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Paget’s disease

A widespread disorder that arises in elderly and middle-aged people that is marked by the disruption of bone formation. The affected bones become enlarged, weak, and thickened, and deformed. Paget’s disease is occasionally called osteitis deformans.

Paget’s disease mostly affects the skull, collarbone, pelvis, vertebrae, and long bones of the leg. Usually, the upkeep of healthy bones involves a balance between the actions of the cells that break down bone tissue and the activity of the cells that make new tissue. In Paget’s disease this balance is disrupted. The disorder can run in familys and in certain cases is believed to be caused by a viral infection.

Symptoms

There are usually no symptoms. If symptoms do arise, the most frequently seen ones are bone deformity and pain, particularly bowing of the legs. Afflicted bones become susceptible to fracture.

Skull changes can lead onto leontiasis ossea (distortion of the facial bones giving a lionlike appearance) and cause injury and damage to the inner ear bones, occasionally leading to deafness, vertigo (a spinning sensation), tinnitus (ringing in the ears), or headache.

In the spine, enlarged vertebrae can press on the spinal cord, causing pain and discomfort and occasionally paralysis of the legs. If the disease affects the pelvis, it can cause  severe arthritis of the hip joints.

Sometimes bone cancer can develop. In unusual cases, when a number of bones are involved, increased blood flow through affected bones can cause heart failure.

Diagnosis

Paget’s disease is diagnosed by X-rays which show areas of pourous and thickened bone, alongside blood tests, which show abnormal levels of the substances involved in bone formation breakdown.

Many people do not require treatment, or need only analgesic drugs (pain killers).  In more extreme cases, treatment with drugs such as bisphosphonapes or calcitonin can be administered to control the rates of bone breakdown and renewal.  The drugs will not reverse any existing deformity, but will slow the progression of the disease.  Surgery can be required to treat arthritis or correct deformities.


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