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