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." Journal Of The College Of Physicians And Surgeons--Pakistan: JCPSP 21.7 (2011): 434-436. MEDLINE Complete.
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." Current Rheumatology Reports 13.3 (2011): 216-224. MEDLINE Complete.
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.
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