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

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

key information

study id #: NCT03786913

condition: Inflammatory Myopathy, Duchenne Muscular Dystrophy

status: completed

purpose:

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.

mechanism of action: No pharmaceutical intervention

results: https://clinicaltrials.gov/ct2/show/results/NCT03786913

last updated: February 15, 2019

study details

start date: March 8, 2016

estimated completion: February 2, 2019

size / enrollment: 48

study 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
• Complete history taking.
• Thorough clinical examination.
• Body mass index (BMI) assessment.
• Quantitative muscle strength tests
• Functional grading
• Childhood Myositis Assessment Scale.
(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

primary outcomes:

  • Kendall's manual muscle testing [ Time Frame: 24 months ]
    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)
  • Childhood myositis assessment scale [ Time Frame: 24 months ]
    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)
  • Serum creatine kinase (CK) levels [ Time Frame: 24 months ]
    CK measured in U/L using ELISA
  • Serum Lactate dehydrogenase (LDH) levels [ Time Frame: 24 months ]
    CK measured in IU/L using ELISA
  • Aspartate aminotransferase (AST) [ Time Frame: 24 MONTHS ]
    AST measured in U/L using ELISA
  • Alanine aminotransferase (ALT) [ Time Frame: 24 months ]
    ALT measured in U/L using ELISA
  • Motor unit potential (MUP) duration [ Time Frame: 24 months ]
    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.
  • Motor unit peak-to-peak amplitude [ Time Frame: 24 months ]
    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
  • Motor unit area to amplitude ratio (AAR) [ Time Frame: 24 months ]
    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 .

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.

study contacts

sponsor: Benha University

investigators: Waleed Hassan, MD

locations: Egypt