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

Study Overview
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Brief Summary
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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.

Detailed Description
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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 Complete history taking. Thorough clinical examination. Body mass index (BMI) assessment. Quantitative muscle strength tests Functional grading Childhood Myositis Assessment Scale. 7 (B) Laboratory assessment: All patients will be subjected to the following measurements: Serum creatine kinase levels (CK). Serum Lactate dehydrogenase levels Serum of Liver enzymes (SGOT& SGPT) levels. (C) Electromyographic (EMG) assessment: (D) Musculoskeletal ultrasound assessment (E) Statistical analysis

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

Conditions
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Inflammatory Myopathy, Duchenne Muscular Dystrophy

Intervention / Treatment
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* Diagnostic Test: Quantitative muscle ultrasound measurements


Participation Criteria
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Eligibility Criteria
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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.

Ages Eligible for Study
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Minimum Age: 2 Years
Maximum Age: 16 Years

Sexes Eligible for Study
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All

Study Plan
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How is the study designed?
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Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| children with muscle disease<br>fifty children diagnosed to have inflammatory myositis or Duchenne muscular dystrophy in whom Quantitative muscle ultrasound measurements will be performed .The captured images will be analyzed for echo intensity by means of computer-assisted grayscale histogram analysis at baseline and after 24 months. | Diagnostic Test: Quantitative muscle ultrasound measurements<br>* 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.<br>|
| control group<br>20 healthy children matching age and sex as control group in whom Quantitative muscle ultrasound measurement will be performed at baseline | Diagnostic Test: Quantitative muscle ultrasound measurements<br>* 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.<br>|

What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| 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 | 
| 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 | 
| Serum creatine kinase (CK) levels | CK measured in U/L using ELISA | 24 months | 
| Serum Lactate dehydrogenase (LDH) levels | CK measured in IU/L using ELISA | 24 months | 
| Aspartate aminotransferase (AST) | AST measured in U/L using ELISA | 24 MONTHS | 
| alanine aminotransferase (ALT) | ALT measured in U/L using ELISA | 24 months | 
| 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 | 
| 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 | 
| 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 | 

 Terms related to the study
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Keywords Provided by Centre Hospitalier Valida
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Inflammatory myositis, Duchenne Muscular Dystrophy, Musculoskeletal ultrasound