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Paget's disease of bone

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https://www.readbyqxmd.com/read/28721304/imaging-features-of-paget-s-disease-on-11-c-choline-pet-ct
#1
Cameron E Leitch, Ajit H Goenka, Benjamin M Howe, Stephen M Broski
The purpose of this study was to investigate the appearance of Paget's disease (PD) on (11)C choline PET/CT and correlate these findings to serum alkaline phosphatase (ALP) level and skeletal scintigraphy. With IRB approval, our institutional (11)C choline PET/CT database (9/2005-6/2015) was searched for patients with PD. Site of osseous involvement, CT appearance, and multiple semi-quantitative measures were measured and correlated with ALP and degree of uptake on bone scan. Our search identified 10 males (mean age 79...
2017: American Journal of Nuclear Medicine and Molecular Imaging
https://www.readbyqxmd.com/read/28690091/-paget-s-disease-of-bone-diagnostic-and-therapeutic-updates
#2
R Alaya, Z Alaya, M Nang, E Bouajina
Paget's disease of bone is the second most common metabolic bone disease after osteoporosis. Its pathogenesis is not yet clearly understood. Geographic distribution and epidemiological variations suggest a role of genetic and environmental factors in its pathophysiology. The frequency of the Paget's disease of bone increases with age. Its discovery can be fortuitous. Prognosis mainly depends on the occurrence of complications involving bones and joints, neurological, cardiovascular or metabolic systems. Treatment of symptomatic forms currently relies on bisphosphonates that have transformed its prognosis...
July 6, 2017: La Revue de Médecine Interne
https://www.readbyqxmd.com/read/28687305/incidental-finding-of-a-monostotic-form-of-paget-disease-of-the-scapula-in-a-lung-cancer-patient
#3
A Sá Pinto, V M Alves, A Oliveira, R H Castro, J G Pereira
Paget Disease of the bone is a metabolic disorder which leads to a profound alteration of the bone's architecture. It may be asymptomatic and, since its radiological features mimic those of bone metastases, may present a diagnostic dilemma during the evaluation of a cancer patient. We illustrate an uncommon monostotic form of the disease, affecting solely the scapula, diagnosed during the staging of a lung cancer patient.
August 2017: Radiography
https://www.readbyqxmd.com/read/28666271/the-distinct-clinical-features-of-giant-cell-tumor-of-bone-in-pagetic-and-non-pagetic-patients-are-associated-with-genetic-biochemical-and-histological-differences
#4
Giuseppina Divisato, Federica Scotto di Carlo, Laura Pazzaglia, Riccardo Rizzo, Domenico A Coviello, Maria Serena Benassi, Piero Picci, Teresa Esposito, Fernando Gianfrancesco
Giant Cell Tumor of Bone (GCT) is a tumor characterized by neoplastic mesenchymal stromal cells and a high number of osteoclast-like multinucleated giant cells. Rarely, GCT could arise in bones affected by Paget's disease of bone (GCT/PDB). Although it is already known that GCT/PDB and GCT show a different clinical profile regarding the age-onset and skeletal localization, our deep clinical comparison between the two GCT/PDB and GCT cohorts, permitted us to identify additional differences (e.g. focality, ALP serum levels, the 5-year survival rate and the familial recurrence), strongly suggesting a different molecular basis...
June 27, 2017: Oncotarget
https://www.readbyqxmd.com/read/28642853/paget-s-disease-skeletal-manifestations-and-effect-of-bisphosphonates
#5
Ho Kang, Young-Chang Park, Kyu Hyun Yang
BACKGROUND: Paget's disease of bone leads to change in the shape and size of the bone and results in reduced bone strength, leading to the complications of deformity, arthritis, and fracture. Due to unknown reasons, Paget's disease is rare in Asian descendants. We report the cases of Paget's disease who visited our institute for 15 years and reviewed the literatures. METHODS: We retrospectively reviewed the medical records and radiograms of 8 patients (6 female and 2 male) who were diagnosed as Paget's disease of bone...
