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Paget's disease of bone

Thorsten Derlin, Desiree Weiberg, Jan M Sohns
Paget disease is a chronic disorder resulting in enlarged and misshapen bones, and is caused by disorganized bone remodeling. We present the case of an 85-year-old man with prostatic adenocarcinoma and known Paget disease of the right iliac bone who underwent Ga-prostate-specific membrane antigen ligand, C-acetate, and F-fluoride PET/CT for restaging of cancer. On all PET scans, increased tracer accumulation was observed in Paget disease of bone. Besides that Paget disease may mimic metastases on PET/CT using various radiotracers, including Ga-prostate-specific membrane antigen ligands and C-acetate, this case highlights the potential of multiparametric disease characterization on PET...
October 5, 2016: Clinical Nuclear Medicine
Terrel Sanders, Thanh D Hoang, James C Clifford, Alfred F Shwayhat
No abstract text is available yet for this article.
October 17, 2016: Endocrine Practice
Marie Kostine, Nadia Mehsen-Cetre, Bernard Bannwarth
Paget's disease of bone is characterised by focal areas of increased bone turnover affecting one or several bones throughout the skeleton. Where indicated, pharmacological management is primarily based on the use of anti-resorptive drugs. In this respect, bisphosphonates are the drugs of choice. However, they are contraindicated in patients with advanced renal dysfunction. Here, we report the efficacy and safety of one denosumab injection (60mg) in a patient with Paget's disease of bone and impaired renal function, who benefited from this treatment but developed a severe hypocalcemia...
September 15, 2016: Thérapie
Radhesh Krishna Lalam, Victor N Cassar-Pullicino, Naomi Winn
Paget disease (PD) is a common disease of bone associated with abnormal bone turnover that in turn is due to an imbalance between osteoclastic and osteoblastic activity. There is good evidence that PD is reducing in incidence, prevalence, and severity. The disease is most often asymptomatic and is usually detected incidentally on imaging examinations performed for other reasons. The features of PD are relatively specific on radiographs and computed tomography. However, the appearances on magnetic resonance imaging are subtle and nonspecific, although it has become the initial imaging choice for several clinical indications including back pain, neurologic dysfunction, and knee pain...
July 2016: Seminars in Musculoskeletal Radiology
Arun Sasikumar, Ajith Joy, M R A Pillai, Vikraman Raman, Arun Vasudevan, Jayaprakash Madhavan
No abstract text is available yet for this article.
October 4, 2016: European Journal of Nuclear Medicine and Molecular Imaging
Ashley Ramirez, Bo Liu, Baiywo Rop, Michelle Edison, Michael Valente, Jeremy Burt
Paget's disease of the bone, historically known as osteitis deformans, is an uncommon disease typically affecting individuals of European descent. Patients with Paget's disease of the bone are at increased risk for primary bone neoplasms, particularly osteosarcoma. Many cases of metastatic disease to pagetic bone have been reported. However, renal cell carcinoma metastasized to pagetic bone is extremely rare. A 94-year-old male presented to the emergency department complaining of abdominal pain. A computed tomography scan of the abdomen demonstrated a large mass in the right kidney compatible with renal cell carcinoma...
2016: Curēus
E M Winter, N A T Hamdy, R T de Jongh, E M W Eekhoff, M C Zillikens, N M Appelman-Dijkstra
Paget's disease of bone is a focal disorder of bone remodelling that leads to changes in the shape and size of affected bones, and is associated with articular and vascular complications. The disorder is characterised by a localised increase in osteoclast number and activity in one or more affected sites while the rest of the skeleton remains unaffected. The excessive bone resorption leads to recruitment of osteoblasts to the remodelling sites, resulting in increased bone formation. This accelerated bone turnover causes deposition of bone with disorganised architecture and structural weakness...
2016: Nederlands Tijdschrift Voor Geneeskunde
Elena Nebot Valenzuela, Peter Pietschmann
Paget's disease of bone (PDB) is a noninflammatory, metabolic, skeletal disorder characterized by localized excessive osteoclastic bone resorption that is followed by compensatory increased osteoblastic activity leading to unstructured, fibroblastic, and biomechanically unstable bone. As a result, there is deformity and enlargement of the bone with a defective and disorganized pattern. Here, we review the epidemiology, etiology, pathology, macrostructure, histology, and quantitative histomorphometry findings of PDB...
