ltrasound imaging as a new tool for assessment of respiratory muscle structure and function in patients with Duchenne Muscular Dystrophy

Recruiting

Conditions: Duchenne disease
Duchenne Muscular Dystrophy
10028302

Registration Number: NL-OMON56724

Lead Sponsor: Radboud Universitair Medisch Centrum

Brief Summary: Not available

Detailed Description: Not available

Recruitment & Eligibility

Status: Recruiting

Sex: Not specified

Target Recruitment: 90

Inclusion Criteria

Healthy subjects: competent healthy male volunteers.
Patients: - Patients with genetically proven Duchenne Muscular Dystrophy who
are currently receiving care at Radboudumc.

All participants: - Ability to participate and comply with requirements
- If >16: willingness and ability to understand nature and content of the study
- If 12-16: Parents and participant with willingness and ability to understand
nature and content of the study.
- If <12: Parents with willingness and ability to understand nature and content
of the study.

Exclusion Criteria

Healthy volunteers: - Previous or ongoing neuromuscular disorders.
- Previous or ongoing pulmonary deficiencies.
- Smoking
Patients: - Chronic lung, cardiac or liver disease
- Additional diagnosed lung disease

Study & Design

Study Type: Observational non invasive

Study Design: Not specified

Primary Outcome Measures

Name	Time	Method
<p>• Respiratory muscle thickness: Thickness of the diaphragm, accessory<br /><br>inspiratory muscles and expiratory muscles will be examined with ultrasound<br /><br>• Diaphragm excursion: Excursion of the diaphragm will be assessed<br /><br>• Respiratory muscle echogenicity: quantitative muscle ultrasound (QMUS) will<br /><br>assess echogenicity of the diaphragm, accessory inspiratory muscles and<br /><br>expiratory muscles. </p><br>

Secondary Outcome Measures

Name	Time	Method
<p>• All participants; Age, length, weight, medication.<br /><br>• Patients: Age at disease onset, age at diagnosis<br /><br>• Pulmonary function testing (PFT): forced vital capacity (FVC), Forced<br /><br>expiratory volume in one second (FEV1), maximal inspiratory pressure (MIP),<br /><br>maximal expiratory pressure (MEP) and sniff nasal inspiratory pressure (SNIP).<br /><br>For patients, the PFT will be assessed by a pediatric rehabilitation doctor.<br /><br>For healthy participants, it will be assessed by a researcher.<br /><br>• Clinical history: A standardized clinical history will be assessed, including<br /><br>documentation of use of steroids and respiratory support, airway infections and<br /><br>degree of scoliosis. </p><br>