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

NCT03786913 | Completed

• 1 other identifier: BenhaU122018
• Study Type: observational
• Target: 48
• Locations: 1 country
• Sites: 1

Brief 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.

Conditions

Inflammatory Myopathy; Duchenne Muscular Dystrophy

Keywords

Inflammatory myositis; Duchenne Muscular Dystrophy; Musculoskeletal ultrasound

Outcome Measures

Primary Outcomes (9)

• 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

Study Arms (2)

children with muscle disease

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

control group

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

Interventions

Quantitative muscle ultrasound measurements DIAGNOSTIC_TEST

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.

children with muscle disease; control group

Eligibility Criteria

• Age: 2 Years - 16 Years
• Sex: all
• Age Groups: Child (0-17)
• Sampling Method: Probability Sample

Study Population

the study will be performed on 2 groups Group (I): fifty children diagnosed to have inflammatory myositis and Duchenne muscular dystrophy Group (II): including 20 healthy children matching age and sex as control group.

You may qualify if:

• 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).

You may not qualify if:

• 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.

Sponsors & Collaborators

• Benha University: lead

Study Sites (1)

Benha University Hospital

Banhā, Qalyubia Governorate, 13518, Egypt

Location

MeSH Terms

Conditions

Myositis; Muscular Dystrophy, Duchenne

Condition Hierarchy (Ancestors)

Muscular Diseases; Musculoskeletal Diseases; Neuromuscular Diseases; Nervous System Diseases; Muscular Dystrophies; Muscular Disorders, Atrophic; Genetic Diseases, X-Linked; Genetic Diseases, Inborn; Congenital, Hereditary, and Neonatal Diseases and Abnormalities

Study Officials

• Waleed Hassan, MD

  Benha university- Qaluibya- Egypt

  PRINCIPAL INVESTIGATOR

Study Design

• Study Type: observational
• Observational Model: COHORT
• Time Perspective: PROSPECTIVE
• Sponsor Type: OTHER
• Responsible Party: PRINCIPAL INVESTIGATOR
• PI Title: Assistant professor

Study Record Dates

• First Submitted: December 21, 2018
• First Posted: December 26, 2018
• Study Start: March 8, 2016
• Primary Completion: February 2, 2019
• Study Completion: February 2, 2019
• Last Updated: February 8, 2019

Record last verified: 2019-02

Data Sharing

• IPD Sharing: Will not share

Locations

EG (1)