September 6, 2016: Wiener Medizinische Wochenschrift
Christian Muschitz, Xaver Feichtinger, Judith Haschka, Roland Kocijan
Paget's disease of bone (osteitis deformans) is a benign focal disorder of accelerated skeletal remodeling. Either a single bone (monostotic) or multiple bones (polyostotic) can be affected. In patients with suspected Paget's disease plain radiographs of the suspicious regions of the skeleton are recommended. The initial biochemical evaluation of a patient should be done using serum total ALP (alkaline phosphatase) or with the use of a more specific marker of bone formation: PINP (intact N-terminal type 1 procollagen propeptide) or CTX (cross-linked C‑telopeptide)...
September 6, 2016: Wiener Medizinische Wochenschrift
Narendra Prakash Rai, Jayaprasad Anekar, Shabil Mohamed Mustafa, Darshan Devang Divakar
Paget's disease is a metabolic disorder of bone caused due to defect in the remodelling process and is very common in western countries but is very rare in Asians and Africans. It was first described by a British scientist Sir James Paget in 1877. It can be monostotic or polyostotic depending on the number of bones involved. It most commonly affects older people of more than 50 years. Disease involvement can be symptomatic or asymptomatic depending on the extent of the disease process. Diagnosis of Paget's disease can be made by raised serum alkaline phosphatase levels, radiological examination and by radioisotope bone scans...
2016: BMJ Case Reports
Arun Sasikumar, Ajith Joy, Raviteja Nanabala, M R A Pillai, Hari T A
65-year-old man with left-sided pelvic pain on evaluation was found to have features suggestive of either Paget disease or prostatic bone metastasis of the left hemipelvis based on Tc-MDP bone scan and MRI. Ga-PSMA PET/CT to assess the possibility of primary prostate cancer and if present to stage it helped to rule out prostate cancer because of absence of focal abnormal increased tracer uptake in the prostate gland. However, false-positive tracer uptake was noted in the left hemipelvis, which was subject to biopsy and histopathologically proven to be Paget disease involvement...
October 2016: Clinical Nuclear Medicine
Sophie Bourgeois, Pieterjan Gykiere, Lode Goethals, Hendrik Everaert, Frank W De Geeter
Ga-PSMA plays an increasing role in prostate cancer management, but several instances of false positivity have now been recognized. We present a patient with metastatic prostatic carcinoma who also showed overexpression of PSMA in Paget disease of the humerus on Ga-PSMA PET. This probably relates to bone remodeling and increased vascularity. It is important to be aware of this aspecific uptake because its recognition may avoid overstaging and may alter the therapeutic choice.
November 2016: Clinical Nuclear Medicine
Tobias B Haack, Erika Ignatius, Javier Calvo-Garrido, Arcangela Iuso, Pirjo Isohanni, Camilla Maffezzini, Tuula Lönnqvist, Anu Suomalainen, Matteo Gorza, Laura S Kremer, Elisabeth Graf, Monika Hartig, Riccardo Berutti, Martin Paucar, Per Svenningsson, Henrik Stranneheim, Göran Brandberg, Anna Wedell, Manju A Kurian, Susan A Hayflick, Paola Venco, Valeria Tiranti, Tim M Strom, Martin Dichgans, Rita Horvath, Elke Holinski-Feder, Christoph Freyer, Thomas Meitinger, Holger Prokisch, Jan Senderek, Anna Wredenberg, Christopher J Carroll, Thomas Klopstock
SQSTM1 (sequestosome 1; also known as p62) encodes a multidomain scaffolding protein involved in various key cellular processes, including the removal of damaged mitochondria by its function as a selective autophagy receptor. Heterozygous variants in SQSTM1 have been associated with Paget disease of the bone and might contribute to neurodegeneration in amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD). Using exome sequencing, we identified three different biallelic loss-of-function variants in SQSTM1 in nine affected individuals from four families with a childhood- or adolescence-onset neurodegenerative disorder characterized by gait abnormalities, ataxia, dysarthria, dystonia, vertical gaze palsy, and cognitive decline...
