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Paget’s Disease Of Bone Revisited: The Changing Indian Scenario
Published 2020 · Medicine
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Classically described as a disorder in which bone remodeling has “gone awry,” Paget’s disease of bone (PDB) (osteitis deformans) is the paradigm of excessive osteoclastic resorption followed by secondary bone deposition by osteoblasts. A focal disorder of bone metabolism, PDB, may demonstrate a monostotic or polyostotic involvement with the commonly affected bones being the skull, spine, pelvis, and long bones of the lower extremities resulting in compromised skeletal integrity. PDB may be familial or sporadic, with both genetic and environmental determinants contributing to its pathogenesis. Among the many genetic factors implicated in the causation of PDB, those directly affecting the receptor activator of nuclear factor kappa-B (RANK)-RANK ligand pathway such as mutations in the tumor necrosis factor receptor superfamily member 11A, the ubiquitin-associated locus of sequestosome 1, zinc finger protein 687, and optineurin have been associated with Paget’s disease. As compared to genetic influences, there is limited information on the role of putative environmental triggers in disease causation. Few studies suggest a plausible viral etiology based on the presence of intranuclear inclusions in pagetic osteoclasts that resemble paramyxoviruses.