Advanced respiratory muscle ultrasound in health and congenital myopathy/muscular dystrophy

Status

Active, not recruiting

Phases

Unknown

Study type

Observational

Source

NL-OMON

Registry ID

NL-OMON23911

Enrollment

122

Registered

2020-12-24

Start date

2021-05-01

Completion date

Unknown

Last updated

2024-06-11

For informational purposes only — not medical advice. Sourced from public registries and may not reflect the latest updates. Terms

Conditions

Congenital myopathies and congenital muscular dystrophies

Interventions

None

Eligibility

Inclusion criteria

Inclusion criteria: - Informed consent from participant or legal representative - Age: o Phase 1: age between 18 and 60 years o Phase 2: age between 8 and 60 years - Healthy subjects: competent volunteers - Patients: genetically confirmed congenital myopathy or congenital muscular dystrophy - Willingness and ability to understand nature and content of the study - Ability to participate and comply with study requirements

Exclusion criteria

Exclusion criteria: - Medical history or current condition affecting respiratory muscle strength or function, other than a congenital myopathy or congenital muscular dystrophy (e.g. COPD). - Active smoker - Known upper airway / oesophageal pathology or anatomical variations of the upper airway (phase 1, healthy subjects only) - Known bleeding disorders or frequent nasal bleeding (phase 1, healthy subjects only).

Design outcomes

Primary

Measure	Time frame
Phase 1: inter-rater, intra-rater and test-retest reliability in healthy subjects and patients of the following measurements: -End-expiratory thickness, end-inspiratory thickness and thickening ratio of all respiratory muscles during respiration -Strain and strain rate of all respiratory muscles during respiration -Echogenicity of all respiratory muscles during respiration -Tissue stiffness expressed as Young’s modulus of all respiratory muscles during respiration Phase 2: two year follow-up of respiratory function using advanced respiratory muscle ultrasound in congenital myopathies/muscular dystrophies	—

Measure

Time frame

Phase 1: inter-rater, intra-rater and test-retest reliability in healthy subjects and patients of the following measurements: -End-expiratory thickness, end-inspiratory thickness and thickening ratio of all respiratory muscles during respiration -Strain and strain rate of all respiratory muscles during respiration -Echogenicity of all respiratory muscles during respiration -Tissue stiffness expressed as Young’s modulus of all respiratory muscles during respiration Phase 2: two year follow-up of respiratory function using advanced respiratory muscle ultrasound in congenital myopathies/muscular dystrophies

—

Secondary

Measure	Time frame
Phase 1: validity of advanced respiratory muscle ultrasound, as assessed by the correlation of all measurements to transdiaphragmatic pressure and surface EMG Phase 2: differences between healthy subjects and patients of advanced respiratory muscle ultrasound	—

Contacts

Public ContactJonne Doorduin

Radboudumc

jonne.doorduin@radboudumc.nl0650155757

Outcome results

None listed

Source: NL-OMON (via WHO ICTRP)