Quantitative Muscle Ultrasound as a Marker of Progression in Children With Muscular Diseases

Duchenne Muscular Dystrophy Clinical Trial

Official title:

Quantitative Muscle Ultrasound as a Monitoring Tool of Disease Progression in Children With Inflammatory Myositis and Duchenne Muscular Dystrophy

Clinical Trial Details — Status: Completed

Administrative data

• NCT number: NCT03786913
• Other study ID #: BenhaU122018
• Status: Completed
• Start date: March 8, 2016
• Est. completion date: February 2, 2019

Study information

• Verified date: February 2019
• Source: Benha University
• Contact: n/a
• Is FDA regulated: No
• Study type: Observational

Clinical Trial Summary

The aim of our study is to Assess skeletal muscle structural status in children with inflammatory myositis and Duchenne muscular dystrophy using musculoskeletal ultrasound and to perform a longitudinal follow up of these changes over 2 years and to assess the relation between these findings with clinical parameters, functional scales, biochemical and electromyographic tests.

Description:

This study will be carried out on two groups:

• Group (I): fifty children diagnosed to have duchenne muscular dystrophy and inflammatory myositis.

Group (II): including 20 healthy children matching age and sex as control group.

patients will be subjected to

(A) Clinical evaluation

1. Complete history taking.

2. Thorough clinical examination.

3. Body mass index (BMI) assessment.

4. Quantitative muscle strength tests

5. Functional grading

6. Childhood Myositis Assessment Scale. 7 (B) Laboratory assessment:

All patients will be subjected to the following measurements:

1. Serum creatine kinase levels (CK).

2. Serum Lactate dehydrogenase levels

3. Serum of Liver enzymes (SGOT& SGPT) levels.

(C) Electromyographic (EMG) assessment:

(D) Musculoskeletal ultrasound assessment (E) Statistical analysis

Recruitment information / eligibility

• Status: Completed
• Enrollment: 48
• Est. completion date: February 2, 2019
• Est. primary completion date: February 2, 2019
• Gender: All
• Age group: 2 Years to 16 Years

Eligibility

Inclusion Criteria:

• children with Duchenne muscular dystrophy (DMD). Diagnosis with DMD was established according to DMD diagnostic criteria (Jennekens et al., 1991).

• children with juvenile dermatomyositis (JDM) according to Bohan and Peter diagnostic criteria ( (Bohan and Peter, 1975).

Exclusion Criteria:

• Patients with age less than 2 years were excluded from the study due to inability to perform manual muscle testing and functional scales.

• If no final diagnosis could be established.

• The presence of a concomitant illness that may result in peripheral neuropathy or myopathy.

Related Conditions & MeSH terms

• Duchenne Muscular Dystrophy
• Inflammatory Myopathy
• Muscular Dystrophies
• Muscular Dystrophy, Duchenne
• Myositis

Intervention

Diagnostic Test:
• Quantitative muscle ultrasound measurements
Quantitative ultrasound measurements will be performed to biceps, forearm flexors, quadriceps and tibialis anterior according to a standard protocol; for each muscle three consecutive measurements will be made to minimize variation in echo intensity during analysis .The captured images will be analyzed offline for echo intensity by means of computer-assisted grayscale histogram analysis.

Locations

Country	Name	City	State
Egypt	Benha University Hospital	Banha	Qalubiya

Sponsors (1)

Lead Sponsor	Collaborator
Benha University

Country where clinical trial is conducted

Egypt, 

Outcome

Type	Measure	Description	Time frame	Safety issue
Primary	Kendall's manual muscle testing	Kendall's 0 -10 point scale measures strength of each muscle group score 0 is the weakest (worst) and 10 is the strongest (best). The following muscles were tested bilaterally: the biceps brachii muscle (BB), the forearm flexors (FF), the rectus femoris muscle (RF), the tibialis anterior muscle (TA)	24 months
Primary	Childhood myositis assessment scale	used to assess the severity of muscle involvement in children with dermatomyositis. The scores for the 14 items are summated to give a total score ranging from 0 (worst) to 52 (best)	24 months
Primary	Serum creatine kinase (CK) levels	CK measured in U/L using ELISA	24 months
Primary	Serum Lactate dehydrogenase (LDH) levels	CK measured in IU/L using ELISA	24 months
Primary	Aspartate aminotransferase (AST)	AST measured in U/L using ELISA	24 MONTHS
Primary	alanine aminotransferase (ALT)	ALT measured in U/L using ELISA	24 months
Primary	motor unit potential (MUP) duration	quantitative electromyography (QEMG) in the most affected rectus femoris and biceps brachii muscles will be performed and The motor unit potentials will be reviewed offline for the needle-detected EMG signals will be analyzed by the device software for the MUP duration measured in milliseconds.	24 months
Primary	motor unit peak-to-peak amplitude	quantitative electromyography (QEMG) in the most affected rectus femoris and biceps brachii muscles will be performed and The motor unit potentials will be reviewed offline for the needle-detected EMG signals will be analyzed by the device software for the peak-to-peak amplitude measured in microvolt	24 months
Primary	motor unit area to amplitude ratio (AAR)	quantitative electromyography (QEMG) in the most affected rectus femoris and biceps brachii muscles will be performed and The motor unit potentials will be reviewed offline for the needle-detected EMG signals will be analyzed by the device software for the motor unit AAR .	24 months