May 2017: Journal of Bone Metabolism
https://www.readbyqxmd.com/read/28639953/impressive-paget-disease-of-the-lumbar-spine-masks-the-coexisting-multiple-myeloma
#6
Xiao Zhong, Yunxia Ye, Xiaohong Ou
Multimodality imaging, including MRI, bone scintigraphy, and FDG PET/CT, was performed to evaluate a 49-year-old man who had right hip and lower limb pain for 4 months because possible malignancy was suspected. All 3 modalities showed obvious Paget disease of the lumbar spine. However, less impressive increased activity was also noted on FDG PET/CT images. Subsequent laboratory tests and pathology examination leads to diagnosis of multiple myeloma. Our case further confirms the notion that it is insufficient to focus on the most obvious and unintentionally overlook the less impressive findings in interpreting nuclear medicine images...
June 20, 2017: Clinical Nuclear Medicine
https://www.readbyqxmd.com/read/28629592/measurement-and-clinical-utility-of-%C3%AE-ctx-in-serum-and-plasma
#7
Stephen A P Chubb, Samuel D Vasikaran
Biochemical markers of bone turnover (BTM) are released during bone remodeling and can be measured in blood or urine as noninvasive surrogate markers for the bone remodeling rate. The C-terminal cross-linked telopeptide of type I collagen (βCTX) is released during bone resorption and is specific to bone tissue. Assays have been developed to measure βCTX in blood and in urine; most current use of βCTX measurement for research and in clinical practice is performed on a blood sample. Method-specific differences for serum and plasma βCTX have led to initiatives to standardize or harmonize βCTX commercial assays...
2017: Advances in Clinical Chemistry
https://www.readbyqxmd.com/read/28589453/bone-seeking-agents-for-the-treatment-of-bone-disorders
#8
REVIEW
Jacqueline Cawthray, Ellen Wasan, Kishor Wasan
The targeting and delivery of therapeutic and diagnostic agents to bone tissue presents both a challenge and opportunity. Osteoporosis, Paget's disease, cancer, and bone metastases are all skeletal diseases whose treatment would benefit from new targeted therapeutic strategies. Osteoporosis, in particular, is a very prevalent disease, affecting over one in three women and one in five men in Canada alone with the cost to the healthcare system estimated at over $2.3 billion in 2010. Bone tissue is often considered a rigid structure when in reality there is a process of continuous remodeling that takes place via complex endocrine-regulated cell signaling pathways in addition to the signaling pathways unique to bone tissue...
August 2017: Drug Delivery and Translational Research
https://www.readbyqxmd.com/read/28583274/benign-bone-conditions-that-may-be-fdg-avid-and-mimic-malignancy
#9
REVIEW
Thomas C Kwee, John M H de Klerk, Maarten Nix, Ben G F Heggelman, Stefan V Dubois, Hugo J A Adams
Positron emission tomography with the radiotracer (18)F-fluoro-2-deoxy-d-glucose (FDG) plays an important role in the evaluation of bone pathology. However, FDG is not a cancer-specific agent, and knowledge of the differential diagnosis of benign FDG-avid bone alterations that may resemble malignancy is important for correct patient management, including the avoidance of unnecessary additional invasive tests such as bone biopsy. This review summarizes and illustrates the spectrum of benign bone conditions that may be FDG-avid and mimic malignancy, including osteomyelitis, bone lesions due to benign systemic diseases (Brown tumor, Erdheim-Chester disease, Gaucher disease, gout and other types of arthritis, Langerhans cell histiocytosis, and sarcoidosis), benign primary bone lesions (bone cysts, chondroblastoma, chondromyxoid fibroma, desmoplastic fibroma, enchondroma, giant cell tumor and granuloma, hemangioma, nonossifying fibroma, and osteoid osteoma and osteoblastoma), and a group of miscellaneous benign bone conditions (post bone marrow biopsy or harvest status, bone marrow hyperplasia, fibrous dysplasia, fractures, osteonecrosis, Paget disease of bone, particle disease, and Schmorl nodes)...
July 2017: Seminars in Nuclear Medicine
https://www.readbyqxmd.com/read/28566875/intermediate-type-of-juvenile-paget-s-disease-a-rare-case-in-indian-population
#10
S Ravi Raja Kumar, Bhavana S Bagalad, Ch Balakrishna Manohar, Puneeth H Kuberappa
Juvenile Paget's disease (JPD), a rare genetic skeletal disorder characterized by accelerated bone turnover with elevated levels of serum alkaline phosphatase, presents in early childhood. We report a female patient with typical features of JPD with dental finding who remained undiagnosed until 18 years of age. Scarcity of this disease in the Indian literature and need for timely diagnosis to avert progression of disease thus incited us to report this case.
