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Wiener Medizinische Wochenschrift (1946) 2004Paget's disease of the bone is a disorder in which there is marked increase in bone turnover by activated osteoclasts in localized parts of the skeleton. The newly... (Review)
Review
Paget's disease of the bone is a disorder in which there is marked increase in bone turnover by activated osteoclasts in localized parts of the skeleton. The newly formed bone is abnormal in structure and mineralization resulting in deformity and increased risk of fracture. The prevalence of the disease has decreased over the last 20 years to 2% in patients over 55 years of age. The etiology of the disease is still unknown. There is strong evidence for viral infection, but recently a genetic disposition has also been discussed. There are important advances in the understanding of the pathophysiology of Paget's disease. increased sensitivity of osteoclast-precursors to 1,25(OH)2Vitamin D3 and RANKL, mediated by IL6 have been described as a possible mechanism. For the diagnosis of the disease the finding of an elevated level of plasma alkaline phosphatase as a marker of increased bone turnover is of great importance. This parameter is also used for monitoring disease activity during therapy. Plain radiology and skeletal scintigraphy are necessary for the diagnosis. CT and MRT can be useful in specific cases. In the last decade bisphosphonates have increasingly assumed--according to their mechanism of action--the prime role in the management of Paget's disease. Recently, more potent bisphosphonates have been developed and therefore the goal of therapy has changed. Initial response to treatment in terms of reducing activity of alkaline phosphatase to normal levels is associated with increased remission duration. In this respect, risedronate and alendronate have proved superior to etidronate. Bisphosphonates have also been used extensively in the form of intravenous infusion in patients with Paget's disease. Especially when gastro-intestinal side-effects occur with oral medication, this might be useful. However, at present more studies are needed to find the optimal drug, the optimal dose and intervals of i.v. therapy.
Topics: Bone Density; Bone and Bones; Diagnosis, Differential; Diagnostic Imaging; Diphosphonates; Fractures, Spontaneous; Humans; Osteitis Deformans
PubMed: 15106891
DOI: 10.1007/s10354-004-0051-6 -
The American Journal of Medicine Nov 2018Paget's disease of bone is a common bone disorder characterized by disorganized bone remodeling. The most likely etiology is a slow paramyxoviral infection in... (Review)
Review
Paget's disease of bone is a common bone disorder characterized by disorganized bone remodeling. The most likely etiology is a slow paramyxoviral infection in genetically susceptible individuals; however, the exact cause is unknown. Enhanced bone resorption due to an increased activity of osteoclasts recruits numerous osteoblasts to resorption sites, with large quantities of new bone matrix produced as a result. The accelerated bone resorption and formation are not as closely coupled as in a healthy bone; a disorganized bone tissue is formed. Many patients are asymptomatic; rising serum alkaline phosphatase or incidental finding of characteristic radiographic lesions are often the only diagnostic clues. Common clinical manifestations include bone pain, bowing of long bones, enlarged skull, and hearing loss. An elevated serum alkaline phosphatase level correlates with the disease activity. The diagnosis is confirmed by characteristic radiographic findings and by nuclear scintigraphy of the bone (the most sensitive test). Bisphosphonates, such as zoledronic acid, pamidronate, alendronate, and risedronate, are the mainstay of treatment. Patients who do not tolerate bisphosphonates can be treated with calcitonin.
Topics: Diphosphonates; Humans; Osteitis Deformans
PubMed: 29752905
DOI: 10.1016/j.amjmed.2018.04.028 -
Best Practice & Research. Clinical... Oct 2018Paget's disease of bone is a focal disorder of bone remodelling that progresses slowly and leads to changes in the shape and size of affected bones and to skeletal,... (Review)
Review
Paget's disease of bone is a focal disorder of bone remodelling that progresses slowly and leads to changes in the shape and size of affected bones and to skeletal, articular and vascular complications. In some parts of the world it is the second most common bone disorder after osteoporosis though in recent years its prevalence and severity appear to decrease. The disease is easily diagnosed and effectively treated but its pathogenesis remains incompletely understood.
Topics: Bone and Bones; Humans; Osteitis Deformans; Osteoporosis; Prevalence
PubMed: 30449547
DOI: 10.1016/j.beem.2018.05.005 -
Radiologic Clinics of North America Jul 2022Paget's disease is a metabolic bone disorder affecting the elderly and characterized by bone resorption followed by compensatory bone formation. Radiography is the... (Review)
Review
Paget's disease is a metabolic bone disorder affecting the elderly and characterized by bone resorption followed by compensatory bone formation. Radiography is the imaging modality of choice for the diagnosis whereas bone scintigraphy helps stage the extent of the disease and assess response to treatment. MRI and CT are important imaging methods in the assessment of complications and surgical planning. Osteolytic lesions of Paget's first phase present with well-defined margins on radiographs, most commonly in the femur, pelvis, and skull. Cortical thickening, trabecular coarsening, bone marrow sclerosis, and deformities of long bones are present in the mixed- and late-sclerotic phases.
