While there are now safe and highly effective treatments, it remains an enigmatic condition with distinctive characteristics that are poorly understood. Because the bones regrow too quickly, they are bigger and softer than normal. In healthy bone, a process called remodeling removes old pieces of bone and replaces them with new bone. Paget s disease of bone disrupts the normal cycle of bone renewal, causing bones to become weakened and possibly deformed. Paget disease of the bone what is paget disease of the bone. Pagets disease pd is a focal disorder of bone remodelling that occurs commonly in older people. Definition paget s disease of bone osteitis deformans is a disease of bone marked by repeated episodes of increased bone resorption followed by excessive attempts at bone formation, resulting in weakened and deformed bones of increased mass.
Paget s disease may affect only one bone or several fig. Paget disease of bone, also known as osteitis deformans, is a focal disorder of bone metabolism characterized by an accelerated rate of bone remodeling, resulting in overgrowth of bone at selected sites and impaired integrity of affected bone. Pagets disease is a chronic focal high turnover bone disorder that is primarily present in middle aged or older adults. Pagets disease causes this process to shift out of balance, resulting in new bone that is weak and brittle. Pdb can affect a single bone monostotic or multiple bones polyostotic and mainly involves the axial skeleton including the bones of the skull, spine, and pelvis, as well as the femur and sacrum, with only 17%. Paget s disease of bone is a chronic condition that causes abnormal enlargement and weakening of bones.
It usually occurs in one or a few bones and does not spread from one bone to another. Pagets disease of bone is a common disorder which may affect one or many bones. Diagnosis and treatment of pagets disease of bone aafp. Images in unusual presentation of paget s disease of bone. In 4 families with paget disease of bone and possible linkage to 18q, hughes et al. Paget s disease of bone may present with bone pain but is often asymptomatic. Pagets disease, or osteitis deformans, is a chronic metabolic disorder characterized by increased bone turnover and breakdown secondary to excessive and disorganized osteoclastic and osteoblastic activity fig. Pagets disease of bone symptoms and causes mayo clinic. Paget disease of bone is a disorder that causes bones to grow larger and weaker than normal. We present the case of a 40yearold woman with pdb affecting mainly the distal extremities. Pagets disease is a localized disorder of bone remodeling affecting one or more bones or parts of bones. Pagets disease of bone is a chronic condition that causes abnormal enlargement and weakening of bones.
Sometimes enlarged bone can press on nerves, causing pain in the back. People with pagets disease may be more susceptible to. Over time, the disease can cause affected bones to become fragile and misshapen. There are treatments that can help keep it under control for many years, but it can cause persistent pain. Stone4, on behalf of the bone and tooth society of great britain and the national association for the relief of pagets disease. But with pagets disease, this happens too fast and gives your bones an odd shape. It is a localized disease that may be unifocal, affecting a single bone, or multifocal, affecting many bones or nearly the entire skeleton. This condition can affect any of the bones in the body. Paget disease of bone, chronic disease of middle age, characterized by excessive breakdown and formation of bone tissue. The clinical presentations and treatments of pagets disease of bone are discussed in this chapter. Paget disease of bone is a disorder that involves abnormal bone destruction and regrowth, which results in deformity. The geographic and familial clusters of the disease have led to the identification of environmental and genetic determinants of paget disease. Jan 11, 2019 paget s disease of bone interferes with your bodys normal recycling process, in which new bone tissue gradually replaces old bone tissue.
Any part of the skeleton can be affected, but the most common sites include the skull, spine, pelvis, thigh bone, shin and the bone of the upper arm. Guidelines on the management of pagets disease of bone. Paget disease of bone is the most common metabolic bone disease after osteoporosis, affecting 24% of adults over 55 years of age. Paget disease of the bone is a chronic bone disorder.
