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osseous Paget disease

MIM.602080

Tuesday 27 September 2005

Paget disease, originally described by Sir James Paget in 1877, is a focal disorder of bone remodelling, involving increased bone resorption and formation.

The etiology is uncertain but both environmental and genetic factors are thought to be involved in pathogenesis. Recent study suggested that sporadic Paget disease of bone may due to somatic mutations in SQSTM.

It is generally considered when there is radiographic evidence of cortical destruction, expansion of bone, coarsened trabeculae, and/or bone sclerosis.

Paget disease usually affects bones of the skull, spine, pelvis, and lower extremities, but it also reported in hand.

Paget disease is associated with significant disability, impaired quality of life and a variety of complications such as pathological fracture, arthritis in adjacent joints, hearing loss, other neurological and cardiac complications, and lastly, secondary osteosarcoma.

Etiology

- germline mutations of SQSTM1 in Paget disease of bone (MIM.602080)

Case reports

- [UPMC #598-­>http://path.upmc.edu/cases/case598/dx.html] (osteosarcoma)

Videos

- Paget disease of bones by Washington Deceit

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