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Paget disease

Garrison Glavich, Jeffrey Gourley, Victor Fong
Paget-Schroetter syndrome, also known as effort thrombosis, is a relatively rare disease process characterized by primary thrombosis to the subclavian and axillary veins. It usually presents in younger individuals, commonly affecting the dominant side upper extremity, and the diagnosis relies on a combination of imaging, laboratory tests, and clinical presentation. Upper extremity deep vein thrombosis can also lead to pulmonary emboli, as in this case of a 20-year-old female discovered to have right sided Paget-Schroetter syndrome...
February 2018: Radiology Case Reports
Ruiguo Zhang, Guizhi Zhang, Renfei Wang, Qiang Jia, Jian Tan
No abstract text is available yet for this article.
March 19, 2018: Korean Journal of Internal Medicine
Paul Glendenning, S A Paul Chubb, Samuel Vasikaran
Bone turnover marker (BTM's) concentrations in blood and urine reflect bone-remodelling activity, and may be useful adjuncts in the diagnosis and management of metabolic bone diseases. Newer biomarkers, mainly bone regulatory proteins, are currently being investigated to elucidate their role in bone metabolism and disease and may in future be useful in clinical diagnosis and management of metabolic bone disease. BTM concentrations increase around menopause in women, and at a population level the degree of increase in BTM's reflect bone loss...
March 12, 2018: Clinica Chimica Acta; International Journal of Clinical Chemistry
Holly White, Simi George, James Gossage, Fuju Chang
No abstract text is available yet for this article.
March 15, 2018: Journal of Gastrointestinal Cancer
L Tagliaferri, C Casà, G Macchia, A Pesce, G Garganese, B Gui, G Perotti, S Gentileschi, F Inzani, R Autorino, S Cammelli, A G Morganti, V Valentini, M A Gambacorta
OBJECTIVE/PURPOSE: Extramammary Paget disease (EMPD) is a rare neoplasm of the skin generally affecting the anogenital area. Because of the low-frequency of the disease, no specific guidelines about the treatment strategy are available. Surgery is the recommended therapy for resectable and localized disease, but several other local treatments have been reported such as radiotherapy (RT). Most articles report small retrospective studies, referring to patients treated decades ago with large heterogeneity in terms of RT dose and technique...
March 17, 2018: International Journal of Gynecological Cancer
Kexu Chen, Hanlin Liang, Jiewen Peng, Yanfang Zheng
Advanced extramammary Paget's disease does not have a standardized treatment guideline as its incidence is low and has been rarely reported in literature. Here we describe a case of metastatic extramammary Paget's disease successfully treated with topical 5-fluorouracil (5-FU) and systemic pemetrexed. The therapy was safe without any appreciable adverse effects like diarrhea, rash, neutropenia or fatigue; maintaining remission for more than 6 months. Thus, we propose 5-FU and pemetrexed as the first-line therapy for advanced extramammary Paget's disease, especially for aged patients with unresectable skin lesions...
March 12, 2018: Indian Journal of Dermatology, Venereology and Leprology
Ali A Damavandy, Vitaly Terushkin, John A Zitelli, David G Brodland, Christopher J Miller, Jeremy R Etzkorn, Thuzar M Shin, Mark A Cappel, Mario Mitkov, Ali Hendi
BACKGROUND: Extramammary Paget's disease (EMPD) is a rare intraepithelial malignancy with high recurrence rates following standard surgical treatments, ranging from 22% to 60% in large retrospective reviews. OBJECTIVE: To evaluate the local recurrence rate of Mohs micrographic surgery (MMS) supplemented with intraoperative immunohistochemistry for cytokeratin-7 (MMS + CK-7) for primary and recurrent EMPD. MATERIALS AND METHODS: Retrospective, multi-center, cross-sectional study of patients treated using MMS + CK-7...
March 2018: Dermatologic Surgery: Official Publication for American Society for Dermatologic Surgery [et Al.]
Tyler M Bauman, Ilana S Rosman, David M Sheinbein
Extramammary Paget's disease (EMPD) is a rare intraepithelial neoplasm with an extremely variable clinical course. The objective of this study was to determine if combination imiquimod and photodynamic therapy could induce remission of EMPD. A 69-year-old man with EMPD was treated with topical imiquimod 5% cream at night for 5 days per week for 1 month, followed by 2 months of 5% imiquimod for three nights a week. For the following 6 months, monthly 5-aminolevulinic acid photodynamic therapy was added. After 6 months, imiquimod was discontinued and the patient continued to be treated with quarterly photodynamic therapy...
March 5, 2018: BMJ Case Reports
Keitaro Fukuda, Takeru Funakoshi
Extramammary Paget's disease (EMPD) is a rare, slow-growing, cutaneous adenocarcinoma that usually originates in the anogenital area and axillae outside the mammary glands. EMPD mostly progresses slowly and is often diagnosed as carcinoma in situ ; however, upon becoming invasive, it promptly and frequently metastasizes to regional lymph nodes, leading to subsequent distant metastasis. To date, several chemotherapy regimens have been used to treat metastatic EMPD; however, they present limited effect and patients with distant metastasis exhibit a poor prognosis...
