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Paget's disease, otherwise known as osteitis deformans, is a chronic disorder that typically results in enlarged and deformed bones. It is named after Sir James Paget, the British surgeon who first described this disease. The excessive breakdown and formation of bone tissue that occurs with Paget's disease can cause bone to weaken, resulting in bone pain, arthritis, deformities, and fractures. Paget's disease may be caused by a slow virus infection, present for many years before symptoms appear. There is also a hereditary factor since the disease may appear in more than one family member.Paget's disease is rarely diagnosed in people under 40 years of age. Men and women are affected equally. Prevalence of Paget's disease ranges from 1.5 to 8 percent depending on age and country of residence. Prevalence of familial Paget's disease (where more than one family member has the disease) ranges from 10 to 40 percent in different parts of the world. Because early diagnosis and treatment is important, after age 40, siblings and children of someone with Paget's disease may wish to have an alkaline phosphatase blood test every 2 or 3 years. If the alkaline phosphatase level is above normal, other tests such as a bone-specific alkaline phosphatase test, bone scan , or x-ray can be performed.
1 Symptoms
Many patients do not know they have Paget's disease because they have a mild case of the disease with no symptoms. Sometimes, symptoms may be confused with those of arthritis or other disorders. In other cases, the diagnosis is made only after complications have developed. Symptoms can include:
- Bone pain is the most common symptom. Bone pain can occur in any bone affected by Paget's disease. It often localizes to areas adjacent to the joints.
- Headaches and hearing loss may occur when Paget's disease affects the skull.
- Pressure on nerves may occur when Paget's disease affects the skull or spine.
- Increased head size , bowing of limb , or curvature of spine may occur in advanced cases.
- Hip pain may occur when Paget's disease affects the pelvis or thighbone .
- Damage to cartilage of joints may lead to arthritis.
2 Diagnosis
Paget's disease may be diagnosed using one or more of the following tests:
- Pagetic bone has a characteristic appearance on x-rays.
- An elevated level of alkaline phosphatase in the blood can be suggestive of Paget's disease.
- Bone scan s are Useful in determining the extent and activity of the condition. If a bone scan suggests Paget's disease, the affected bone(s) should be x-rayed to confirm the diagnosis.
3 Prognosis
The outlook is generally good, particularly if treatment is given before major changes in the affected bones have occurred. Any bone or bones can be affected, but Paget's disease occurs most frequently in the spine, skull, pelvis, thighs, and lower legs. In general, symptoms progress slowly, and the disease does not spread to normal bones. Treatment can control Paget's disease and lessen symptoms but is not a cure. Osteogenic sarcoma , a form of bone cancer, is an extremely rare complication that occurs in less than one percent of all patients.
4 Other medical conditions
Paget's disease may lead to other medical conditions, including:
- Arthritis: Long bones in the leg may bow, distorting alignment and increasing pressure on nearby joints. In addition, Pagetic bone may enlarge, causing joint surfaces to undergo excessive wear and tear. In these cases, pain may be due to a combination of Paget's disease and osteoarthritis.
- Loss of hearing in one or both ears may occur when Paget's disease affects the skull and the bone that surrounds the inner ear. Treating the Paget's disease may slow or stop hearing loss. Hearing aids may also help.
- Heart disease: In severe Paget's disease, the heart works harder to pump blood to affected bones. This usually does not result in heart failure except in some people who also have hardening of the arteries.
- Kidney stones are somewhat more common in patients with Paget's disease.
- Nervous system: Pagetic bone can cause pressure on the brain, spinal cord, or nerves, and reduced blood flow to the brain and spinal cord.
- Sarcoma: Rarely, Paget's disease is associated with the development of a malignant tumor of bone. When there is a sudden onset or worsening of pain, sarcoma should be considered.
- When Paget's disease affects the facial bones, the teeth may become loose. Disturbance in chewing may occur.
- Rarely, when the skull is involved, the nerves to the eyeThis article refers to the sight organ. See Eye (disambiguation) for other usages. human eye. Note that not all eyes have the same anatomy as a human eye. An eye is an organ that detects light. Different kinds of light-sensitive organ are found in a varie may be affected, causing some loss of vision .
Paget's disease is not associated with osteoporosisOsteoporosis is a disease of bone in which the amount of bone is decreased and the strength of trabecular bone is reduced, cortical bone becomes thin and bones are susceptible to fracture. It is defined according to the bone mineral density (BMD) as measu. Although Paget's disease and osteoporosis can occur in the same patient, they are completely different disorders. Despite their marked differences, several treatments for Paget's disease are also used to treat osteoporosis.
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