The truth about the disease that turns muscle into bone
A three-year study followed dozens of patients with an extremely rare disorder that gradually turns muscles, tendons and ligaments into bone. The lifelong condition is known as progressive fibrous dysplasia (FOP).
Only about 800 patients have been diagnosed with progressive fibrous dysplasia worldwide, and in 2006, researchers found that 97% of patients had the same genetic variant of the disease.
And "RT" quoted "Science Aller" that this mutation occurs in the gene that codes for the future regulation of bone growth, and it appears that it causes stem cells to produce bone tissue in places that they should not normally contain.
Given the rarity of fibrous dysplasia, the researchers hope that the results of the current research will allow clinicians to better meet the medical needs of underserved patients.
The gradual soft tissue calcification of fibrous dysplasia is not consistent and comes in waves.
The seizures usually begin in childhood and tend to start in the neck and shoulders.
The hard tissue extends over the joints, gradually limiting movement, fixing body parts in place and reducing the life of the injured.
Not everyone with fibrous dysplasia shows the same rate of calcification, but once bones have formed in one part of the body, they are permanent, causing most sufferers to need a wheelchair by age 20.
Unfortunately, no treatments are available for fibrous dysplasia to date, although the pain and swelling experienced by patients can be relieved to some extent with medication.
Over the course of a patient's life, illness and physical trauma can cause swollen muscles and inflammation that can last from days to months, often leading to bone formation afterward.
Fibrous dysplasia does not only affect the musculoskeletal system, as the patients studied were commonly found to have respiratory problems, such as a reduced ability to expand the chest (this can be fatal), and hearing loss.
High doses of corticosteroids taken for four days during an episode have been shown to be very effective in reducing inflammation associated with excessive bone growth.
The new study, along with ongoing clinical trials, could help improve the lives of those with this rare condition in the coming years.
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