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Revista Brasileira de Reumatologia 2017To evaluate the parameters associated with quality of life in patients with Paget's disease of bone.
OBJECTIVE
To evaluate the parameters associated with quality of life in patients with Paget's disease of bone.
METHODS
Patients with Paget's disease of bone were evaluated with SF-36 and WHOQOL-bref questionnaires. Patients with other diseases that could cause significant impairment of their quality of life were excluded. We searched for correlations between the results and: age, time from diagnosis, type of involvement, pain related to Paget's disease of bone, limitation to daily activities, deformities, bone specific alkaline phosphatase, the extent of involvement and treatment.
RESULTS
Fifty patients were included. Results of the SF-36 total score and its domains, physical and mental health, were significantly correlated with bone pain and deformities. Marital status was significantly correlated with the SF-36 total score and Mental Health Domain. BAP levels and disease extension were significantly correlated to SF-36 Physical Health Domain. After multivariate analysis, the only parameters that remained significantly associated with the SF-36 total score and to its Mental Health and Physical Health Domains were pain and marital status. The WHOQOL-bref total score was significantly associated with pain, physical impairment and deformities. WHOQOL-bref Domain 1 (physical) score was significantly associated with marital status, pain and deformities, while Domain 2 (psychological) score was associated with marital status, physical impairment and kind of involvement. After multivariate analysis, the presence of pain, deformities, and marital status were significantly associated with results of the WHOQOL-bref total score and its Domain 1. WHOQOL-bref domain 2 results were significantly predicted by pain and marital status.
CONCLUSION
The main disease-related factor associated with SF-36 results in Paget's disease of bone patients was bone pain, while bone pain and deformities were associated with WHOQOL-bref.
Topics: Aged; Female; Health Status; Humans; Male; Middle Aged; Osteitis Deformans; Osteoarthritis; Pain; Quality of Life; Surveys and Questionnaires
PubMed: 28967630
DOI: 10.1016/j.rbre.2017.06.002 -
Drug Design, Development and Therapy 2011Paget's disease of bone (PDB) is a progressive monostotic or polyostotic metabolic bone disease characterized by focal abnormal bone remodeling, with increased bone... (Review)
Review
Paget's disease of bone (PDB) is a progressive monostotic or polyostotic metabolic bone disease characterized by focal abnormal bone remodeling, with increased bone resorption and excessive, disorganized, new bone formation. PDB rarely occurs before middle age, and it is the second most frequent metabolic bone disorder after osteoporosis, affecting up to 3% of adults over 55 years of age. One of the most striking and intriguing clinical features is the focal nature of the disorder, in that once the disease is established within a bone, there is only local spread within that bone and no systemic dissemination. Despite many years of intense research, the etiology of PDB has still to be conclusively determined. Based on a detailed review of genetic and viral factors incriminated in PDB, we propose a unifying hypothesis from which we can suggest emerging strategies and therapies. PDB results in weakened bone strength and abnormal bone architecture, leading to pain, deformity or, depending on the bone involved, fracture in the affected bone. The diagnostic assessment includes serum total alkaline phosphatase, total body bone scintigraphy, skull and enlarged view pelvis x-rays, and if needed, additional x-rays. The ideal therapeutic option would eliminate bone pain, normalize serum total alkaline phosphatase with prolonged remission, heal radiographic osteolytic lesions, restore normal lamellar bone, and prevent recurrence and complications. With the development of increasingly potent bisphosphonates, culminating in the introduction of a single intravenous infusion of zoledronic acid 5 mg, these goals of treatment are close to being achieved, together with long-term remission in almost all patients. Based on the recent pathophysiological findings, emerging strategies and therapies are reviewed: ie, pulse treatment with zoledronic acid; denosumab, a fully human monoclonal antibody directed against RANK ligand; tocilizumab, an interleukin-6 receptor inhibitor; odanacatib, a cathepsin K inhibitor; and proteasome and Dickkopf-1 inhibitors.
Topics: Animals; Bone Resorption; Clinical Trials as Topic; Diphosphonates; Humans; Osteitis Deformans
PubMed: 21607019
DOI: 10.2147/DDDT.S11306 -
LaeknabladidConsidering the changes in moral principles, human behavior and behavioral values through the ages, in Egill Skallagrimsson's Saga, Egill presents us with altered mental...
