Assume that, you are walking in the street. Suddenly an accident takes place. Many part of your body is injured including muscle and bone. After a few days, you will notice that some of your injured muscle are becoming painful, swelled up. Eventually there will be no muscle. That injured area will convert into hard bones!!!
You may suspects I am telling you a fairy tales ass it seems unbelievable. But actually it is truth. Almost 800 people were identified with this circumstances till now. It is a rare genetic disorder. The first case was identified in 17th century. The name of the disease is fibrodysplasia ossificans progressiva. It was before called myositis ossificans. Mutation in chromosome 2 occurs in this disease in autosomal dominant
pattern- that is the basic pathology. Frequency is one in one and half million in united kingdom population.
pattern- that is the basic pathology. Frequency is one in one and half million in united kingdom population.
Children born with this disease has malformed big toe. In 50% of cases, malformed thumb is present. First bone formation from muscle generally occurs before ten years of age. Not only muscle, tendon can also be ossified.
If a bone is formed, then surgical removal is harmful for patient. Patient develops more aggressive bone formation from surrounding muscle and tendon.
Some muscles are spared from this ossification process. Diaphragm, tongue, extra ocular muscles, cardiac muscle, smooth muscles are those. But the cause is still unknown.
The muscle can be formed into bone any time but usually formation occur after a viral illness or a muscle injury.
Is there any cure? At present NO. Only supportive treatment can be given to reduce sufferings of affected patient. Most of the patients die before forty years of age.
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