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