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Paget disease

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https://www.readbyqxmd.com/read/27872543/extra-mammary-paget-s-disease-of-vulva-a-case-report
#1
Than Singh Tomar, Suchetha Sambasivan, Rema Prabhakaran Nair, Shaji Thomas, Preethi T Ramadas
Extra mammary Paget's disease (EMPD) is a rare condition involving the vulva, anogenital region, and axilla. Vulvar disease usually presents as a slow growing well-defined itchy plaque with crustations or ulcerations over the affected area in postmenopausal women. Well-established guidelines for diagnosis and management are not available for this rare condition. Our patient is a 64-year-old postmenopausal woman with a history of similar complaints of 2 years duration, not responding to multiple topical treatments...
December 2016: Indian Journal of Surgical Oncology
https://www.readbyqxmd.com/read/27866237/long-term-effects-of-intravenous-ibandronate-in-paget-s-disease-of-bone
#2
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
#3
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
#4
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
#5
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/27832673/extramammary-paget-disease-of-the-vulva-case-report
#6
Bianca Ruschel Hillmann, Amanda Amaro Pereira, Luiz Fernando Sommacal
Extramammary Paget disease (EPD) is a rare malign neoplasm that may affect the vulva and has manifestations common to benign diseases such as itching, pain and eczema. This leads to delay in diagnosis and consequent worse prognosis. The definitive diagnosis is obtained by biopsy of the vulva, which shows Paget cells. The treatment of choice is wide excision with margins, which leads to sequelae, functional and aesthetic. Recurrence is common. This article reports the case of a 48-year-old female patient with history of vulvar itching for the past 2 years, who had been submitted to various treatments for benign pathologies...
October 2016: Revista Brasileira de Ginecologia e Obstetrícia
https://www.readbyqxmd.com/read/27823978/clinical-characteristics-and-prognoses-of-six-patients-with-multicentric-giant-cell-tumor-of-the-bone
#7
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
#8
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
#9
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
#10
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
#11
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/27772880/primary-invasive-extramammary-paget-s-disease-on-penoscrotum-a-clinicopathological-analysis-of-41-cases
#12
Yunyi Kong, Xuxia Shen, Jiaojie Lv, Bo Shu
No abstract text is available yet for this article.
February 2016: Pathology
https://www.readbyqxmd.com/read/27768726/ibmpfd-disease-causing-mutant-vcp-p97-proteins-are-targets-of-autophagic-lysosomal-degradation
#13
Oznur Bayraktar, Ozlem Oral, Nur Mehpare Kocaturk, Yunus Akkoc, Karin Eberhart, Ali Kosar, Devrim Gozuacik
The ubiquitin-proteasome system (UPS) degrades soluble proteins and small aggregates, whereas macroautophagy (autophagy herein) eliminates larger protein aggregates, tangles and even whole organelles in a lysosome-dependent manner. VCP/p97 was implicated in both pathways. VCP/p97 mutations cause a rare multisystem disease called IBMPFD (Inclusion Body Myopathy with Paget's Disease and Frontotemporal Dementia). Here, we studied the role IBMPFD-related mutants of VCP/p97 in autophagy. In contrast with the wild-type VCP/p97 protein or R155C or R191Q mutants, the P137L mutant was aggregate-prone...
2016: PloS One
https://www.readbyqxmd.com/read/27763794/evaluation-of-the-vulvar-cancer-histology-code-reported-by-central-cancer-registries-importance-in-epidemiology
#14
David A Siegel, Reda Wilson, Edward J Wilkinson, Julia W Gargano, Meg Watson, Brenda Y Hernandez, Marc T Goodman, Charles F Lynch, Elizabeth R Unger, Mona Saraiya
CONTEXT: -Knowing the subtype of vulvar cancer histology is important for estimating human papillomavirus-related cancer etiology. Surveillance of human papillomavirus-related vulvar cancers informs public health decisions related to vaccination against human papillomavirus. OBJECTIVE: -To assess the accuracy of registry classifications of vulvar cancer and determine the histologic classification of cases reported as not otherwise specified. DESIGN: -Pathology specimens were collected from Florida, Iowa, and Hawaii cancer registries...
October 20, 2016: Archives of Pathology & Laboratory Medicine
https://www.readbyqxmd.com/read/27761746/imaging-of-paget-s-disease-of-bone
#15
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/27761174/spinal-metastases-of-extramammary-paget-disease-with-radiologic-pathologic-correlation
#16
Jason T Little, Vance T Lehman, Jonathan M Morris, Julia S Lehman, Felix E Diehn
Extramammary Paget disease (EMPD) is an uncommon malignancy. It manifests either in the primary form in the skin as an intraepithelial neoplasm, or in secondary form as pagetoid (intraepithelial) spread of an underlying internal carcinoma to the skin. Although local invasion and recurrence of primary extramammary Paget disease are relatively frequent, widespread metastases are rare. As such, there are very few reports and little characterization of the radiologic features of widespread spinal metastases. To our knowledge, there are no prior reports of a metastatic extramammary Paget disease presenting as a painful pathologic vertebral body compression fracture...
May 2016: Journal of Radiology Case Reports
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
#17
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
#18
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
#19
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/27746660/glandular-paget-s-disease-of-the-male-nipple
#20
Jong Hyuk Moon, Hye Soo Ko, Ji Won Byun, Gwang Seong Choi, Jeonghyun Shin
No abstract text is available yet for this article.
October 2016: Annals of Dermatology
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