Respiratory Functions, Thoracoabdominal Movements and Exercise Capacity in Neuromuscular Diseases

Sponsor
Lokman Hekim University
Study ID
NCT06756633
Status
Recruiting
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Conditions

  • Duchenne Muscular Dystrophy
  • Neuromuscular Diseases in Children
  • Plethysmography
  • Respiratory Function Impaired
  • Respiratory Insufficiency
  • Spinal Muscular Atrophy

Eligibility Criteria

Sex
ALL
Age
5 Years - 15 Years
Healthy Volunteers
Accepted

Interventions

  • Respiratory Function Test — OTHER
    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.
  • Structured light plethysmography — OTHER
    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 measuring device are set up 90-100 cm away to encompass the chest area. The light field, which is in a checkerboard pattern, rectangular or square, should align its upper edge with the participant's clavicles and its lower edge with the anterior-superior iliac spine. During this measurement, the participant is asked to look straight ahead and breathe normally for five minutes. At the end of the measurement, the three-dimensional movements of the chest and respiratory parameters are recorded.
  • 6 Minute Walking Test — OTHER
    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. In such cases, the test duration is paused, and the rest time is recorded, then the test resumes from where it was left off. The distance walked in meters at the end of the test is recorded.
  • 6-Minute Peg Board Ring Test — OTHER
    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 rods will be used. The iron rods will be placed in the designated holes according to the patients' shoulder width. An adjustable chair will be used to suit the patients' heights, and the distance between the chair and the board will be adjusted according to arm length. Patients will be asked to place the rings from top to bottom and bottom to top with both hands simultaneously. The total number of rings placed in six minutes will be recorded in units.
  • Hand Grip Strength — OTHER
    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 taken to determine the participant's hand grip strength. The measurement takes five minutes and does not need to be repeated.

Study Details

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.

Key Dates

First listed
Jan 3, 2025
Start date
Apr 1, 2024
Status verified
Dec 2024
Primary completion
Dec 1, 2025
Completion
Dec 28, 2025

Study Design

Enrollment
42 participants (estimated)

Arms

  • Arm: Duchenne muscular dystrophy
    Participants in this group will be clinically diagnosed with Duchenne Muscular Dystrophy. Eligibility criteria include being over 5 years of age, having the ability to breathe independently without mechanical support, and voluntarily agreeing to participate in the study. Participants will be excluded if they have cognitive problems that may affect test results, have undergone pulmonary surgery, or have advanced heart failure.
  • Arm: Spinal muscular atrophy
    This group will include participants with a clinical diagnosis of Spinal Muscular Atrophy. Similar to the DMD group, eligibility criteria include being over 5 years old, having independent respiratory function, and providing informed consent for study participation. Exclusion criteria for this group are the presence of cognitive impairments that could influence evaluation results, a history of pulmonary surgery, or advanced heart failure.
  • Arm: Healthy controls
    The control group will consist of healthy participants over 5 years of age with no known neuromuscular or respiratory conditions. Participants must be capable of independent breathing and willing to participate in the study. Individuals with cognitive impairments, a history of pulmonary surgery, or any underlying medical condition that could interfere with study outcomes will be excluded from this group.

Primary Outcome Measure

Forced Vital Capacity (FVC) [ Time Frame: Baseline (Day 1) ]

Central Contacts

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