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VIII.100 - Paget’s Disease of Bone

from Part VIII - Major Human Diseases Past and Present

Published online by Cambridge University Press:  28 March 2008

Kenneth F. Kiple
Affiliation:
Bowling Green State University, Ohio
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Summary

Paget’s disease of bone was described as “osteitis deformans,” a “chronic inflammation of bone” by Sir James Paget in an address to the Royal Medical Chirurgical Society of London in 1876.

His original description was masterful and thus has withstood the test of time. Paget’s disease of bone describes an abnormal osseous (bony) structure whereby isolated and sometimes contiguous areas of the skeleton undergo changes leading to clinical deformity for some of those affected. Clinically affected people may have the appearance of enlarged bone, bowed extremities, shortened stature, and simian posturing because the body’s usual system for maintaining strong and healthy bone malfunctions. Normal bone turnover is altered in the affected areas. The resorption process accelerates, and the repair process responds by building a heavy, thickened, and enlarged bone. Although the new bone contains normal or increased amounts of calcium, the material of the bone is disorganized, and the bone is structurally weak. The result may be pain, deformity, fracture, and arthritis.

Distribution and Incidence

The disease appears to have its greatest prevalence in Europe and in regions inhabited by European emigrants such as Australia, New Zealand, and areas of South America. For no apparent reason, the disease is distinctly uncommon in African blacks, Orientals, and inhabitants of India and Scandinavia. Where studied, the incidence ranges between 3.5 and 4.5 percent of the population in high prevalence regions of the world, with a high of 8.3 percent in a part of Lancashire, England, to a low of 0.4 percent in Sweden.

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Publisher: Cambridge University Press
Print publication year: 1993

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References

Altman, R. D., and Collins, B.. 1980. Musculoskeletal manifestations of Paget’s disease of bone. Arthritis and Rheumatism 23.CrossRefGoogle ScholarPubMed
Barry, H. C. 1969. Paget’s disease of bone. Edinburgh and London.Google Scholar
Cullen, P., et al. 1976. Frequencies of HLA-A and HLA-B: Histocompatibility antigens in Paget’s disease of bone. Tissue Antigens 7.Google ScholarPubMed
Dickson, D. D., et al. 1945. Osteitis deformans: Paget’s disease of bone. Radiology 44.CrossRefGoogle Scholar
Fotino, M., et al. 1977. Evidence for linkage between HLA and Paget’s disease. Transplant Proceedings 9.Google ScholarPubMed
Howatson, A. F., and Fornasier, V. L.. 1982. Microfilaments associated with Paget’s disease of bone: Comparison with nucleocapsids of measles virus and respiratory syncytial virus. Intervirology 18.CrossRefGoogle ScholarPubMed
Mills, B. J., et al. 1980. Cell cultures form bone affected by Paget’s disease. Arthritis and Rheumatism 23.CrossRefGoogle Scholar
Mills, B. J., et al. 1984. Evidence for both respiratory syncytial virus and measles virus antigens in the osteoclasts of patients with Paget’s disease of bone. Clinical Orthopaedics and Related Research 183.Google Scholar
Morales, A., et al. 1980. Manifestaciones articulares de la enfermedad de Paget. Revisión de 84 casos. Revista Clínica Española 159.Google Scholar
Paget, J. 1877. On a form of chronic inflammation of bones (osteitis deformans). Medico–Chirurgical Transactions 60.Google Scholar
Rebel, A., et al. 1974. Particularités ultrastructuales des osteoclastes de la maladie de Paget. Revue du Rhumatisme et des Maladies Osteo–articulaires 41.Google Scholar
Rebel, A., et al. 1980. Viral antigens in osteoclasts from Paget’s disease of bone. Lancet 2.Google ScholarPubMed
Rosenbaum, H. D., and Hanson, D. J.. 1969. Geographic variation in the prevalence of Paget’s disease of bone. Radiology 92.CrossRefGoogle ScholarPubMed
Sofaer, J. A., et al. 1983. A family study of Paget’s disease of bone. Journal of Epidemiology and Community Health 37.CrossRefGoogle ScholarPubMed
Tilyard, M. W., et al. 1982. A probable linkage between familial Paget’s disease and the HLA loci. Australian and New Zealand Journal of Medicine 12.CrossRefGoogle ScholarPubMed

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