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Medical Journal of Australia

Source: www.mja.com.au
Topic: Paget's disease of bone
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Sort Desciption: Pagets disease of bone is a chronic disorder, characterised by focal areas of excessive osteoclastic bone resorption accompanied by a secondary increase in osteoblastic bone formation. ...

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Pagets disease of bone is a chronic disorder, characterised by focal areas of excessive osteoclastic bone resorption accompanied by a secondary increase in osteoblastic bone formation. The classic description of the disease was by Sir James Paget in 1877 in a paper describing the clinical features of a disorder he called osteitis deformans. 1 The disease results in bone expansion and structural weakness, which can cause pain, deformity, and a range of complications. Here, I review recent advances in understanding of the disease, as well as practical management. For more detailed reviews, see two recently published sets of consensus management guidelines. 2,3 Epidemiology and pathogenesis of Pagets disease Pagets disease predominantly affects the elderly. It is uncommon in people under the age of 55 years and is more common in males than females, with a sex ratio of 1.8 : 1. There are marked geographical differences in prevalence. The disease is most common in the United Kingdom (particularly Lancashire), followed by Australia, New Zealand and North America, in part reflecting the large number of British migrants to those countries. 4 By contrast, the disease is uncommon in Scandinavia, Africa and Asia. A radiographic study carried out in Western Australia in 1978 reported a prevalence of 4% in people aged over 55 years. 5 Recent studies from the United Kingdom and New Zealand suggest that the incidence of Pagets disease is falling, perhaps by as much as 50% over the past 20 years, and that the severity of newly diagnosed cases is falling. 6,7 The reason for this is unknown. There are no recent prevalence studies from Australia. The cause of Pagets disease is unknown. There is a strong genetic component, and 15%20% of ...

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