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Paget's disease of bone

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https://www.readbyqxmd.com/read/27866237/long-term-effects-of-intravenous-ibandronate-in-paget-s-disease-of-bone
#1
Ian R Reid, Diana Wattie, Gregory D Gamble, Ramanamma Kalluru, Tim Cundy
We have previously demonstrated that intravenous ibandronate produces high initial response rates in Paget's disease, but the durability of this effect is unknown. It might be expected to be short lived because ibandronate has a low affinity for bone. Here we report long-term follow-up (up to 14 years) of patients from that trial. Twenty-five patients with active Paget's disease [baseline serum total alkaline phosphatase (ALP) ~3 times the upper limit of normal] received either 6 or 12 mg intravenous ibandronate at baseline...
November 19, 2016: Calcified Tissue International
https://www.readbyqxmd.com/read/27856382/prognostic-determinants-and-treatment-outcomes-analysis-of-osteosarcoma-and-ewing-sarcoma-of-the-spine
#2
Armin Arshi, Justin Sharim, Don Y Park, Howard Y Park, Hamed Yazdanshenas, Nicholas M Bernthal, Arya N Shamie
BACKGROUND CONTEXT: Osteosarcoma (OGS) and Ewing sarcoma (EWS) are the two classic primary malignant bone tumors. Due to the rarity of these tumors, evidence on demographics, survival determinants, and treatment outcomes for primary disease of the spine are limited and derived from small case series. PURPOSE: To use population-level data to determine the epidemiology and prognostic indicators in patients with OGS and EWS of the osseous spine. STUDY DESIGN/SETTING: Large-scale retrospective study...
November 14, 2016: Spine Journal: Official Journal of the North American Spine Society
https://www.readbyqxmd.com/read/27853907/editorial-paget-s-disease-of-bone
#3
EDITORIAL
Peter Pietschmann
No abstract text is available yet for this article.
November 16, 2016: Wiener Medizinische Wochenschrift
https://www.readbyqxmd.com/read/27843872/an-unusual-presentation-of-metastatic-bone-disease-in-a-subject-with-paget-s-disease-of-bone
#4
Shrinath Shetty, Sahana Shetty, Annie Jennifer Prabhu, Nitin Kapoor, Julie Hepzibah, Thomas Vizhalil Paul
Solid organ malignancies involving breast, prostate, and lung frequently metastasize to the skeleton. However, the occurrence of Paget's disease and metastatic bone disease in the same patient is uncommon. We report a case of a 63-year-old man who presented with back pain and a lump in the right breast. He was earlier diagnosed to have Paget's disease of bone based on characteristic skeletal radiological features,(99m)Tc methylene diphosphonate bone scan and elevated alkaline phosphatase, and treated with bisphosphonates, and his disease was in remission...
April 2016: Journal of Family Medicine and Primary Care
https://www.readbyqxmd.com/read/27823978/clinical-characteristics-and-prognoses-of-six-patients-with-multicentric-giant-cell-tumor-of-the-bone
#5
Chenglei Liu, Yawen Tang, Mei Li, Qiong Jiao, Huizhen Zhang, Qingcheng Yang, Weiwu Yao
Multicentric giant cell tumor of the bone (MGCT) is a rare entity whose radiographic, pathological and biological features remain confusing. We retrospectively reviewed six patients (1 male, 5 female; average age, 22.33 years) treated for confirmed MGCT between 2001 and 2015. The patients' clinical information, images from radiographs (n = 14), CT (n = 13), MRI (n = 8), bone scintigraphy (n = 1) and PET-CT (n = 2), as well as histologic features, treatment and prognosis were analyzed. A total of 17 lesions were detected: 4 around the knee joint, 3 in the greater trochanter and head of the femur, 5 in the small bones of the feet, and 2 in flat bones...
