time frame: 6 minutes

    difference pre and post test heart beat number

  2. oxygen saturation

    Time frame: 6 minutes

    difference pre and post test percentage of oxygen saturation

Sponsors and collaborators

Lead sponsor

Hacettepe University

Other

Registry information

Important dates

Study start
2019
Primary completion
2020
Study completion
2020
First posted
Jul 29, 2020
Registry last updated
Jul 29, 2020

OpenTrials presents study information sourced from ClinicalTrials.gov. The official registry record should be consulted for the latest information.

View the official ClinicalTrials.gov record (opens in a new tab)

This listing is for discovery and informational purposes only. It is not medical advice, does not guarantee that a study is recruiting, and does not determine eligibility. Contact the study team and a qualified healthcare professional when considering participation.

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