Paget disease


Paget disease

Description, Causes and Risk Factors:

Alternative Name: Osteitis deformans

Bone is a living, active tissue that is constantly renewing itself. Old bone is broken down by cells called osteoclasts and replaced by new bone produced by cells called osteoblasts. This turnover of bone is finely balanced; otherwise bones would be much more prone to fracture. Paget's disease is the one, which alters the way new bone develops. The disease is the second most common bone disease after osteoarthritis.

Paget's disease causes bones to grow too large and weak. You can have Paget's disease in any bones in your body, but most people have it in their spine, pelvis, skull, or leg bones.

The disease occurs worldwide and affects both genders, is rarely found in people under the age of 40, and occurs in up to 5% of the European population beyond that age. Epidemiology has revealed that hereditary (genetic) factors are important in Paget's disease. This is reflected by the fact that up to 15 % individuals who suffer from it also have an affected family relative.

The cause of Paget's disease is not entirely known, but it is thought to be caused in part from a childhood virus. A virus particle, known as a paramyxovirus nucleocapsid (a genus of viruses family Paramyxoviridae that includes parainfluenza viruses (types 1 and 3) has been identified within the bone cells of individuals with Paget's disease and in some cases even measles. This virus particle is not found in normal bone. While this relationship has been identified, a clear connection between the virus and the cause of Paget's disease is not known.

An estimated 15% of people who have Paget's disease have a close family member who also has the condition. If one has a first degree relative with Paget's disease, such as a parent, brother or sister, risk is elevated and a person is seven to eight times more likely to develop the condition.

Symptoms:

Symptoms may include:

    Severe and persistent bone pain.

  • Joint pain or stiffness.

  • Bowing of the legs and other visible deformities.

  • Fracture.

  • Warmth of skin over the affected bone.

  • Neck pain.

  • Reduced height.

  • Enlarged head.

  • Skull deformities.

  • Headache.

  • Hearing loss.

Diagnosis:

Paget's is rarely discovered in individuals before they reach the age of 40, and the number of people identified increases in each progressive age group. Typically, it is the appearance of the bones on an X?ray that signals the physician to make the diagnosis. Blood tests taken most often will indicate an increase in serum alkaline phosphatase (SAP), which is reflective of the rapid new bone turnover. Urine test results also will indicate the speed at which this rebuilding is taking place.

Physicians usually obtain a non?invasive bone scan to determine the extent of bone involvement. Only if cancer is suspected will it be necessary to biopsy the bone to examine it under a microscope.

Treatment:

Treatment approaches can focus on providing physical assistance, including the addition of wedges in the shoe, canes as walking aids and the administration of physical therapy.

Pharmacological Approach: Medications that help reduce the pain associated with Paget's include acetaminophen and anti-inflammatory drugs such as ibuprofen and naproxen. In addition, a group of medications called bisphosphonates reduce the pain and help the body regulate the bone-building process to stimulate more normal bone growth.

Your physician may also prescribe an oral medication:

    Risedronate (Actonel) to be taken by mouth every day for 2 months.

  • Alendronate (Fosamax) or etidronate (Didronel) to be taken by mouth every day for 6 months.

  • Tiludronate (Skelid) to be taken by mouth every day for 3 months.

Side effects of these medicines may involve heartburn and sometimes increasing bone pain for a short period of time.

There are also injectable medications. They may include:

    Pamidronate (Aredia), which is injected in the vein once a month or once every few months. The injection takes a few hours. Unusually, there can be inflammation of the eye or loss of bone around the teeth (osteonecrosis).

  • Zoledronate (Reclast), which is injected in the vein once a year. The injection takes less than 30 minutes. Risks and benefits must be discussed with the physician.

  • Calcitonin, a hormone that is injected under the skin several times a week. Risks and benefits must be discussed with the physician.

Surgery: Sometimes surgery is needed if there is a significant bone deformity or if there is a break in the bone. Fractures are most common in the femur (thigh bone) and tibia (shin bone), and are usually treated with an intramedullary rod, a rod that is inserted within the marrow cavity in the center of the bone. Unfortunately, fractures often take a long time to heal in patients with Paget's disease because of the abnormalities in bone turnover.

Diet: People with Paget's disease do not need a special diet. But, to maintain strong bones, you should get 1,200 mg of calcium and at least 400 IU of vitamin D everyday.

Exercise: Exercise helps build strong bones, prevents weight gain, and keeps joints mobile. Before starting a new exercise plan, talk with your doctor.

Disclaimer: The above information is educational purpose. The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition.

DISCLAIMER: This information should not substitute for seeking responsible, professional medical care.

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