November 3, 2016: Oncotarget
https://www.readbyqxmd.com/read/27815949/total-hip-arthroplasty-in-paget-s-disease-a-review
#6
Vineet Tyagi, Claudette Lajam, Ajit J Deshmukh
Paget's disease of the bone is a chronic osteopathy that leads to structural weakness, hypervascularity, and bone deformities. Rapid bone turnover in patients with Paget's disease may affect outcomes following total hip arthroplasty (THA). Most literature on THA in the setting of Paget's disease is limited to isolated case reports or case series documenting a single institution experience. By completing a comprehensive analysis of the available cases, this study aims to investigate the outcomes and complications of THA in patients with Paget's disease...
November 2016: Bulletin of the Hospital for Joint Diseases
https://www.readbyqxmd.com/read/27815347/the-first-scube3-mutant-mouse-line-with-pleiotropic-phenotypic-alterations
#7
Helmut Fuchs, Sibylle Sabrautzki, Gerhard K H Przemeck, Stefanie Leuchtenberger, Bettina Lorenz-Depiereux, Lore Becker, Birgit Rathkolb, Marion Horsch, Lillian Garrett, Manuela A Östereicher, Wolfgang Hans, Koichiro Abe, Nobuho Sagawa, Jan Rozman, Ingrid L Vargas-Panesso, Michael Sandholzer, Thomas S Lisse, Thure Adler, Juan Antonio Aguilar-Pimentel, Julia Calzada-Wack, Nicole Ehrhard, Ralf Elvert, Christine Gau, Sabine M Hölter, Katja Micklich, Kristin Moreth, Cornelia Prehn, Oliver Puk, Ildiko Racz, Claudia Stoeger, Alexandra Vernaleken, Dian Michel, Susanne Diener, Thomas Wieland, Jerzy Adamski, Raffi Bekeredjian, Dirk H Busch, John Favor, Jochen Graw, Martin Klingenspor, Christoph Lengger, Holger Maier, Frauke Neff, Markus Ollert, Tobias Stoeger, Ali Önder Yildirim, Tim M Strom, Andreas Zimmer, Eckhard Wolf, Wolfgang Wurst, Thomas Klopstock, Johannes Beckers, Valerie Gailus-Durner, Martin Hrabě de Angelis
The vertebrate Scube (Signal peptide, CUB and EGF-like domain-containing protein) family consists of three independent members Scube1-3, which encode secreted cell surface-associated membrane glycoproteins. Limited information about the general function of this gene family is available, and their roles during adulthood. Here, we present the first Scube3 mutant mouse line (Scube3(N294K/N294K)) that clearly shows phenotypic alterations by carrying a missense mutation in exon 8, and thus contributes to understand SCUBE3 functions...
November 4, 2016: G3: Genes—Genomes—Genetics
https://www.readbyqxmd.com/read/27809640/loss-of-functional-osteoprotegerin-more-than-a-skeletal-problem
#8
Corinna Grasemannn, Nicole Unger, Matthias Hövel, Diana Arweiler-Harbeck, Ralf Herrmann, Michael M Schündeln, Oliver Müller, Bernd Schweiger, Ekkehart Lausch, Thomas Meissner, Cordula Kiewert, Berthold P Hauffa, Nick J Shaw
INTRODUCTION: Juvenile Pagets disease (JPD), an ultra-rare, debilitating bone disease stemming from unopposed RANKL action due to loss of functional osteoprotegerin (OPG) is caused by recessive mutations in TNFRSF11B. A genotype-phenotype correlation spanning from mild to very severe forms is described. AIM: To describe the complexity of the human phenotype of OPG deficiency in more detail and to investigate heterozygous mutation carriers for clinical signs of JPD...
November 3, 2016: Journal of Clinical Endocrinology and Metabolism
https://www.readbyqxmd.com/read/27808435/durability-of-response-to-zoledronate-treatment-and-competing-mortality-in-paget-s-disease-of-bone
#9
Tim Cundy, Katherine Maslowski, Andrew Grey, Ian R Reid
There has been a marked secular trend in recent decades toward patients with Paget's disease presenting at a greater age and having less extensive skeletal involvement. Over a similar time frame more potent bisphosphonates with a long duration of effect have been developed, raising the prospect of many patients needing only once in a lifetime treatment. We studied a cohort of 107 patients who had been treated with intravenous zoledronate for the first time at a mean age of 76 years. Sequential measurements of the bone turnover marker procollagen-1 NT-peptide (P1NP) were made for up to 10 years...
