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Clinical Problem Solving: Radiology
September 2001

Diagnosis Radiology Quiz Case

Author Affiliations


Arch Otolaryngol Head Neck Surg. 2001;127(9):1138-1139. doi:

Paget disease of the bone (osteitis deformans) is characterized by an abnormally rapid rate of bone turnover. Repeated episodes of osteoclast-mediated resorption are followed by exaggerated repair attempts by osteoblasts, culminating in an overall weakened and deformed skeleton. The primary defect is thought to occur in the osteoclasts, which are increased in number, size, and activity and have an increased number of nuclei. Defects in the osteoclast precursor, osteoclast hypersensitivity to 1,25(OH)2D3, an osteoclastogenic bone marrow microenvironment, and a defect in the osteoblasts are all currently thought to be involved in the cell biology of Paget disease.1,2 The role of a slow viral infection in the pathogenesis of Paget disease has been proposed as a result of studies that have demonstrated the presence of paramyxoviral nuclear inclusions and antigens in osteoclasts taken from pagetic lesions.3,4 A genetic link for Paget disease has been hypothesized because 25% of the patients have a family history that is positive for the disease. Also, a predisposition gene located on chromosome 18q has been associated with Paget disease, but studies have shown a variable penetrance, making a multifactorial role for the pathogenesis of the disease more likely.5

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