Paget's disease of bone

Paget's disease of bone

    * Introduction
    * Pathogenesis and causes
    * Signs and symptoms
    * Diagnosis
    * Treatment
Paget's disease of bone or deforming osteitis is a localized disorder of bone remodeling that typically begins with excessive bone resorption followed by excessive proliferation of bone. The osteoclastic activity followed by compensatory bone formation (osteoblast activity) leads to a disorganized patchwork of bone that is mechanically weaker, larger, less compact, more vascular and more succeptibil fracture from normal adult lamellar bone. Sir James Paget first described as inflammation of the bone osteitis deformans in 1877. Paget's disease, the condition is called today as the second most common cause of bone disease after osteoporosis in older people.
Approximately 70-90% of people with Paget disease are asymptomatic, though a minority of affected persons accused various symptoms, including bone pain, the most common symptom, secondary osteoarthritis when Paget's disease affects an area adjacent joints, bone deformation, excessive local heat hipervascularizare and neurological complications by compression of nerve tissue. Paget's disease can be monostotic, but is more frequently multifocal, with predilection for the axial skeleton: spine, pelvis, femur, sacrum and skull. Any bone can be affected, however. After onset Paget's disease of the bone does not extend to the bone, but can become progressive and worsen pre-existing location.
Although the etiology is unknown and genetic factors have been proposed of the environment. Etinice and geographic clusters of disease are well known. Paget's disease is common in Europe, North America, Australia and rare in Asia and Africa. Approximately 40% of patients described a family history of disease. Some studies suggest a genetic link to chromosome arm 18q disease, although this has not been demonstrated in many families with Paget's disease, suggesting genetic heterogeneity. An exogenous factor was considered but never proved in the pathogenesis of the disease. The results of bone biopsies from patients with Paget's disease show different Paramyxoviridae viral antigens, including chicken pox and respiratory syncytial virus, localized in osteoclasts. However trigger antigen still remains unknown.
Paget disease morbidity may be extensive and result in bone pain, osteoarthritis and fractures. Increasing mortality associated with Paget's disease is due to disease complications, especially those associated with fracture and sarcoma. Most patients with Paget's disease who develop sarcoma die within three years after diagnosis. Cause excessive bleeding after Hipervascularizarea bone fractures or surgery and is associated with potentially severe consequences.
Treatment aims to control disease activity and reducing or preventing the progression of pathogenic complications. Indications for treatment include complications of Paget's disease: skeletal deformities, bone pain, congestive heart failure, hypercalcemia, spinal cord compression and nerve radacinolor, eight cranial nerve compression or bone of the optic nerve, the formation of kidney stones by hypercalcemia or recurrent fractures. When Paget's disease occurs around a joint treatment is administered in an attempt to prevent the development of osteoarthritis. Also younger patients with Paget's disease are treated to prevent future complications. Medical therapy includes administration of bisphosphonates and bone markers for monitoring series. Due to increased risk of malignancy, Paget's disease patients should be monitored indefinitely. Steroidal anti-inflammatory drugs and acetaminophen are effective pain control. Orthopedic devices, and the walking sticks are useful in patients with Paget's disease affecting the lower limbs and have weight problems. Chemotherapy, radiotherapy or both are used to treat Paget's bone neoplasms occurring.