January 2017: Contemporary Clinical Dentistry
https://www.readbyqxmd.com/read/28551891/-bisphosphonates-and-ocular-inflammation
#11
Daniel Rappoport, Hana Leiba
Bisphosphonates are a group of drugs used for treatment in several bone diseases such as osteoporosis in women, Paget's disease, hypercalcemia of malignancy, primary malignancies of the bone and metastatic bone disease (breast and prostate carcinoma). Numerous reports in the medical literature described ocular side effects in patients treated with these drugs. We report on two patients, treated with bisphosphonates due to malignancy, who presented with unilateral and bilateral uveitis. Treatment of the ocular inflammation should include local and systemic treatment and stopping the systemic use of bisphosphonates...
February 2017: Harefuah
https://www.readbyqxmd.com/read/28525684/223ra-dichloride-response-in-a-patient-with-paget-disease-and-bone-metastases-secondary-to-castration-resistant-prostate-cancer
#12
Lina García Cañamaque, Cristina Rioja Parada, Paloma García De la Peña
PURPOSE: Paget disease is commonly asymptomatic and discovered when an imaging test is performed for another clinical indication or when elevated serum alkaline phosphatase is found. Bone pain usually appears late in the disease process and is only present in a minority of patients. For diagnosis, X-ray and bone scan are the most recommended imaging methods; radionuclide imaging of the skeleton has become the standard, since it is the most sensitive test for detecting increased bone activity...
May 18, 2017: Tumori
https://www.readbyqxmd.com/read/28525460/coexistent-superscan-and-lincoln-sign-on-bone-scintigraphy
#13
Mukta Kulkarni, Atul Soni, Shubhangi Shetkar, Momin Amer, Amruta Mulavekar, Prathamesh Joshi
A 70-year-old man underwent Tc-methylene diphosphonate scintigraphy for staging of adenocarcinoma prostate. Scintigraphy revealed diffuse increased tracer uptake in skeletal system along with faint renal visualization, a pattern compatible with metastatic superscan. The scintigraphy also revealed increased radiotracer uptake in the body of the mandible-Lincoln sign or black beard sign. Radiological imaging revealed sclerotic lesions throughout the skeleton including the mandible, confirming widespread skeletal metastases...
August 2017: Clinical Nuclear Medicine
https://www.readbyqxmd.com/read/28524179/a-fkbp5-mutation-is-associated-with-paget-s-disease-of-bone-and-enhances-osteoclastogenesis
#14
Bingru Lu, Yulian Jiao, Yinchang Wang, Jing Dong, Muyun Wei, Bin Cui, Yafang Sun, Laicheng Wang, Bingchang Zhang, Zijiang Chen, Yueran Zhao
Paget's disease of bone (PDB) is a common metabolic bone disease that is characterized by aberrant focal bone remodeling, which is caused by excessive osteoclastic bone resorption followed by disorganized osteoblastic bone formation. Genetic factors are a critical determinant of PDB pathogenesis, and several susceptibility genes and loci have been reported, including SQSTM1, TNFSF11A, TNFRSF11B, VCP, OPTN, CSF1 and DCSTAMP. Herein, we report a case of Chinese familial PDB without mutations in known genes and identify a novel c...
May 19, 2017: Experimental & Molecular Medicine
https://www.readbyqxmd.com/read/28512218/ubiquitin-and-atp-dependent-unfoldase-activity-of-p97-vcp%C3%A2-nploc4%C3%A2-ufd1l-is-enhanced-by-a-mutation-that-causes-multisystem-proteinopathy
#15
Emily E Blythe, Kristine C Olson, Vincent Chau, Raymond J Deshaies
p97 is a "segregase" that plays a key role in numerous ubiquitin (Ub)-dependent pathways such as ER-associated degradation. It has been hypothesized that p97 extracts proteins from membranes or macromolecular complexes to enable their proteasomal degradation; however, the complex nature of p97 substrates has made it difficult to directly observe the fundamental basis for this activity. To address this issue, we developed a soluble p97 substrate-Ub-GFP modified with K48-linked ubiquitin chains-for in vitro p97 activity assays...
