Respiratory Functions, Thoracoabdominal Movements and Exercise Capacity in Neuromuscular Diseases
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An automatic summary of structured registry data. It is an orientation aid, not a substitute for the official protocol or a physician assessment.
- What is being studied
- The protocol lists: Respiratory Function Test, Structured light plethysmography, 6 Minute Walking Test, 6-Minute Peg Board Ring Test.
- Who it may be relevant to
- Registry conditions: Neuromuscular Diseases in Children, Respiratory Function Impaired, Respiratory Insufficiency, Duchenne Muscular Dystrophy. Basic parameters: 5 years — 15 years · All.
- What needs checking
- Age, condition and sex are only basic indicators. Prior treatment, laboratory values and other mandatory requirements appear in the eligibility criteria below.
- Where it takes place
- Turkey (Türkiye)
- Next step
- Save the trial, show it to the treating physician, and confirm current recruitment with the study center. Costs, documents and travel →
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Official title
Evaluation of Respiratory Functions, Thoracoabdominal Movements, and Exercise Capacity in Neuromuscular Diseases
Overview
The clinical trial titled "Investigation of Respiratory Functions, Thoracoabdominal Movements, and Exercise Capacity in Neuromuscular Diseases" aims to evaluate the respiratory functions, thoracoabdominal movements, and exercise capacity in children with Duchenne Muscular Dystrophy (DMD) and Spinal Muscular Atrophy (SMA) compared to healthy controls. The study will use spirometry, structured light plethysmography (SLP), the six-minute walk test, and the six-minute pegboard ring test to assess these functions. This trial will be conducted at the Lokman Hekim University Muscle and Nerve Diseases Application and Research Center from May 2024 to Dec 2025.
Detailed description
This cross-sectional study will be conducted at the Lokman Hekim University Muscle and Nerve Diseases Application and Research Center over a 20-month period from May 2024 to December 2025. The study will include a total of 42 children, with three groups of 14 participants each: children diagnosed with Duchenne Muscular Dystrophy (DMD), children diagnosed with Spinal Muscular Atrophy (SMA), and a healthy control group. The study is designed to evaluate key respiratory, functional, and muscular parameters in these groups.
Respiratory parameters will be assessed using spirometry and Structured Light Plethysmography (SLP). Spirometry will involve maximal inspiratory and expiratory maneuvers to measure lung volumes and capacities, conducted under standardized conditions and monitored by a trained technician. SLP will be conducted using the PneumoCare device, a non-invasive system that measures thoracoabdominal movement during tidal breathing. A grid of light will be projected onto the chest to capture three-dimensional movement patterns over a five-minute period with participants in a seated position.
Functional and muscular assessments will include the Six-Minute Walk Test (6MWT), the Six-Peg Board Ring Test (6PBRT), and hand grip strength measurements. The 6MWT will be conducted in a 20-meter corridor, where participants will walk for six minutes at their own pace, with heart rate, blood pressure, and oxygen saturation recorded before and after the test. The 6PBRT will assess upper-limb endurance and strength through the timed movement of rings across a pegboard, with accuracy and task duration recorded. Hand grip strength will be measured using a handheld dynamometer, with participants performing three trials, and the highest value noted for analysis.
Data analysis will be performed using IBM SPSS Statistics version 23.0. Descriptive statistics will summarize the demographic and clinical characteristics of participants. Group comparisons will be conducted using independent samples t-tests, Mann-Whitney U tests for non-normally distributed variables, and one-way ANOVA for multi-group comparisons. Correlations between variables will be assessed using Pearson or Spearman correlation coefficients, depending on the distribution of the data. A significance threshold of p\<0.05 will be applied, with Bonferroni corrections for multiple comparisons as needed.
Ethical approval for this study was granted by the Lokman Hekim University Scientific Research Ethics Committee (approval number: 2024114). All participants and their legal guardians will provide informed consent prior to enrollment, and all data will be anonymized and securely stored in compliance with the General Data Protection Regulation (GDPR). The study will follow Good Clinical Practices (GCP) and adhere to the ethical principles of the Declaration of Helsinki.
The primary objective is to evaluate and compare respiratory, functional, and muscular parameters in children with DMD and SMA against those in healthy peers. The study aims to identify distinctive patterns and impairments within the patient groups, which could provide insights for developing targeted therapeutic interventions.
