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Paget's Disease.

Last updated Monday, December 27, 2004

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Causes and effects

Causes

The cause of Paget's disease is unknown. One of the most accepted theories points to an early viral infection of the bone. It is thought that this infection lies inactive for many years, only to surface later in life as Paget's disease.

Effects

To understand what happens in Paget's disease, it is helpful to understand the difference between normal bone cell activity and Pagetic bone cell activity. Bone is active and living tissue that constantly is being remodeled in a three-stage process. First, old bone is resorbed (broken down) by cells called osteoclasts to make way for new bone. Next, cells called osteoblasts lay down an orderly framework for new bony tissue. Finally, the bone hardens when calcium is added to the framework laid down by the osteoblasts.

Normal bone cell activity is a very controlled process that is necessary for bone development and repair throughout life. The bone turnover process slows somewhat after puberty and continues at a slower rate through adult life. (This is why an older person's bones break more easily.)

Bone affected by Paget's disease is broken down faster than usual. Consequently, new bone formation increases. Because the new bone forms so rapidly, its structure becomes jumbled and disorganized. Extra blood vessels appear, and the new formation becomes bulky and somewhat softer than normal bone. This can result in joints near the affected area developing arthritis.

Because the bones are weaker than normal, they may break or fracture more easily. The thigh or leg can become bowed. If the vertebrae (bones of the spine) are involved, they may collapse, making the spine curve forward.

A person may experience other forms of discomfort as well. The increased flow of blood through the bone may cause a feeling of warmth over the affected areas. Thickened bones in some locations can pinch nerves. For example, pinched nerves in the spine may cause sciatica, a syndrome of radiating pain from the back into the buttocks and down the legs. Paget's disease of the bones inside the ears also can cause ringing or a decrease in hearing.

Eventually, the rapid bone breakdown and disorganized bone formation slows down, and Paget's disease appears to "burn out." Symptoms may come and go. However, any bone enlargement or bowing that has occurred will remain.


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