Topics: Aged; Bone Marrow; Bone and Bones; Humans; Magnetic Resonance Imaging; Osteitis Deformans; Radiography
PubMed: 35672089
DOI: 10.1016/j.rcl.2022.02.005 -
Journal of the American Geriatrics... Aug 1998Paget's disease of bone is important in geriatric populations because it is the second most common bone disorder after osteoporosis. In older people, it may be... (Review)
Review
Paget's disease of bone is important in geriatric populations because it is the second most common bone disorder after osteoporosis. In older people, it may be responsible for chronic back pain and joint pain, skeletal deformities, hearing loss, and cranial nerve compression. Paget's disease can reduce both function and mobility in the older people. In addition to newer tests for assessing the activity of Paget's disease, effective therapy is available in the form of salmon calcitonin for nasal administration and new third generation bisphosphonates. Frequently, treatment can reverse the course of the disease. For these reasons, it is feasible for the physician to adopt an aggressive approach to diagnosis and treatment. The objective should be to relieve pain, improve mobility, and forestall debilitating complications. This review will focus on the manifestations and clinical management of Paget's disease. Two cases are presented that illustrate common management problems in older patients.
Topics: Aged; Humans; Osteitis Deformans
PubMed: 9706897
DOI: 10.1111/j.1532-5415.1998.tb02763.x -
Veterinary Pathology 1972
Topics: Animals; Haplorhini; Macaca; Monkey Diseases; Osteitis Deformans
PubMed: 4210691
DOI: 10.1177/030098587200900409 -
Journal of Endodontics Nov 2003A review of the signs, symptoms, and treatment of Paget's disease is presented. Paget, or osteitis deformans, is a chronic, progressive, bone disease of viral etiology.... (Review)
Review
A review of the signs, symptoms, and treatment of Paget's disease is presented. Paget, or osteitis deformans, is a chronic, progressive, bone disease of viral etiology. Recent investigations about the causative agent of this disorder have implicated the measles virus of the paramyxovirus family. Additional studies have demonstrated that abnormal virus-infected osteoclasts generate interleukin (IL)-6, a resorptive cytokine from the bone marrow of patients with Paget's bone disease.
Topics: Humans; Jaw Diseases; Osteitis Deformans; Tooth Diseases
PubMed: 14651277
DOI: 10.1097/00004770-200311000-00009 -
Metabolism: Clinical and Experimental Mar 2018Juvenile Paget disease (JPD) is a rare disorder, mainly caused by mutations in the gene TNFRSF11B that encodes osteoprotegerin (OPG). Loss of OPG action causes... (Review)
Review
Juvenile Paget disease (JPD) is a rare disorder, mainly caused by mutations in the gene TNFRSF11B that encodes osteoprotegerin (OPG). Loss of OPG action causes generalized, extremely rapid bone turnover. The clinical manifestations are both skeletal - progressive skeletal deformity that develops in childhood - and extra-skeletal, including hearing loss, retinopathy, vascular calcification and internal carotid artery aneurysm formation. The severity of the phenotype seems to be related to the severity of TNFRSF11B gene deactivation. JPD is characterized biochemically by very high alkaline phosphatase activity, as well as other bone turnover markers. Bisphosphonates are commonly used to reduce the greatly accelerated bone turnover and can ameliorate the skeletal phenotype, if started early enough in childhood and continued at least until growth is complete. Limited evidence from patients treated with recombinant OPG or denosumab also provided favorable results. Recombinant OPG would represent a replacement treatment, but it is unavailable for clinical use. It seems that life-long treatment with anti-resorptives is required, since the disease is reactivated after treatment discontinuation. An international collaborating network for the continuous registration and follow-up of JPD patients could be helpful in the future.
Topics: Adolescent; Age of Onset; Child; Humans; Osteitis Deformans; Osteoprotegerin
PubMed: 29080812
DOI: 10.1016/j.metabol.2017.10.007 -
Handbook of Clinical Neurology 2014Paget's disease is an osteoclastic-mediated disorder of bone that results in abnormal bone resorption associated with inadequate remodeling that leads to mechanically... (Review)
Review
Paget's disease is an osteoclastic-mediated disorder of bone that results in abnormal bone resorption associated with inadequate remodeling that leads to mechanically weakened bone. Demonstrating variable geographic prevalence, it is becoming less frequent and age of onset is lengthening in areas of once high prevalence prior to the institution of effective medical therapies, suggesting its etiology involves both environmental as well as genetic factors. Insights into its pathophysiology are helping to clarify other inherited osteolytic disorders of bone by providing additional insights into related cellular processes.
Topics: History, 19th Century; History, 20th Century; History, 21st Century; Humans; Osteitis Deformans
PubMed: 24365317
DOI: 10.1016/B978-0-7020-4086-3.00034-5 -
American Journal of Physiologic Imaging 1990New knowledge of osteoclastic function and new treatments to reduce osteolytic activity have combined to yield new degrees of biochemical control over the increased rate... (Review)
Review
New knowledge of osteoclastic function and new treatments to reduce osteolytic activity have combined to yield new degrees of biochemical control over the increased rate of bone resorption and formation that characterized osteitis deformans (OD; Paget's disease). We have briefly outlined current concepts, and provided an example of the role radionuclide imaging can play in delineating the skeletal areas involved in this disease.
Topics: Bone and Bones; Humans; Osteitis Deformans; Radionuclide Imaging
PubMed: 2196916
DOI: No ID Found