May 30, 2017: Proceedings of the National Academy of Sciences of the United States of America
https://www.readbyqxmd.com/read/28506889/nfam1-signaling-enhances-osteoclast-formation-and-bone-resorption-activity-in-paget-s-disease-of-bone
#16
Yuvaraj Sambandam, Kumaran Sundaram, Takamitsu Saigusa, Sundaravadivel Balasubramanian, Sakamuri V Reddy
Paget's disease of bone (PDB) is marked by the focal activity of abnormal osteoclasts (OCLs) with excess bone resorption. We previously detected measles virus nucleocapsid protein (MVNP) transcripts in OCLs from patients with PDB. Also, MVNP stimulates pagetic OCL formation in vitro and in vivo. However, the mechanism by which MVNP induces excess OCLs/bone resorption activity in PDB is unclear. Microarray analysis identified MVNP induction of NFAM1 (NFAT activating protein with ITAM motif 1) expression. Therefore, we hypothesize that MVNP induction of NFAM1 enhances OCL differentiation and bone resorption in PDB...
August 2017: Bone
https://www.readbyqxmd.com/read/28498272/paget-s-disease-of-the-temporal-bone-a-single-institution-contemporary-review-of-27-patients
#17
Nicholas L Deep, Jake G Besch-Stokes, John I Lane, Colin L W Driscoll, Matthew L Carlson
OBJECTIVES: To report a contemporary review from a single-institution series on Paget's disease of the temporal bone (PDTB). STUDY DESIGN: Retrospective chart review of patients evaluated from 1998 to 2016. SETTING: Quaternary referral center. PATIENTS: Patients with radiographically confirmed PDTB. MAIN OUTCOME MEASURES: Clinical, audiological, and radiological features and management strategies of PDTB...
July 2017: Otology & Neurotology
https://www.readbyqxmd.com/read/28481796/18f-sodium-fluoride-pet-ct-in-paget-disease
#18
Francesca Cucchi, Lene Simonsen, Annemette Geilager Abild-Nielsen, Rikke Broholm
Paget disease (PD) of bone is a benign but chronic disorder of bone metabolism. We report a case of an 85-year-old man with several fractures in the pelvis and radiograph raising suspicion of metastases. An F-NaF PET/CT demonstrated high F-NaF uptake in the same regions. The integrated assessment of imaging findings, supported by biochemistry, allowed diagnosing PD. The F-NaF PET/CT is a supplementary diagnostic instrument in the diagnosis of PD, and use of F-NaF PET/CT in this context, especially finalized to the differential diagnosis with metastatic lesions, requires the physician to be familiar with imaging patterns of this disease...
July 2017: Clinical Nuclear Medicine
https://www.readbyqxmd.com/read/28473965/total-hip-arthroplasty-in-patients-with-paget-s-disease-of-bone-a-systematic-review
#19
Sammy A Hanna, Sebastian Dawson-Bowling, Steven Millington, Rej Bhumbra, Pramod Achan
AIM: To investigate the clinical and functional outcomes following total hip arthroplasty (THA) in patients with Paget's disease. METHODS: We carried out a systematic review of the literature to determine the functional outcome, complications and revision rates of THA in patients with Paget's disease. Eight studies involving 358 hips were reviewed. The mean age was 70.4 years and follow-up was 8.3 years. There were 247 cemented THAs (69%), 105 uncemented THAs (29%) and 6 hybrid THAs (2%)...
April 18, 2017: World Journal of Orthopedics
https://www.readbyqxmd.com/read/28454368/anti-osteoclastic-agent-denosumab-for-a-giant-cell-tumor-of-the-bone-with-concurrent-paget-s-disease-a-case-report
#20
Takaaki Tanaka, John Slavin, Sue-Anne McLachlan, Peter Choong
Paget's disease of the bone may predispose the development of malignant bone tumors such as osteosarcoma. Giant cell tumor (GCT) as a consequence of Paget's disease is rare. Bone GCT is characterized by rapid growth, the destruction of bone, extension to the surrounding soft tissue and abnormal bone turnover caused by an abnormality of the receptor activator of nuclear factor-κB (RANK)-RANK ligand (RANKL) pathway. Denosumab is a RANK-RANKL inhibitor, which is used to treat osteoporosis and bone GCT. In the current study, a 60-year-old male presented with severe pain located between the right thigh and the knee...
April 2017: Oncology Letters
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