Related Experiment Video
Updated: Mar 9, 2026
07:50
Establishment of a Clinic-based Biorepository
Published on: May 29, 2017
7.0K
Juvenile dermatomyositis: a tertiary center experience.
Kenan Barut1, Pinar Ozge Avar Aydin1, Amra Adrovic1
1Department of Pediatric Rheumatology, Cerrahpasa Medical School, Istanbul University, Istanbul, Turkey.
Clinical Rheumatology
|January 7, 2017
Summary
Juvenile dermatomyositis (JDM) is a rare childhood inflammatory disease affecting muscles and skin. Early, aggressive treatment with corticosteroids and methotrexate leads to remission in most JDM patients, preventing complications.
Area of Science:
- Pediatric Rheumatology
- Dermatology
- Immunology
Background:
- Juvenile dermatomyositis (JDM) is a rare, chronic, idiopathic inflammatory myopathy affecting children.
- It primarily targets muscle and skin tissues, presenting unique diagnostic and therapeutic challenges.
Purpose of the Study:
- To analyze the demographic, clinical, and laboratory features of JDM patients.
- To evaluate treatment strategies and patient outcomes at a Turkish pediatric rheumatology center.
Main Methods:
- Retrospective review of medical records for 50 JDM patients diagnosed between 2003-2016.
- Analysis of presenting symptoms, laboratory data (muscle enzymes, ANA), and diagnostic procedures (EMG, biopsy).
- Assessment of treatment regimens including corticosteroids, methotrexate, and cyclosporine, and patient follow-up.
Main Results:
- Common symptoms included heliotrope rash, Gottron papules, muscle weakness, and erythroderma; 38% had calcinosis.
- All patients exhibited elevated muscle enzymes; 68% were ANA-positive.
- Early, aggressive treatment with corticosteroids and methotrexate achieved remission in nearly all patients, with cyclosporine used for refractory cases.
Conclusions:
- Early diagnosis and intensive therapy are crucial for managing JDM and mitigating complications.
- Effective treatment strategies involving corticosteroids and methotrexate can lead to favorable outcomes.
- Further international collaboration is essential for advancing JDM understanding and management.
Related Concept Videos
01:23Skin Diseases and Disorders
6.0K
Skin is the first line of defense and encounters a variety of microbes. Some pathogenic strains are often the cause of a broad range of infections of the skin and other body systems. These conditions can affect people of all ages and may have different causes, including genetic factors, infections, autoimmune reactions, environmental factors, and lifestyle choices.
Gram-positive Staphylococcus spp. and Streptococcus spp. are responsible for many of the most common skin infections. However, many...
Gram-positive Staphylococcus spp. and Streptococcus spp. are responsible for many of the most common skin infections. However, many...
6.0K
01:14Myocarditis III: Medical Management
282
Myocarditis: Comprehensive Medical ManagementMyocarditis, the heart muscle inflammation, requires a comprehensive medical management strategy that addresses the underlying cause, provides supportive care, manages symptoms, and reduces cardiac workload.Infections and Autoimmune CausesAdminister appropriate antimicrobial therapy when an infectious agent causes myocarditis. For instance, penicillin treats infections caused by Group A Streptococcus. In cases where autoimmune processes are...
282
01:26Nephrotic Syndrome II : Assessment and Medical Management
289
IntroductionNephrotic syndrome is a kidney disorder marked by excessive protein loss in the urine, leading to various systemic complications. This condition often results from damage to the glomeruli—the kidney's filtering units—causing proteinuria, low blood protein levels, and fluid retention. Understanding the assessment, diagnosis, and management of nephrotic syndrome is essential for effective treatment and prevention of further kidney damage.AssessmentPatient History: Document...
289
01:11Papillary Dermis
6.5K
Dermis
The dermis might be considered the "core" of the integumentary system, as distinct from the epidermis and hypodermis. It contains blood and lymph vessels, nerves, and other structures, such as hair follicles and sweat glands. The dermis is made of two layers of connective tissue that comprise an interconnected mesh of elastin and collagenous fibers, produced by fibroblasts.
Papillary Layer
The papillary layer is made of loose, areolar connective tissue, which means the collagen...
The dermis might be considered the "core" of the integumentary system, as distinct from the epidermis and hypodermis. It contains blood and lymph vessels, nerves, and other structures, such as hair follicles and sweat glands. The dermis is made of two layers of connective tissue that comprise an interconnected mesh of elastin and collagenous fibers, produced by fibroblasts.
Papillary Layer
The papillary layer is made of loose, areolar connective tissue, which means the collagen...
6.5K
01:24Nephrotic Syndrome III : Nursing Management
403
Nursing management for nephrotic syndrome adapts as the disease progresses, with strategies evolving to address advancing symptoms and complications.Early-Stage Management In the early stages, nursing interventions for nephrotic syndrome resemble those used in managing acute glomerulonephritis, focusing on symptom monitoring, fluid balance, and managing mild to moderate edema.Vital Signs: Regularly monitor blood pressure, pulse, respiratory rate, and temperature to promptly identify...
403
01:24Nephrotic Syndrome I : Introduction
790
Nephrotic Syndrome is a chronic kidney disorder defined by clinical findings such as severe proteinuria, hypoalbuminemia, hyperlipidemia, and edema. These symptoms result from damage to the glomeruli, the kidney’s filtering units, increasing their permeability to proteins.Definition and Meaning:Proteinuria, defined as the loss of more than 3.5 grams of protein per day in adults, is a crucial feature of nephrotic syndrome. This condition is often accompanied by edema, the accumulation of...
790