Pagets disease disease of unknown etiology involving destruction and reparation

Contributor: Christopher J Bennett - National Capital Consortium Paget's disease at osseous scintigraphy Factoid 4755 - Created: 2003-04-24 12:50:56-04 - Modified: 2003-05-04 19:15:37-04 ACR Codes: 4.5 Paget’s disease is characterized by high rates of bone turnover and the formation of abnormal, weak bone. It is extremely common, found in approximately 10% of patients over 80 years of age. Classically, the course of Paget’s disease can be divided into lytic, mixed, and blastic phases, but in reality a continuum of pathologic changes are present. The pelvis, spine, skull and proximal long bones are frequently affected. Patients are often asymptomatic, but pain, fracture, bowing deformity and a variety of other complications occur. Sarcomatous degeneration (to osteosarcoma, chondrosarcoma, or malignant fibrous histiocytoma/fibrosarcoma) is thought to occur in 1% of cases. Expansion of bone, cortical thickening, and coarsened trabeculae are the radiographic hallmarks. Osseous scintigraphy is useful to define polyostotic disease. Intense uptake is seen in affected bones, but expansion may be difficult to appreciate. In quiescent disease, uptake may be minimal even if radiographic changes are pronounced.

Paget disease is a localized disorder of bone remodeling that typically begins with excessive bone resorption followed by an increase in bone formation. This osteoclastic activity followed by compensatory bone formation (osteoblastic activity) leads to a structurally disorganized mosaic of bone (woven bone), which is weaker mechanically, larger, less compact, more vascular, and more susceptible to fracture than normal adult lamellar bone. Full Text

Contributor: James H. Chang Paget Disease of Bone Factoid 3596 - Created: 2002-01-29 22:10:01-05 - Modified: 2002-04-17 09:18:33-04 ACR Codes: 4.7 Paget disease (osteitis deformans) is a condition of unknown cause affecting approximately 3 per cent of the population over age 40 years. Paget disease appears to be particularly common in Australia, Great Britain and certain areas of Europe.The disease is characterized excessive and abnormal remodeling of bone. The process of osseous resorption produces a diagnostic radiographic appearance of coarsened trabeculae.

Contributor: Sara Buckelew, medical student Paget Disease of Bone Factoid 1288 - Created: 2001-03-07 09:15:37-05 - Modified: 2001-03-14 17:01:08-05 ACR Codes: 4.84 Paget disease of bone involves localized, uncontrolled formation of highly active osteoclasts. Initial bone resorption is followed by an intense increase in osteoblastic activity. The bone that is formed is chaotic and excessive. The resulting bone has a mosaic pattern with loss of lamellar structure and therefore is weakened. The marrow is fibrotic with increased vascularity. The increase in osteolytic, osteoblastic, and vascular activity make it intensely "hot" on bone scan.

Paget disease is a metabolic bone disease characterized by excessive bone resorption and formation due to activated osteoclasts.

Paget disease of the bone (osteitis deformans) is a metabolic disorder characterized by abnormal osseous remodeling.

Paget disease, characterized by excessive and abnormal remodeling of bone, is a common disorder in middle-aged and elderly patients. The excessive remodeling gives rise to bones that are extensively vascularized, weak, enlarged, and deformed with subsequent complications.

Overview of this bone disorder including information on cause, facts and myths, symptoms, diagnosis, and treatment.

Paget's Disease of Bone Definition Incidence Aetiology Clinically Investigations Pathology Treatment Differential Diagnosis Complications Recent Literature

Clinical presentation: 80 year old lady with pain in her right hip. There is increased density of the right hemi-pelvis. The bone dimensions are increased. There is coarsening of the bone architecture with loss of detail. There is protrusio acetabulae, indicating bone softening. ACR-code 448-843

Radiology case 438-843: Pagets Disease

Radiology case 468-842: _Pagets Disease Tibia

Wheeless' Textbook of Orthopaedics Discussion: - a chronic progressive disease osteoblasts and osteoclasts which results in abnormal bone remodeling; - etiology, staging, and pathogenesis - prevalence: - uncommon in pts under age of 55 yrs but relatively common in later life, occurring in 3-4% of persons over 55 yrs & occurs in 10% of population over the age of 80 yrs;

Paget's Disease Topic 2602 - Created: 2001-07-27 05:09:01-04 - Modified: 2002-04-30 23:00:33-04 ACR Index: 4.2 -------------------------------------------------------------------------------- Paget’s disease most commonly occurs in England, Australia, New Zealand, Scandanavia, Canada and the northern U.S. The average age of onset is between 50 and 55 years. It is twice as common in men as women. The cause is unknown. Pathologically, it is characterized by destruction of bone (lysis) followed by attempts at repair (blastic) which are often disorganized. Paget’s disease may affect any bone in the body. It may affect a single bone and never extend to others, or it may spread. In order of frequency, the following bones are affected: pelvis, vertebrae, femur, skull, tibia, clavicle, humerus, ribs, and rarely the sternum, calcaneous, talus, phalanges, metatarsals, mandible, patella, and other sesamoid bones. Only 20% of the patients are symptomatic, usually complaining of ill-defined pain at the site. Characteristically, there is an elevation of alkaline phosphatase (15-20x normal). Serum calcium and phosphorous are usually normal, but serum calcium may be elevated in an immobilized patient.