Feb 25, 2019 paget s disease of the bone is a nonmalignant skeletal disorder characterized by focal abnormalities in bone remodeling at one monostotic or more polyostotic skeletal sites. Pagets disease causes this process to shift out of balance, resulting in new bone that is abnormally shaped, weak, and brittle. Although many patients are asymptomatic, a variety of symptoms and. Paget disease of bone ppt free download as powerpoint presentation. A bone scan is a test that helps identify which bones have been affected by pagets disease. Signs of pagets disease include joint and bone pain, headaches, hearing loss, enlarged bones, bowed arms or legs, weak or soft bones that bend or break, tingling and numbness. Pagets disease typically involves just one bone monostotic or a few bones poly ostotic, primarily the skull or pelvis, or a vertebra, femur, or. Pagets disease of the bone is a chronic ailment affecting the skeleton. Oct 07, 2008 pagets disease of bone osteitis deformans hosain bouallouch shen zhou university 10 maart 2006 slideshare uses cookies to improve functionality and performance, and to provide you with relevant advertising. Altman, md paget s disease affects 1% of the us population over the age of 40 years and is a chronic disorder of adult skeleton characterized by increased resorption and deposition of bone resulting in replacement of the normal matrix with softened and enlarged bone. In people with paget disease, there is an abnormal breakdown of bone tissue in specific areas. Pagets disease information booklet versus arthritis. The procedure is also a useful way to determine the extent and activity of the disease. This epidemiologic study of paget s disease of bone used data from 788 cases and 387 spouse controls to investigate the following.
Paget s disease of bone pdb is a condition of unknown etiology characterized by excessive and abnormal bone remodeling. May 14, 2019 pagets disease of the bone is a chronic ailment affecting the skeleton. Your body constantly builds new bone to replace the old. Paget s disease of bone interferes with your bodys normal recycling process, in which new bone tissue gradually replaces old bone tissue. Treatment typically with bisphosphonates is indicated in patients with pain that is localized to an affected site but. Guidelines on the management of pagets disease of bone p. Bone may become dense, but fragile, because of excessive breakdown and deformation of bone. Paget disease of bone pdb, also known historically as osteitis deformans, is a focal disorder of bone metabolism that occurs in the aging skeleton.
Paget disease of bone, also called osteitis deformans, chronic disease of middle age, characterized by excessive breakdown and formation of bone tissue. With treatment, many people can manage their symptoms, improve pain, and control the effects of the disease. Diagnosis and management of pagets disease of bone in. Paget s disease is a poorly understood condition characterized by a marked increase in turnover in certain bones within the skeleton, while other bones remain unaffected. May 15, 2002 paget s disease of bone also known as osteitis deformans is a nonmalignant disease involving accelerated bone resorption followed by deposition of dense, chaotic, and ineffectively mineralized. Pagets disease of bone is a chronic longlasting disorder that causes bones to grow larger and become weaker than normal. Paget bone disease an overview sciencedirect topics. The major techniques available for imaging patients with paget disease are radiography, bone scintigraphy, ct, and mri. Paget s disease of bone commonly known as paget s disease or, historically, osteitis deformans is a condition involving cellular remodeling and deformity of one or more bones. Feb 29, 2020 in terms of management of paget s disease of bone pdb, early diagnosis and proper management achieving remission is essential with lifelong specialist followup. Pagets disease of bone pdb is a condition of unknown etiology characterized by excessive and abnormal bone remodeling. The excessive breakdown of bone is followed by the formation of weak and abnormal bone.
The disease has become much less common over the last 50 years. Its aetiology is only partly understood, but includes both genetic and environmental factors. It is a disorder of the bone remodeling process, in which the body absorbs old bone and forms abnormal new bone. Unregulated bone turnover causes bone enlargement, deformity, fragility, compression phenomena and, rarely, malignant transformation. Paget disease of bone merck manuals consumer version. Nov 30, 2018 pagets disease is the second most common type of bone disease after osteoporosis. People with pagets disease may be more susceptible to arthritis, hearing problems, pain and general discomfort. This article outlines the clinical features, diagnosis and management options for paget disease of bone. Paget disease of bone is a focal disorder of the aging skeleton that may be expressed as a familial or sporadic disease and may affect one or many bones in a given person. Pagets disease is the second most common type of bone disease after osteoporosis. The affected bones show signs of dysregulated bone remodeling at the microscopic level, specifically excessive bone breakdown and subsequent disorganized new bone. Its a fairly common condition in the uk, particularly in older people. Paget disease can affect any bone, but the most commonly affected bones are the pelvis, thighbone femur, and skull.