Considering the changes in moral principles, human behavior and behavioral values through the ages, in Egill Skallagrimsson's Saga, Egill presents us with altered mental status. This is in terms of what at present is considered symptoms of an anti-social personality, and bipolar affective disorder. Egill Skallagrimsson is considered one of the most famous Vikings in the Icelandic Sagas. Archaeological findings mentioned in Egill's Saga indicate disfigurement of his skull, which has led many authors to suggest that Egill suffered from skeletal dysplasia. The primary assumption in the literature is that Egill Skallagrimsson was affected by Paget's disease of bone. This consideration is additionally based on the scholar's interpretation of the Saga text. The unique storytelling style in the Saga of Egill Skallagrimsson is evident; however, the question of the story's truthfulness remains open. In this article, we investigate Egill Skallagrimsson's assumed Paget's disease of bone, based on the physical and mental symptoms disclosed in the Saga of Egill Skallagrimsson. Associated with the assumption, the author's hermeneutics of Egill's Saga in the context of modern-day knowledge of Paget's disease of bone, brings forward the probability estimate to the range of permille. In Scandinavian folklore and mythology, a tale by Saxo Grammaticus of a notorious shield-maiden named Visna, reminds of Egill, as noted by Snorri Sturluson. Hence, in reference to Egill Skallagrimsson's mental status and physical appearance as listed in Egill's Saga, the authors recommend the name for his condition to be "Visna of Egill Skallagrimsson".
Topics: Antisocial Personality Disorder; Bipolar Disorder; History, Medieval; Humans; Iceland; Mental Health; Narration; Osteitis Deformans
PubMed: 31048556
DOI: 10.17992/lbl.2019.05.231 -
Journal of Bone and Mineral Research :... Dec 2006Biochemical measurements of bone turnover provide an objective assessment of disease activity and the response to treatment. Alkaline phosphatase is the best... (Review)
Review
Biochemical measurements of bone turnover provide an objective assessment of disease activity and the response to treatment. Alkaline phosphatase is the best characterized of the bone turnover markers and reflects the extent and activity of Paget's disease. However, in addition to bone-specific alkaline phosphatase (Bone ALP), there is also osteocalcin (OC) and procollagen type 1 N-terminal propeptide (P1NP) as formation markers. A variety of telopeptides (C-terminal telopeptide of type I collagen, [CTX], N-telopeptide of type I collagen [NTX]) or cross-link breakdown products of type 1 collagen can be used to assess bone resorption. Total alkaline phosphatase (Total ALP), Bone ALP, and P1NP all perform similarly in diagnosis and in evaluating the response to treatment, but the general availability, low interassay variation, and inexpensiveness of Total ALP makes it the best test for routine use. Measurement of the biological variability of the different markers in stable, untreated Paget's disease indicates how great a change (critical difference) is needed to define a true alteration in disease activity. Bone ALP, P1NP, and NTX show the highest therapy induced change/critical difference ratio during antiresorptive treatment. Some of the resorption markers show more complex changes in response to treatment. Pyridinoline (PYD) or deoxypyridinoline (DPD) cross-links of type 1 collagen are excreted in urine either as free or as peptide bound moieties, but it is the latter which decrease by the greatest amount in response to bisphosphonate therapy. Newly formed type 1 collagen contains an aspartyl-glycine motif (alphaCTX), which undergoes spontaneous isoaspartyl formation to betaCTX as the bone ages. In untreated Paget's disease, the alphaCTX is raised proportionately more (16-fold) than betaCTX (3-fold) and decreases in response to bisphosphonate therapy to a greater extent than betaCTX (measured in the sCTX assay). As bisphosphonates have become more potent, the aim of treatment has shifted toward the achievement of a rate of bone turnover in the lower part of the reference range. This is important because the duration of remission of disease activity is strongly determined by the post treatment nadir bone turnover.
Topics: Alkaline Phosphatase; Biomarkers; Bone Remodeling; Humans; Osteitis Deformans; Osteocalcin; Peptides
PubMed: 17229003
DOI: 10.1359/jbmr.06s204 -
Journal of Bone and Mineral Research :... Dec 2006Paget's disease is a chronic nonmetabolic bone disorder that is characterized by increased bone resorption, bone formation, and remodeling. This unbalanced process may... (Review)
Review
Paget's disease is a chronic nonmetabolic bone disorder that is characterized by increased bone resorption, bone formation, and remodeling. This unbalanced process may lead to osseous deformities, structural weakness, and altered joint biomechanics, all of which can make surgical reconstruction difficult. Although few patients with Paget's disease ever need surgical treatment, successful surgical management of orthopedic manifestations of the disease has improved the quality of life for these patients. Surgical options include corrective osteotomy for long bone deformity, fracture fixation, joint arthroplasty, spinal decompression, and tumor resection. Patients are at increased risk for surgical complications such as blood loss and heterotopic bone formation. Issues relating to the surgical management of patients with Paget's disease such as appropriate preoperative diagnosis, technical challenges of surgery, and strategies to improve the long-term outcome of surgical intervention are discussed.
Topics: Biopsy; Humans; Orthopedic Procedures; Osteitis Deformans; Radiography
PubMed: 17229013
DOI: 10.1359/jbmr.06s214 -
Journal of Bone and Mineral Research :... Aug 2020
Topics: Bone and Bones; Genetic Predisposition to Disease; Humans; Osteitis Deformans; Profilins
PubMed: 32589291
DOI: 10.1002/jbmr.4090 -
Journal of Bone and Mineral Research :... Dec 2006Secular trends in the severity and prevalence of Paget's disease over a 30-year period are described. Paget's disease has become less prevalent and patients are... (Review)
Review
UNLABELLED
Secular trends in the severity and prevalence of Paget's disease over a 30-year period are described. Paget's disease has become less prevalent and patients are presenting later, with less severe disease than previously. These data suggest that environmental factors are important in the etiology of Paget's disease.
INTRODUCTION
Data from several countries support the view that there are important secular trends in the prevalence and severity of Paget's disease. In this paper, recent trends in the epidemiology of Paget's disease are described.
MATERIALS AND METHODS
A database of all newly referred patients (n = 1487) with Paget's disease (1973-2002 inclusive, 30 years) was examined. Of these subjects, 56% had scintiscans. Plasma total alkaline phosphatase (total ALP) activity and disease extent on scintiscan were used as indices of severity. A radiographic prevalence survey of 1019 subjects of European origin >55 years of age in Dunedin was undertaken- approximately 20 years after an earlier survey had shown New Zealand to be a high prevalence area.
RESULTS
The number of new referrals with Paget's disease declined sharply from 1994 onward, to one half the rate seen 20 years earlier, whereas the mean age at presentation increased by 4 years per decade (p < 0.0001). Total ALP at diagnosis, disease extent on scintiscan, and the number of bones involved were all negatively correlated with both date of birth (p < 0.0001) and year of presentation (p < 0.0001), indicating that more recently born and presenting subjects had substantially less severe bone disease. The radiographic survey showed that the current prevalence was only approximately 50% of that in the 1983 survey (p = 0.012).
CONCLUSIONS
Although there are a number of potential biases, these data are consistent with a continued secular trend to presentation in older subjects with less extensive skeletal involvement and a declining prevalence of Paget's disease.
Topics: Alkaline Phosphatase; Humans; Osteitis Deformans; Prevalence
PubMed: 17229016
DOI: 10.1359/jbmr.06s202 -
Joint Bone Spine May 2019This study explored the role of outdoor and indoor air pollutants in Paget's disease of bone (PDB). (Comparative Study)
Comparative Study
OBJECTIVES
This study explored the role of outdoor and indoor air pollutants in Paget's disease of bone (PDB).
METHODS
We performed a survey in 140 French-Canadian patients with PDB, including 39 carriers of p.Pro392Leu mutation (SQSTM1 gene) and 113 healthy not mutated controls. The survey covered outdoor air pollution near the residence and indoor air pollutants by focusing on heating fuels and exposure to tobacco smoke. In a subgroup of patients, urinary concentrations of 17 heavy metals and 11 polycyclic aromatic hydrocarbons were measured by mass spectrometry. In light of what we learned from the survey and urinary assays, we explored the in vitro effects of certain toxics on osteoclasts in PDB. We conducted in vitro monocytes differentiation from peripheral blood of more than 40 participants, whose osteoclasts were treated with or without the toxic. The morphology of osteoclasts, their bone resorption abilities, gene and protein expression levels, and cellular oxidative stress levels were assayed.
