Paget disease is a disorder of bone of unknown aetiology with increased bone turnover that results in defective bone microarchitecture. Defective microarchitecture results in deformity in skull and long bones, arthritis, deafness and pathologic fractures. Therefore, quality of life in patients with Paget disease is worse than normal population. Peer review refers to the work done during the screening of submitted manuscripts and funding applications. This process encourages authors to meet the accepted standards of their discipline and reduces the dissemination of irrelevant findings, unwarranted claims, unacceptable interpretations, and personal views. Publications that have not undergone peer review are likely to be regarded with suspicion by academic scholars and professionals.
The most likely aetiology is a slow paramyxoviral infection in genetically susceptible individuals; however, the exact cause is unknown. Enhanced bone resorption due to an increased activity of osteoclasts recruits numerous osteoblasts to resorption sites, with large quantities of new bone matrix produced as a result. The accelerated bone resorption and formation are not as closely coupled as in a healthy bone; a disorganized bone tissue is formed. Many patients are asymptomatic; rising serum alkaline phosphatase or incidental finding of characteristic radiographic lesions are often the only diagnostic clues. Common clinical manifestations include bone pain, bowing of long bones, enlarged skull, and hearing loss. An elevated serum alkaline phosphatase level correlates with the disease activity. The diagnosis is confirmed by characteristic radiographic findings and by nuclear scintigraphy of the bone (the most sensitive test). Bisphosphonates, such as zoledronic acid, pamidronate, alendronate, and risedronate, are the mainstay of treatment. Patients who do not tolerate bisphosphonates can be treated with calcitonin.