November 3, 2016: Journal of Bone and Mineral Research: the Official Journal of the American Society for Bone and Mineral Research
https://www.readbyqxmd.com/read/27761746/imaging-of-paget-s-disease-of-bone
#10
Naomi Winn, Radhesh Lalam, Victor Cassar-Pullicino
Paget's disease of bone is a disorder of bone remodelling, leading to changes in the architecture and overall appearance of the bone. The disorder may be monostotic or polyostotic and affect any bone in the body, although most commonly it involves the spine, pelvis, skull and femur. This article explores the different imaging modalities used in the assessment of Paget's disease of bone in its different phases. The relative merits of each imaging modality is discussed with illustrative examples, in particular with respect to radiographs, nuclear medicine bone scan, computed tomography (CT) and magnetic resonance imaging (MRI)...
October 19, 2016: Wiener Medizinische Wochenschrift
https://www.readbyqxmd.com/read/27760964/-a-case-of-early-anal-canal-cancer-with-pagetoid-spread-with-different-antitumor-effects-of-chemotherapy-on-different-metastatic-sites
#11
Kazuhiko Yoshimatsu, Gakuji Osawa, Hajime Yokomizo, Yuki Yano, Sachiyo Okayama, Akiko Sakuma, Masaya Satake, Yasufumi Yamada, Shinichi Asaka, Takebumi Usui, Kentaro Yamaguchi, Shunichi Shiozawa, Takeshi Shimakawa, Takao Katsube, Yoshihiko Naritaka
A 78-year-old man visited our hospital with a prolapsed hemorrhoid. He was referred to the dermatology unit due to the thickness and redness of the perianal skin. He was diagnosed as having extra mammary Paget's disease by skin biopsy. After a biopsy of the anal polyp was performed to investigate the primary site, he was diagnosed with early anal canal cancer with Pagetoid spread and underwent a radical operation. Abdominoperineal resection with skin(D2 prx D3 lymphadenectomy) was performed with perineal reconstruction using a gracilis muscle graft...
October 2016: Gan to Kagaku Ryoho. Cancer & Chemotherapy
https://www.readbyqxmd.com/read/27749427/multitracer-molecular-imaging-of-paget-disease-targeting-bone-remodeling-fatty-acid-metabolism-and-psma-expression-on-pet-ct
#12
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
https://www.readbyqxmd.com/read/27749129/paget-s-disease-of-the-bone
#13
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
https://www.readbyqxmd.com/read/27745697/denosumab-induced-severe-hypocalcemia-in-a-patient-with-paget-s-disease-of-bone-and-impaired-renal-function
#14
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
https://www.readbyqxmd.com/read/27741544/paget-disease-of-bone
#15
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
https://www.readbyqxmd.com/read/27704192/a-rare-case-of-rectal-carcinoma-and-prostate-carcinoma-with-coexistent-paget-s-disease-mimicking-bone-metastases-in-both-18-f-fdg-and-68-ga-psma-pet-ct
#16
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
https://www.readbyqxmd.com/read/27660736/renal-cell-carcinoma-metastasized-to-pagetic-bone
#17
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
https://www.readbyqxmd.com/read/27650015/-paget-s-disease-of-bone-diagnosis-and-treatment
#18
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
https://www.readbyqxmd.com/read/27600564/epidemiology-and-pathology-of-paget-s-disease-of-bone-a%C3%A2-review
#19
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
https://www.readbyqxmd.com/read/27600563/diagnosis-and-treatment-of-paget-s-disease-of-bone-a%C3%A2-clinical-practice-guideline
#20
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
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