Interventions
- Other Respiratory Function Test
A spirometer (microQuark, COSMED) will be used to assess respiratory functions. During a forced expiratory maneuver after a deep, full inspiration, airway and lung volumes are measured. During the measurement, a deep inspiration followed by a strong, fast, and continuous expiration until unable to exhale anymore should be performed. The expiration time should be at least 6 seconds, and if necessary, extended up to 15 seconds. More than eight repetitions at one time are not recommended. - Other Structured light plethysmography
Participants' thoracoabdominal movements will be assessed using the PneumoCare device that measures with the SLP technique. The evaluation begins with positioning participants wearing a white tank top, t-shirt, or with a bare chest. In our study, measurements will be taken with participants seated, supported by their backs, and their chest area exposed. The participant's age, height, and weight information are entered into the system. After the participant is positioned, the lights of the SLP me - Other 6 Minute Walking Test
For Ambulatory Participants: Exercise capacity will be assessed using the Six-Minute Walk Test (6MWT). The test will be performed according to ATS/ERS guidelines . The test involves walking for six minutes at a submaximal level in a 20-meter corridor. Blood pressure, heart rate, respiratory rate, oxygen saturation, and dyspnea, general fatigue, and leg fatigue according to the Modified BORG Scale (MBS) will be assessed before and after the test. Participants can stop and rest during the test. I - Other 6-Minute Peg Board Ring Test
For Non-Ambulatory Participants: The 6-Minute Pegboard Ring Test will be used to assess upper extremity exercise capacity . Pre- and post-test evaluations of heart rate, blood pressure, respiratory rate, and MBS for dyspnea and fatigue will be made. The board used for the test has a total of 6 holes spaced 10 cm apart horizontally and another set of 6 holes 20 cm below aligned with the top row. There are four 20 cm long iron rods suitable for the holes. A total of 20 rings suitable for the iron - Other Hand Grip Strength
Hand grip strength will be measured using a digital hand dynamometer (CAMRY Digital Hand Dynamometer) . The device handle will be adjusted according to the participants' hand size. The test is conducted while seated, with the dominant upper extremity in 90 degrees of elbow flexion. Participants are asked to squeeze the dynamometer as strongly as possible. The value displayed on the dynamometer is recorded. The measurement is repeated three times, and the average of the three measurements is take
Primary outcome measures
- Forced Vital Capacity (FVC) [Time frame: Baseline (Day 1)]
- Forced Expiratory Volume in 1 Second (FEV1) [Time frame: Baseline (Day 1)]
- Peak Expiratory Flow (PEF) [Time frame: Baseline (Day 1)]
- FEV1/FVC Ratio [Time frame: Baseline (Day 1)]
- 6-Minute Walk Test (6MWT) [Time frame: Baseline (Day 1)]
- Thoracoabdominal movement [Time frame: 8 min]
- Hand Grip Strength [Time frame: 5 min]
- 6-Minute Peg Board Ring Test (6PBRT) [Time frame: Baseline (Day 1)]
Eligibility criteria
- Duchenne Muscular Dystrophy (DMD)
-Inclusion Criteria: Clinically diagnosed with Duchenne Muscular Dystrophy. Age above 5 years. Able to breathe independently. Willing to participate in the study.
-Exclusion Criteria: Presence of cognitive problems that affect the assessment results. History of pulmonary surgery. Advanced heart failure.
- Spinal Muscular Atrophy (SMA)
-Inclusion Criteria: Clinically diagnosed with Spinal Muscular Atrophy. Age above 5 years. Able to breathe independently. Willing to participate in the study.
-Exclusion Criteria: Presence of cognitive problems that affect the assessment results. History of pulmonary surgery. Advanced heart failure.
- Healthy Controls
- Inclusion Criteria:
No diagnosis of neuromuscular diseases. Age above 5 years. Able to breathe independently. Willing to participate in the study.
-Exclusion Criteria: Presence of cognitive problems that affect the assessment results. History of pulmonary surgery. Advanced heart failure.
Criteria are shown verbatim from the registry (in English). Final eligibility is always assessed by the study center.
Healthy volunteers: Yes
Study design
- Observational model
- Other
Study locations
Turkey (Türkiye) · 1 center
- Lokman Hekim University — Ankara
Identifiers
NCT: NCT06756633 · LokmanHekimUniv