| Paget's disease can cause bones to expand and | | | | age. This type of malignant bone tumor is very |
| weaken and may result in bone pain, arthritis, bone | | | | aggressive and carries a poor prognosis. |
| deformity and fractures. | | | | Paget's disease occurs most frequently in the spine, |
| Paget's disease is the second most common bone | | | | skull, pelvis, thighs, and lower legs. Paget's disease |
| disease. Paget's disease is a chronic bone disorder | | | | may be caused by a slow virus infection (i.e., |
| that is due to irregular breakdown and formation of | | | | paramyxoviruses such as measles and respiratory |
| bone tissue.The disease occurs worldwide, but is | | | | syncytial virus). Paget's disease of bone, though they |
| more common in Europe, Australia, and New Zealand | | | | have discovered several genes that appear to be |
| -- where it is seen in up to 5% of the elderly | | | | linked to the disorder. Hereditary factors seem to |
| population. Men and women are approximately equally | | | | influence whether you're susceptible to the disease. |
| affected. The prevalence of Paget's disease ranges | | | | Environmental factors may also play a role. Several |
| from 1.5 to 8% depending on age and country of | | | | studies have postulated that viruses, particularly |
| residence. The prevalence of familial Paget's disease | | | | paramyxoviruses such as canine distemper or |
| (where more than one family member has the | | | | measles virus, play a role in pathogenesis, but |
| disease) ranges from 10 to 40% in different parts of | | | | definitive evidence for this is lacking. Presumably, the |
| the world. It is usually localized to one bone, but can | | | | declining incidence of Paget's disease reflects a decline |
| involve many bones. The actual cause of Paget's | | | | in one or more as yet unidentified environmental |
| disease is not known. Paget's disease is also known | | | | influences. The SQSTM1/p62 protein is a selective |
| as osteitis deformans. The disease may localize to | | | | activator of the transcription factor NFB, which plays |
| one or two areas within the skeleton, or become | | | | an important role in osteoclast differentiation and |
| widespread. Frequently, bones of the pelvis, leg, | | | | activation in response to the cytokines RANK-ligand |
| spine, arm, or the collar bone are involved. The | | | | and interleukin-1.Mutations in the SQSTM1/p62 gene |
| effect on the skull may enlarge head size and cause | | | | are therefore a plausible cause of Paget's disease. |
| hearing loss, if the cranial nerves are damaged by the | | | | Germline DNA mutations (present in every |
| bone growth. | | | | osteoclast) cause bone disease that is focal in nature. |
| Paget's disease may affect only one or two areas of | | | | Medical are generally treating Paget's disease. |
| your body, or may be widespread. Paget's disease | | | | Treatment include is aspirin, other anti-inflammatory |
| tends to appear in families. It can be present in as | | | | medications, pain medications, and medications that |
| many as 25 percent to 40 percent of the relatives | | | | slow the rate of bone turnover, such as calcitonin |
| of someone with the disease. It is also more | | | | (Calcimar, Miacalcin), etidronate (Didronel), alendronate |
| common in people of Anglo-Saxon descent. Many | | | | (Fosamax), and pamidronate (Aredia). Surgery is used |
| patients do not know they have Paget's disease | | | | mainly to treat the complications of Paget's disease. |
| because they have a mild case with no symptoms. | | | | A newer drug, risedronate (Actonel), appears to |
| Sometimes, symptoms may be confused with those | | | | have a powerful effect against severe Paget's |
| of arthritis or other disorders. Symptoms can arise | | | | disease. Miacalcin is administered by injection; 50 to |
| from the effect on calcium levels in the blood stream. | | | | 100 units daily or 3 times per week for 6-18 months. |
| When Paget's disease is active in several bones, the | | | | Didronel (etidronate disodium) -- Tablet; approved |
| overactive osteoclasts can release enough calcium | | | | regimen is 200-400 mg once daily for 6 months; the |
| from the bone as they break it down to cause an | | | | higher dose (400 mg) is more commonly used; no |
| elevated calcium level in the blood. This rare | | | | food, beverages, or medications for 2 hours before |
| complication might cause fatigue, weakness, loss of | | | | and after taking. Fosamax (alendronate sodium) -- |
| appetite, abdominal pain, or constipation. A doctor can | | | | Tablet; 40 mg once daily for 6 months; patients |
| also detect Paget's disease from its effect on the | | | | should wait at least 30 minutes after taking before |
| heart if excess blood supply is shunted to overactive | | | | eating any food, drinking anything other than tap |
| bones in severe cases. When pain is severe and | | | | water, taking any medication, or lying down (patient |
| unrelenting in an area affected by Paget's disease, | | | | may sit). Eating a healthy diet with sufficient calcium |
| the disease may have degenerated into a bone | | | | and vitamin D and getting exercise are important |
| cancer. Paget's sarcoma (Figure 6) occurs in only | | | | components in maintaining skeletal health and joint |
| about 1 percent of patients with Paget's disease. | | | | mobility. |
| These patients are usually older than 70 years of | | | | |