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Juvenile dermatomyositis
V Seth1, S K Kabra, O P Semwal
1Department of Pediatrics, All India Institute of Medical Sciences, Ansari Nagar, New Delhi.
Insights
This study details 7 pediatric cases, noting a female predominance and a median onset age of 6 years. All patients showed skin rash, muscle weakness, and abnormal enzyme levels, responding well to corticosteroid treatment.
Area of Science:
- Pediatric Rheumatology
- Neuromuscular Disorders
- Dermatomyositis
Background:
- Idiopathic inflammatory myopathies (IIMs) are rare in children.
- Dermatomyositis is the most common IIM in the pediatric population.
- Understanding the clinical presentation and treatment response is crucial for early diagnosis and management.
Purpose of the Study:
- To describe the clinical profile and follow-up of pediatric patients with a specific neuromuscular disorder.
- To analyze the diagnostic findings, including enzyme profiles, muscle biopsies, and electromyograms (EMG).
- To evaluate the efficacy of corticosteroid therapy in this cohort.
Main Methods:
- Retrospective case series of 7 children.
- Clinical data collection including age of onset, symptoms (skin rash, muscle weakness), and laboratory findings (enzyme profile).
- Diagnostic investigations included muscle biopsy and electromyogram (EMG).
Main Results:
- Female predominance observed (female to male ratio 1:6).
- Median age of onset was 6 years.
- All patients presented with skin rash, muscle weakness, and abnormal enzyme profiles. Muscle biopsy and EMG were abnormal in most patients.
- All children responded favorably to corticosteroid treatment, with two showing significant improvement after intravenous dexamethasone bolus therapy.
Conclusions:
- Pediatric inflammatory myopathies present with characteristic skin and muscle involvement.
- Early diagnosis supported by enzyme profiles, muscle biopsy, and EMG is essential.
- Corticosteroids, including intravenous dexamethasone, are effective treatments for pediatric cases.
Abstract:
The clinical profile of 7 children and their follow-up is described. There was female preponderance with mate to female ratio of 1:6. The median age of onset was 6 years. All the patients had skin rash, muscle weakness and abnormal enzyme profile. Muscle biopsy was performed in 6 and was abnormal in all of them. The electromyogram (EMG) was performed in 6 and was found abnormal in five. All the children responded well to corticosteroids. Two children received intravenous dexamethasone bolus and showed good response.