2018: Frontiers in Oncology
E N Gonc, A Ozon, G Buyukyilmaz, A Alikasifoglu, O P Simsek, N Kandemir
Juvenile Paget's disease (JPD) is a rare autosomal recessive osteopathy. There is still a question about the most effective treatment modality in long-term prognosis. A 9-month-old boy who suffered from bone pain and deformities with a very high alkaline phosphatase level was diagnosed as JPD by radiographic findings. Genetic analysis showed a homozygous large deletion in TNFRSF11B gene encoding osteoprotegerin. Clinical improvement was observed with intravenous pamidronate therapy. However, the effect of drug reduced in time so the annual dose per kilogram body weight was increased after 2 years...
March 3, 2018: Osteoporosis International
Chiara Rossetti, Lidia Pasquinelli, Andrea Verzeletti, Giuseppe Armocida, Marta Licata, Ezio Fulcheri
An excavation conducted in 2002 by the Lombardy Archaeological Heritage in the St. Giulio Church (Cassano Magnago-Northern Italy) revealed an adult skeleton with an important alteration of the left femur. The femur, longer than the contralateral, exhibited an expansion of the full diaphysis; the surface was coarsened and porous. In cross section, it appeared that almost all the cortical bone had been converted into cancellous bone. Macroscopic and radiological alterations are suggestive of the monostotic form of Paget's disease, a chronic disorder, characterized by focal areas of excessive osteoclastic bone resorption followed by a secondary increase in osteoblastic bone formation...
March 2018: International Journal of Paleopathology
G Divisato, F S di Carlo, N Petrillo, T Esposito, F Gianfrancesco
Paget's disease of bone (PDB) is a skeletal disorder whose molecular basis are not fully elucidated. However, 10% of patients show a familial PDB and 35% of them carry mutations in the SQSTM1 gene. We recently reported a founder mutation (p.Pro937Arg) in the ZNF687 gene, underlying PDB complicated by Giant Cell Tumor (GCT/PDB) and rarely occurring in PDB patients without neoplastic degeneration. Since 80% of Italian GCT/PDB patients derive from Avellino, we hypothesized that ZNF687 mutation rate was higher in this region than elsewhere...
March 1, 2018: Clinical Genetics
Rajendra Kumar, Meena Kumar, Kavin Malhotra, Shreyaskumar Patel
PURPOSE OF REVIEW: Osteosarcoma is mostly seen in bones of children and young adults. When it occurs in older persons, the tumor is considered secondary usually complicating Paget disease or irradiated bone. However, there is a second incidence peak of primary osteosarcoma later in life when these tumors occur de novo. This article describes the clinical, imaging, and treatment of POS in older patients, including demographic data of patients from our institution. FINDINGS: We present our experience with 920 cases of osteosarcoma that were seen between 1984 and 2003 at the University of Texas MD Anderson Cancer Center in Houston, TX, USA...
February 28, 2018: Current Oncology Reports
Lihong Fan, Shutao Yin, Enxiang Zhang, Hongbo Hu
p62 is a multifunctional adaptor protein implicated in various cellular processes. It has been found to regulate selective autophagy, cell survival, cell death, oxidative stress, DNA repair and inflammation, and to play a role in a number of diseases, such as tumourigenesis, Paget's disease of bone, neurodegenerative disease, diabetes, and obesity. Cell death induction is an important cellular process. The dysregulation of cell death induction is involved in the pathogenesis of various diseases, such as cancer, neurodegeneration diseases, and diabetes...
February 26, 2018: Apoptosis: An International Journal on Programmed Cell Death
Kou Fujisawa, Motoi Kato, Tatsuki Kono, Hiroki Utsunomiya, Azusa Watanabe, Shoji Watanabe
Nipple adenoma is an uncommon proliferative process of the breast and predominantly occurs in women aged 40-50. Its incidence is extremely low in men, and it has not been reported in a boy. Although nipple adenoma is rare and benign, being familiar with it is important because it clinically resembles Paget disease and histologically adenocarcinoma. We report a case of nipple adenoma in a boy.
February 26, 2018: Pediatric Dermatology
Fernando Mota, Miguel Horta, Claudia Marques, Susan Foreid, Manuela Selores
Extramammary Paget disease of the vulva is a rare condition that accounts for only 1-2% of vulvar malignancies and represents a frequent cause of misdiagnosis. It is most commonly seen in postmenopausal women. Clinically it is similar to Paget disease of the breast, appearing as red, well-demarcated eczematoid lesions, with slightly raised edges. A high degree of clinical suspicion is very important when evaluating these lesions in order to avoid misdiagnosis and delay of effective treatment. We present a case of vulvar Paget disease treated with vulvectomy...
October 15, 2017: Dermatology Online Journal
Caitlin M Seykora, Scott R Dalton, Patrick J Brown J Brown
A 12-month-old boy presented with three months of asymptomatic hypopigmented flat-topped papules on the suprapubic skin and lower abdomen. Emollients and topical steroids offered no improvementand the patient was referred to the dermatology department. Shave biopsy revealed a papillated epidermis with scattered solitary mononuclearclear cells at all levels of the epidermis and an overlying basket weave orthokeratosis. The cells were epithelioid with increased amphophilic cytoplasm. