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Paget's disease of bone

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https://www.readbyqxmd.com/read/28430378/treating-paget-s-disease-why-and-how-much
#1
Tim Cundy
Paget's disease (PDB) is a benign chronic focal disorder of older people. Affected bones have greatly accelerated bone turnover, a chaotic structure and are highly vascular. People with PDB are commonly asymtomatic but can also suffer chronic bone pain. Affected bones may become enlarged and deformed; and they are also liable to fracture. This article is protected by copyright. All rights reserved.
April 21, 2017: Journal of Bone and Mineral Research: the Official Journal of the American Society for Bone and Mineral Research
https://www.readbyqxmd.com/read/28406527/modified-weekly-regimen-of-cisplatin-epirubicin-and-paclitaxel-induced-a-durable-response-in-two-cases-of-metastatic-extramammary-paget-s-disease
#2
Ikuko Hirai, Takeru Funakoshi
Metastatic extramammary Paget's disease (EMPD) is a rare cancer with no standardized treatment. We report two cases of metastatic EMPD treated with a modified weekly PET (cisplatin, epirubicin and paclitaxel) regimen given biweekly (i.e. 2 weeks on/2 weeks off) that had durable responses. Case 1 was a 74-year-old man with EMPD metastatic to lymph nodes, lung, and bone who presented with a hemorrhagic tumor on the scrotum. We tried the PET regimen weekly, but adjusted the interval to biweekly after two doses because of hematological side-effects...
April 13, 2017: Journal of Dermatology
https://www.readbyqxmd.com/read/28396816/psma-pet-ct-positive-paget-disease-in-a-patient-with-newly-diagnosed-prostate-cancer-imaging-and-bone-biopsy-findings
#3
Michael Froehner, Marieta Toma, Klaus Zöphel, Vladimir Novotny, Michael Laniado, Manfred P Wirth
A 67-year-old man diagnosed with Gleason score 4 + 5 = 9 clinically localized prostate cancer with (68)Ga-labeled prostate-specific membrane antigen-targeted ligand positron emission tomography/computed tomography (PSMA-PET/CT) positive Paget bone disease is described. Immunohistochemical staining revealed weak PSMA positivity of the bone lesion supporting the hypothesis that neovasculature might explain positive PSMA-PET/CT findings in Paget disease.
2017: Case Reports in Urology
https://www.readbyqxmd.com/read/28389692/familial-early-onset-paget-s-disease-of-bone-associated-with-a-novel-hnrnpa2b1-mutation
#4
Xuan Qi, Qianqian Pang, Jiawei Wang, Zhen Zhao, Ou Wang, Lijun Xu, Jiangfeng Mao, Yan Jiang, Mei Li, Xiaoping Xing, Wei Yu, Asan, Weibo Xia
Paget disease of bone (PDB) is a common metabolic bone disease characterized by increased bone resorption and disorganized bone formation which affect single or multiple sites of bones. Although the exact cause of PDB is still controversial, genetic factors are considered to play an important role in PDB. Several genes involved in the differentiation or function of osteoclast were shown to be associated with PDB or related syndrome such as SQSTM1, TNFRSF11A, TNFRSF11B, and ZNF687. Multisystem proteinopathy (MSP), a newly proposed syndrome including inclusion body myopathy (IBM), PDB, frontotemporal dementia (FTD), and amyotrophic lateral sclerosis (ALS), is mainly caused by mutation in VCP gene...
April 7, 2017: Calcified Tissue International
https://www.readbyqxmd.com/read/28361207/antibody-response-to-paramyxoviruses-in-paget-s-disease-of-bone
#5
Micaela Rios Visconti, Ricardo Usategui-Martín, Stuart H Ralston
Paget's disease of bone (PDB) is a common skeletal disorder characterised by focal abnormalities of increased and disorganised bone turnover. Genetic factors play a central role in the pathogenesis of PDB but environmental factors also contribute. Measles virus (MV), respiratory syncytial virus (RSV) and canine distemper virus (CDV) have all been implicated as potential disease triggers but the data are conflicting. Since chronic paramyxovirus infection with measles is known to be accompanied by increased production of antiviral antibodies, we have analysed circulating concentrations of antibodies to MV, CDV, and RSV as well as mumps, rubella and varicella zoster virus (VZV) in 463 patients with PDB and 220 aged and gender-matched controls...
March 31, 2017: Calcified Tissue International
https://www.readbyqxmd.com/read/28358955/bilateral-ocular-pain-and-hyperemia-in-a-patient-with-paget-disease-of-the-bone
#6
Ali S Haider, Andrew M Burbidge, Anthony Dunlop
No abstract text is available yet for this article.
March 30, 2017: JAMA Ophthalmology
https://www.readbyqxmd.com/read/28339664/adult-paget-s-disease-of-bone-a-review
#7
Stephen Paul Tuck, Robert Layfield, Julie Walker, Babitha Mekkayil, Roger Francis
Adult PD of bone is the second commonest metabolic bone condition after osteoporosis. The condition is characterized by increased bone cell activity, with bone-resorbing osteoclasts often larger and containing more nuclei than normal, and osteoblasts producing increased amounts of disorganized bone. This leads to expanded bone of poor quality possessing both sclerotic and lytic areas. PD of bone has a strong genetic element, with a family history being noted in 10-20% of cases. A number of genetic defects have been found to be associated with the condition...
February 17, 2017: Rheumatology
https://www.readbyqxmd.com/read/28339363/proangiogenic-gene-polymorphisms-are-associated-with-susceptibility-to-paget-s-disease-of-bone-and-with-its-clinical-features
#8
Ismael Calero-Paniagua, Ricardo Usategui-Martín, Luís Corral-Gudino, Judit García-Aparicio, Javier Del Pino-Montes, Rogelio González-Sarmiento
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March 23, 2017: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/28337806/the-pharmacological-profile-of-a-novel-highly-potent-bisphosphonate-ox14-1-fluoro-2-imidazo-1-2-%C3%AE-pyridin-3-yl-ethyl-bisphosphonate
#9
M A Lawson, F H Ebetino, A Mazur, A D Chantry, J Paton-Hough, H R Evans, D Lath, M K Tsoumpra, M W Lundy, R L M Dobson, M Quijano, A A Kwaasi, J E Dunford, X Duan, J T Triffitt, G Jeans, R G G Russell
Bisphosphonates are widely used in the treatment of clinical disorders characterized by increased bone resorption, including osteoporosis, Paget's disease and the skeletal complications of malignancy. The anti-resorptive potency of the nitrogen-containing bisphosphonates on bone in vivo is now recognised to depend upon two key properties, namely mineral binding affinity and inhibitory activity on farnesyl pyrophosphate synthase (FPPS), and these properties vary independently of each other in individual bisphosphonates...
March 24, 2017: Journal of Bone and Mineral Research: the Official Journal of the American Society for Bone and Mineral Research
https://www.readbyqxmd.com/read/28324123/long-term-bone-scintigraphy-results-after-intravenous-zoledronate-in-paget-s-disease-of-bone
#10
Ian R Reid, Katherine Maslowski
Zoledronate produces long-term normalization of biochemical markers in Paget's disease but whether this implies the absence of active disease is unknown. We have determined whether bone scintigraphy, a more sensitive index of disease activity, is also normalized ≥5 years after treatment with zoledronate. A consecutive case series of 11 individuals with Paget's disease treated with zoledronate 5 mg ≥5 years previously is reported. Eight patients received a single zoledronate infusion and were assessed 55-120 months later...
March 21, 2017: Calcified Tissue International
https://www.readbyqxmd.com/read/28303975/a-conserved-inter-domain-communication-mechanism-regulates-the-atpase-activity-of-the-aaa-protein-drg1
#11
Michael Prattes, Mathias Loibl, Gertrude Zisser, Daniel Luschnig, Lisa Kappel, Ingrid Rössler, Manuela Grassegger, Altijana Hromic, Elmar Krieger, Karl Gruber, Brigitte Pertschy, Helmut Bergler
AAA-ATPases fulfil essential roles in different cellular pathways and often act in form of hexameric complexes. Interaction with pathway-specific substrate and adaptor proteins recruits them to their targets and modulates their catalytic activity. This substrate dependent regulation of ATP hydrolysis in the AAA-domains is mediated by a non-catalytic N-terminal domain. The exact mechanisms that transmit the signal from the N-domain and coordinate the individual AAA-domains in the hexameric complex are still the topic of intensive research...
March 17, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28255281/clinical-and-genetic-advances-in-paget-s-disease-of-bone-a-review
#12
REVIEW
N Alonso, I Calero-Paniagua, J Del Pino-Montes
Paget's disease of bone (PDB) is the second most common metabolic bone disorder, after osteoporosis. It is characterised by focal areas of increased and disorganised bone turnover, coupled with increased bone formation. This disease usually appears in the late stages of life, being slightly more frequent in men than in women. It has been reported worldwide, but primarily affects individuals of British descent. Majority of PDB patients are asymptomatic, but clinical manifestations include pain, bone deformity and complications, like pathological fractures and deafness...
2017: Clinical Reviews in Bone and Mineral Metabolism
https://www.readbyqxmd.com/read/28183835/benign-spine-lesions-advances-in-techniques-for-minimally-invasive-percutaneous-treatment
#13
REVIEW
A Tomasian, A N Wallace, J W Jennings
Minimally invasive percutaneous imaging-guided techniques have been shown to be safe and effective for the treatment of benign tumors of the spine. Techniques available include a variety of tumor ablation technologies, including radiofrequency ablation, cryoablation, microwave ablation, alcohol ablation, and laser photocoagulation. Vertebral augmentation may be performed after ablation as part of the same procedure for fracture stabilization or prevention. Typically, the treatment goal in benign spine lesions is definitive cure...
February 9, 2017: AJNR. American Journal of Neuroradiology
https://www.readbyqxmd.com/read/28176386/long-term-randomized-trial-of-intensive-versus-symptomatic-management-in-paget-s-disease-of-bone-the-prism-ez-study
#14
Adrian Tan, Kirsteen Goodman, Allan Walker, Jemma Hudson, Graeme S MacLennan, Peter L Selby, William D Fraser, Stuart H Ralston
It has been suggested that normalization of bone turnover may improve clinical outcome in Paget's disease of bone (PDB) by preventing complications such as fractures and the development of osteoarthritis. Here we investigated the long-term effects of a treatment strategy that aimed to normalize bone turnover in PDB with that of symptomatic treatment. The study group comprised 502 subjects who were enrolled into a 3-year extension of the Paget's Disease: Randomized Trial of Intensive versus Symptomatic Management (PRISM) study...
February 8, 2017: Journal of Bone and Mineral Research: the Official Journal of the American Society for Bone and Mineral Research
https://www.readbyqxmd.com/read/28164066/giant-cell-tumor-developing-in-paget-s-disease-of-bone-a-case-report-with-review-of-literature
#15
Vivek Verma, Ajay Puri, Sanket Shah, Bharat Rekhi, Ashish Gulia
INTRODUCTION: Paget's disease of bone (PDB) is a disease of elderly characterized by disorganized bone remodeling. Development of secondary neoplasm in PDB is a known but rare phenomenon. Development of giant cell tumor in PDB (GCT-PDB) is extremely rare, and little is known about its etiopathogenesis and management. We present a case report of such a development with a review of the literature and the role of various new modalities of treatment available in the management of this rare condition...
September 2016: Journal of Orthopaedic Case Reports
https://www.readbyqxmd.com/read/28161640/gastrodin-inhibits-osteoclastogenesis-via-down-regulating-the-nfatc1-signaling-pathway-and-stimulates-osseointegration-in%C3%A2-vitro
#16
Feng Zhou, Yi Shen, Bo Liu, Xia Chen, Lu Wan, Dan Peng
Bone is a rigid yet dynamic organ, and this dynamism is mediated by the delicate balance between osteoclastic bone resorption and osteoblastic bone formation. However, excessive activation of osteoclasts is responsible for many bone diseases such as osteoporosis, Paget disease, and tumor bone metastasis. Agents that could inhibit osteoclast formation or function are regarded as promising alternatives to treat osteoclast-related diseases. Recently, traditional Chinese medicine has attracted attention because of its multiple activities in bone metabolism...
February 2, 2017: Biochemical and Biophysical Research Communications
https://www.readbyqxmd.com/read/28132074/the-incidental-bone-lesion-on-computed-tomography-management-tips-for-abdominal-radiologists
#17
REVIEW
Michelle Nguyen, Christopher Beaulieu, Stefanie Weinstein, Lewis K Shin
Incidental bone lesions are commonly seen on abdominal and pelvic computed tomography (CT) examinations. These incidental bone lesions can be diagnostically challenging to the abdominal radiologist who may not be familiar with their appearance or their appropriate management. The characterization of such bone lesions as non-aggressive or aggressive based on their CT appearance involves similar principles to their morphologic evaluation on radiographs. Knowledge of the age of the patient and the presence of symptoms, mainly bone pain, can improve analysis...
January 28, 2017: Abdominal Radiology
https://www.readbyqxmd.com/read/28110348/erratum-to-a-rare-case-of-rectal-carcinoma-and-prostate-carcinoma-with-coexistent-paget-s-disease-mimicking-bone-metastases-in-both-18f-fdg-and-68ga-psma-pet-ct
#18
Arun Sasikumar, Ajith Joy, M R A Pillai, Vikraman Raman, Arun Vasudevan, Jayaprakash Madhavan
No abstract text is available yet for this article.
April 2017: European Journal of Nuclear Medicine and Molecular Imaging
https://www.readbyqxmd.com/read/28105116/computer-assisted-quantitative-evaluation-of-bisphosphonate-treatment-for-paget-s-disease-of-bone-using-the-bone-scan-index
#19
Satoshi Nagano, Shunsuke Nakamura, Hirofumi Shimada, Masahiro Yokouchi, Takao Setoguchi, Yasuhiro Ishidou, Hiromi Sasaki, Setsuro Komiya
The purpose of the present study was to analyze the effect of treatment of Paget's disease of bone (PDB) with bone scintigraphy using a computer-assisted diagnosis system (BONENAVI) that quantitatively evaluates bone metabolism. Seven patients with PDB (three male, four female; average age, 60 years; age range, 33-80 years) underwent bone scintigraphy and measurement of serum alkaline phosphatase (ALP), bone-specific ALP (BAP), serum cross-linked N-telopeptide (NTx) of type I collagen, urinary NTx, and deoxypyridinoline (DPD) before and after bisphosphonate treatment...
December 2016: Experimental and Therapeutic Medicine
https://www.readbyqxmd.com/read/28089128/management-of-paget-s-disease-of-the-calcaneum
#20
Joel Humphrey, Anum Pervez, Roland Walker, Ali Abbasian, Sam Singh
The calcaneum is not the most common site for Paget's disease of bone, with only a few reports of monostotic involvement. We present 2 cases of Paget's disease of bone affecting the calcaneus, present an overview of the published data, and describe our management of these interesting cases.
March 2017: Journal of Foot and Ankle Surgery: Official Publication of the American College of Foot and Ankle Surgeons
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