Paget's disease of bone

Abstract

Paget's disease of bone is a focal disorder of bone remodeling accompanied initially by an increase in bone resorption, followed by a disorganized and excessive formation of bone, leading to pain, fractures and deformities. It exhibits a marked geographical variation in its prevalence. In Brazil it predominantly affects persons of European descent. The majority of the reported cases of the disease in Brazil are from Recife, owing to its peculiar mixed European colonization over approximately four centuries. The etiology is complex and involves both genetic and environmental factors. The disease is often asymptomatic and diagnosis is usually based on biochemical markers of bone turnover, radionuclide bone scan and radiological examination. Bisphosphonates, in particular zoledronic acid, are regarded as the treatment of choice for Paget's disease of bone.

Epidemiological features and the neuropathological manifestations of canine distemper virus-induced infections in Brazil: a review

“…PDB was first described by Sir James Paget in 1877, and considered as prevalent in elderly populations of Europe, USA, and Australia . In Brazil, the occurrence of PDB seems to be restricted to Recife/ PE, with predominance in patients of European descent (GRIZ et al, 2006); since cases have not been described in other geographical regions. The true etiology of PDB is currently unknown, but studies have implicated paramyxoviruses, including CDV, measles virus (MV), respiratory syncytial virus, parainfluenza virus-3, and simian virus (MEE; SHARPE, 1993) as possible etiologic agents.…”

Diagnosis and management of Paget?s disease of bone

“…Serum alkaline phosphatase has a sensitivity of 78%, and a specificity of almost 100%. However it may be normal in up to 20% of patients with monostotic disease (4,56,57). In a study by Bandeira and cols.…”

“…The highest prevalence of Paget's disease of bone is found in England, the United States, Australia and New Zealand, mainly among patients older than 55 years (4). In other locations, such as Asia, it is a rare disorder (6,7) (C4), as it is in Scandinavia (4) and most of Latin America (8) (C4).…”

“…The role of SQSTM1/p62 has yet to be fully elucidated. There is evidence that mu tations may reduce the ability to sequester cytoplasm proteins, altering nuclear factor kB (NFkB), resulting in increased osteoclastogenesis (4). The action of os teoprotegerine (OPG) on the RANK receptor has also been described.…”

Genetic aspects of the Paget’s disease of bone: concerns on the introduction of DNA‐based tests in the clinical practice. Advantages and disadvantages of its application

“…In extra-European countries, such as Australia, New Zealand, North America, Argentina and Brazil, the epidemiology of PDB closely parallels patterns of migration from Western Europe, exhibiting a relatively high prevalence in descendants of the large number of migrants coming from: (i) the United Kingdom for the English-speaking countries [Australia, New Zealand and North America (New England)] [46,47]; (ii) France, with a higher prevalence in the French-Canadian population from Quebec than other Canadian regions; (iii) Italy, with a higher prevalence within the Italian descent population in Argentina; and (iv) from the Jewish population originally settled in Recife, Brazil [48,49].…”