RESULTS
An inhibitory effect of cigarette smoke condensate and heavy metals was observed on morphology and bone resorption activity of patients' osteoclasts. SQSTM1 gene expression was upregulated in osteoclasts from patients with PDB versus healthy controls in presence of cadmium, and SQSTM1 protein expression was upregulated in presence of bismuth and tobacco smoke condensates, in particular in osteoclasts from carriers of the SQSTM1 mutation. Furthermore, high levels of oxidative stress in patients' osteoclasts were observed.
CONCLUSIONS
Our in vitro experiments suggest an interaction between SQSTM1 gene and exposure to cadmium and tobacco smoke condensates.
Topics: Adaptor Proteins, Signal Transducing; Adult; Age Factors; Air Pollutants; Cadmium; Cross-Sectional Studies; Environmental Exposure; Female; Gene Expression Regulation; Gene-Environment Interaction; Genetic Predisposition to Disease; Humans; Incidence; Logistic Models; Male; Middle Aged; Multivariate Analysis; Mutation; Osteitis Deformans; Osteoclasts; Quebec; RANK Ligand; Reference Values; Risk Assessment; Sequestosome-1 Protein; Sex Factors; Tobacco Smoke Pollution
PubMed: 30594595
DOI: 10.1016/j.jbspin.2018.12.007 -
American Family Physician Aug 2020Paget disease of bone is a benign disorder characterized by focal areas of increased bone turnover in one or more skeletal sites. It usually affects older adults, and...
Paget disease of bone is a benign disorder characterized by focal areas of increased bone turnover in one or more skeletal sites. It usually affects older adults, and men are at a higher risk than women. Any bone may be affected, but the disease has a high preference for the pelvis, spine, skull, and long bones. Pain is the most common symptom, and presentation of the disease may depend on which bones are affected, the extent of involvement, and the presence of complications. Paget disease of bone may be asymptomatic, and suspicion arises from incidental findings of elevated serum alkaline phosphatase levels on routine blood work or abnormalities on imaging tests performed for an unrelated cause. Evidence-based guidelines recommend the use of plain radiography and serum alkaline phosphatase testing for initial diagnosis and radionuclide scans for delineation of the extent of disease. Treatment with nitrogen-containing bisphosphonates is recommended in active disease or when risk of complications is possible. Complications of the disease include arthritis, gait changes, hearing loss, nerve compression syndromes, and osteosarcoma. Total serum alkaline phosphatase is the suggested marker for assessing treatment response when high bone turnover occurs, and it should be measured at three to six months to evaluate initial response. Early diagnosis of Paget disease of bone remains key to its management because patients generally have a good prognosis if treatment is initiated before major complications arise. The primary care physician may need to consult with a specialist for confirmation of diagnosis and initiation of treatment.
Topics: Alkaline Phosphatase; Analgesics; Arthritis; Bone Density Conservation Agents; Bone Neoplasms; Collagen Type I; Diphosphonates; Fractures, Compression; Gait; Hearing Loss; Humans; Nerve Compression Syndromes; Osteitis Deformans; Osteosarcoma; Pain; Pain Management; Peptides; Practice Guidelines as Topic; Primary Health Care; Radiography; Radionuclide Imaging
PubMed: 32803929
DOI: No ID Found -
Seminars in Musculoskeletal Radiology Aug 2023Although the prevalence of Paget's disease has decreased over the past 20 years, incidental discovery on imaging is not unusual. The challenge is to establish the... (Review)
Review
Although the prevalence of Paget's disease has decreased over the past 20 years, incidental discovery on imaging is not unusual. The challenge is to establish the diagnosis, especially in unusual forms that may be mistaken for metastases. This review describes the typical imaging features of Paget's disease and some rare presentations that may be more difficult to recognize.
Topics: Humans; Osteitis Deformans; Incidental Findings
PubMed: 37748472
DOI: 10.1055/s-0043-1771036