September 1, 2016: American Journal of Human Genetics
Agessandro Abrahao, Osório Abath Neto, Fernando Kok, Edmar Zanoteli, Bibiana Santos, Wladimir Bocca Vieira de Rezende Pinto, Orlando Graziani Povoas Barsottini, Acary Souza Bulle Oliveira, José Luiz Pedroso
BACKGROUND: VCP (valosin-containing protein gene) variants have been associated with peripheral and central neurodegenerative processes, including inclusion body myopathy (IBM), Paget disease of bone (PDB), frontotemporal dementia (FTD), and familial amyotrophic lateral sclerosis (ALS) type 14. The combination of IBM, PDB (IBMPFD1) can presented in one individual. However, the association of IBMPFD1 and ALS in the same family is rare. METHODS: We reported three individuals from a Brazilian kindred with intrafamilial phenotype variability...
September 15, 2016: Journal of the Neurological Sciences
Pompiliu HoraŢiu Petrescu, Dragoş Andrei Izvernariu, Cătălina Iancu, Gabriel Ovidiu Dinu, Marcel Mihai Berceanu-Văduva, Dan Crişan, Mihaela Iacob, Venera Margareta Bucur, Ion Călin RăuŢia, Ion Radu Prejbeanu, Sorin Dema, Ciprian Constantin DuŢă
Paget's disease of bone is a benign disease characterized by exaggerated remodeling of the bone matrix after osteoclast-mediated bone destruction. Its etiology is still unknown, despite the fact that it was discovered and described in 1877, but genetic factors and environmental triggers were shown to play their part in the pathogenesis of the disease. The main clinical presentations of the disease are related to bone pain and deformities. Radiological diagnosis is the main detection tool, though many monostotic Paget's disease cases may remain undiagnosed...
2016: Romanian Journal of Morphology and Embryology, Revue Roumaine de Morphologie et Embryologie
Adrian Goldman, Gustav Boije af Gennis, Henri Xhaard, Seppo Meri, Jari Yli-Kauhaluoma
In all organisms from bacteria to humans, specific hydrolases--pyrophosphatases--hydrolyse inorganic pyrophosphate to phosphate. Without this, DNA, RNA and protein synthesis stops. Pyrophosphatases are thus essential for all life. In humans, disorders in pyrophosphate metabolism cause chondrocalcinosis and hypophosphatasia. Currently, pyrophosphate analogues, e.g. alendronate, are in clinical use in osteoporosis and Paget's disease but also for e.g. complications of prostate cancer. In bacteria and protozoan parasites, membrane-bound pyrophosphatases (mPPases), which do not occur in humans, convert pyrophosphate to a proton or sodium gradient...
2016: Duodecim; Lääketieteellinen Aikakauskirja
Karolina Slowicka, Lars Vereecke, Geert van Loo
Optineurin (OPTN) was initially identified as a regulator of NF-κB and interferon signaling, but attracted most attention because of its association with various human disorders such as glaucoma, Paget disease of bone, and amyotrophic lateral sclerosis. Importantly, OPTN has recently been identified as an autophagy receptor important for the autophagic removal of pathogens, damaged mitochondria, and protein aggregates. This activity is most likely compromised in patients carrying OPTN mutations, and contributes to the observed phenotypes...
September 2016: Trends in Immunology
K Narayanan, N Ramakrishnan, D Majumdar
No abstract text is available yet for this article.
January 2003: Medical Journal, Armed Forces India
John Kenneth Blazak, Paul Thomas
We present a case of an 81-year-old man with multifocal Paget disease found on bone scan that was performed for incidentally diagnosed prostate cancer. The subsequent Ga-PSMA (HBED-CC) PET scan also displayed increased uptake in the same distribution. Multiple known tumors display increased Ga-PSMA uptake due to neovasculature. We postulate that increased Ga-PSMA uptake within the pagetoid bone relates to neovascularity known to occur in Paget disease. Such pagetic uptake could result in false-positive studies for bone metastases, particularly in the setting of less typical Paget disease...
September 2016: Clinical Nuclear Medicine
Zeynep Burçin Gönen, Canay Yılmaz Asan
BACKGROUND: Bisphosphonates are commonly prescribed antiresorptive agents for the management of patients with osteoporosis, Paget's disease, multiple myeloma, and metastatic tumors. Platelet-rich fibrin (PRF) is a second generation platelet concentrate, and has the ability of regulating the inflammation and stimulation of chemotactic agents. The aim of this report is to present the treatment of Stage-3 bisphosphonate-related osteonecrosis of the jaw (BRONJ) by PRF. CLINICAL PRESENTATION: A 77-year-old male patient with Stage-3 BRONJ was treated with minimal surgical operations and PRF membrane...
June 30, 2016: Cranio: the Journal of Craniomandibular Practice
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