Laxer, Ronald, David Sherry. Pediatric Clinic Pediatric Rheumatology. Philadelphia: Elsevier, 2012. 365-369. Print.
“Juvenile idiopathic inflammatory myopathies (JIMM) are a group of rare, chronic autoimmune diseases that have muscle involvement as a primary feature.” This muscle inflammation creates muscles weakness and can cause the patient to have loss of muscles control. Juvenile Dermatomyositis (JDM) is the most common in the family of these illnesses. Along with the loss of muscle control, the patient can also experience skin rashes. Many children also have a fever, fatigue, and have weight loss. The amount of muscle loss can vary between child. In order to find out if someone has JDM there are many test that may be performed. One of these tests is called the Childhood Myositis Assessment Scale (CMAS). This tests endurance using timing in how long a child can keep their leg raised off of the examination table for a specified time, how long they can keep their head up as well.” I will use this source in my paper to show how the doctors can test if someone has JDM and to show what the symptoms are.
Meneghini Carlo, E. Bonifazi. An Atlas of Pediatric Dermatology. Chicago: Year Book Medical Publishers. 1986. 153-156. Print.
Dermatomyositis is different in children and adults. Children with the disease are more likely to suffer from gastrointestinal problems. In children, dermatomyositis does not appear to be associated with any underlying neoplastic disorder. Whereas in adults this is the case in approximately one fifth of patients. Another way to diagnose JDM is finding elevated levels of muscle enzymes in the blood also known as aldolase or phosphocreatine kinase. This source is useful because they show that there might be other reasons why a person might get JDM instead of it just being genetics.
Ishaque, Sidra, et al. "Juvenile Dermatomyositis." 21.7 (2011): 434-436. . Web. 7 Apr. 2014.
JDM is quite rare only one to 3.2 cases per million in children. Recently a scoring tool was proposed for muscle biopsy evaluation in patients with JDM, which is based on 4 domains of change: inflammatory, vascular, muscle fiber, and connective tissue. In a case of a 14-year old girl who was diagnosed with JDM at the age of 3. She had difficulty in walking and running and had a fever. She was taking a multi vitamin and used an ointment for her rash. Her rash was visible on the top of her hands, predominately on the knuckles, and also on her shoulders and back. The joint of her lower limbs were weak and she had a difficulty standing up from a sitting position and sitting from a standing up position. This source is useful because it goes into the life of someone who has JDM. With this story I can also interview my cousin about what it is like to have JDM.
Batthish, Michelle, and Brian M Feldman. "Juvenile Dermatomyositis." 13.3 (2011): 216-224. . Web. 7 Apr. 2014.
“The etiology of JDM is not clearly understood. However, it is suggested that JDM is caused by a combination of an autoimmune reaction in genetically susceptible individuals in response to environmental triggers such as infectious agents.”
Lauren M. Pachman’s Research Interests. Lurie Children's Hospital of Chicago Research Center, 2014. Web. 07 Apr. 2014.
“Dr. Pachman's translational and collaborative team studies juvenile dermatomyositis (JDM), an often chronic pediatric systemic vasculopathy of unknown etiology. JDM is associated with inflammation in the skin, as well as in the proximal musculature – resulting in extensive weakness. The goal of their studies is to discover biomarkers of disease activity to guide the utilization and/or creation of more effective therapies. Dr. Pachman’s laboratory has identified genetic and environmental factors that not only play a role in the onset of symptoms, but also govern the child's outcome. For example, the TNF-α-308 promoter A polymorphism is associated with a prolonged disease course requiring immunosuppressive therapy for at least three years. Increased production of the protein, TNF-α, by JDM children with the A allele is seen throughout the disease course, in contrast to serum levels of IFN-α activity, which are usually elevated early in the disease. Gene expression micro array studies of untreated children's diagnostic muscle biopsies disclosed a strong dysregulation of IFN-α induced genes in JDM, similar to an anti-viral immune model, which also shares some features of the IFN-α signature characteristic of systemic lupus erythematosus (SLE). This chronic inflammatory setting promotes the development of often debilitating pathologic calcifications, which have been clearly defined as dystrophic in nature, displaying an increased mineral/matrix ratio, and extensive integrin involvement.” I know that Dr. Pachman is a credible source because she is my cousins doctors at Children’s Memorial Hospital in Chicago.
What Is JM? Cure JM Foundation, 2014. Web. 08 Apr. 2014.
JDM affects only 17,000 children in the United States. Weak muscles are a skin rash are the main symptoms of JDM but many children see various forms and severities of them. JDM affects girls twice as much as boys. Once a child is diagnose with JDM is it always considered to be the juvenile form, even when the patient reaches adulthood. I have used this website for another school project and I asked my cousin to look at the website and to tell me if it correctly depicts what it is like for someone to have JDM and she said it does.
Dermatomyositis Juvenile. American College of Rheumatology. Web. 08 Apr. 2014.
There are a few treatments for JDM one is using a steroid called Prednisone. It helps to take away the inflammation but it has bad side effects. Some of these are weight gain, mood swings, and stunting of growth. Another medication that can be used to treat JDM is Methotrexate, it is a chemo drug but patients who has JDM are only on very low doses. They do not get any chemo symptoms like hair loss. Methotrexate is taken orally or through an injection. This source is useful because it talks about all the different medications that can be taken to help reduce the symptoms of JDM.
Juvenile Dermatomyositis Research Group. Juvenile Dermatomyositis Cohort Biomaker Study and Repository UK and Ireland, 2014. 23 April 2014
In the UK various researchers, scientists, nurses, physiotherapists, and doctors who work with patients who suffer from JDM, have begun research. This is called the Juvenile Dermatomyositis Cohort Biomarker Study and Repository. The have been able to do studies on clinical treatments, physiotherapy, new drugs for JDM, and what is the best way to analyze muscle tissue.