Sir james paget described pagets disease of bone in 1877 and the disease is named after him. This epidemiologic study of pagets disease of bone used data from 788 cases and 387 spouse controls to investigate the following. Pagets disease of bone disrupts the normal cycle of bone renewal, causing bones to become weakened and possibly deformed. Almost any bone can be affected, but there is a predilection for the pelvis, spine, femur, tibia, and skull. Pagets disease of bone is a common disorder characterized by increased but disorganized bone remodelling. Pagets disease is a chronic disorder that can result in enlarged and misshapen bones. Pagets disease of bone is a chronic disease of the skeleton. Diagnosis and treatment of pagets disease of bone american. Pagets disease, rapid bone repair leads to bones that are too soft or enlarged. The disease is the most common bone disorder after osteoporosis in adults older than age 50. Pagets disease of bone symptoms and treatment orthoinfo.
But with paget s disease, this happens too fast and gives your bones an odd shape. Paget s disease, like osteoporosis, is a disease of older age that results in marked increases in bone fractures. It is a disorder of the bone remodeling process, in which the. Paget s disease of bone most commonly occurs in the pelvis, skull, spine and legs. Diagnosis and management of pagets disease of bone scielo. Affected bones may be misshapen and easily broken fractured. Pagets disease of bone may present with bone pain but is often asymptomatic. Paget s disease is a chronic disorder that can result in enlarged and misshapen bones. Some patients are asymptomatic but others present with bone pain or other complications such as fracture and deformity. If the serum alkaline phosphatase level is elevated, a bone scan can be per. Pagets disease of bone signs of pagets disease include joint and bone pain, headaches, hearing loss, enlarged bones, bowed arms or legs, weak or soft bones that bend or break, tingling and numbness. Paget s disease of the bone is a nonmalignant skeletal disorder characterized by focal abnormalities in bone remodeling at one monostotic or more polyostotic skeletal sites.
In a bone scan, a safe amount of a radioactive substance is injected into the persons vein. Pagets disease of bone is characterized by highly localized areas of increased bone resorption accompanied by exuberant, but aberrant new bone formation. Pagets disease of bone is the second commonest metabolic bone condition after osteoporosis. Learn vocabulary, terms, and more with flashcards, games, and other study tools. Normally, there is a process in which your bones break down and then regrow. Pagets disease of the bone is a nonmalignant skeletal disorder characterized by focal abnormalities in bone remodeling at one monostotic or more polyostotic skeletal sites.
Paget s disease of bone is a chronic bone disorder. Treatments can include medications, surgery, and lifestyle changes to increase bone health, such as exercise. Diagnosis and management of pagets disease of bone in adults. Paget disease of bone is a chronic disorder of the skeleton in which areas of bone undergo abnormal turnover, resulting in areas of enlarged and softened bone. Siris, in the bone and mineral manual second edition, 2005. Imaging of paget disease of bone and its musculoskeletal. Pagets disease of bone pdb is a chronic progressive disorder, which is pathologically characterized by increased osteoclastic activity and abnormal bone formation. It may be localized to one or several skeletal segments. The disease occurs worldwide, but is more common in europe, australia, and new zealand. D from the institute of genetics and molec ular medicine, university of edinburgh. Paget disease of bone is a chronic progressive disease of the bone of uncertain etiology, characterized initially by an increase in bone resorption, followed by a disorganized and excessive formation of bone, leading to pain, deformities and fractures. Pagets disease of bone interferes with your bodys normal recycling process, in which new bone tissue gradually replaces old bone tissue. The classic form of paget disease of bone typically appears in middle age or later.
Pagets disease should be screened with a serum alkaline phosphatase test every two to three years. Pagets disease of bone metabolism clinical and experimental. Pagets disease of bone osteitis deformans hosain bouallouch shen zhou university 10 maart 2006 slideshare uses cookies to improve functionality and performance, and to provide you with relevant advertising. Pagets disease of bone most commonly occurs in the pelvis, skull, spine and legs. Pdf pagets disease of bone pdb is a condition of unknown etiology characterized by excessive and abnormal bone remodeling.
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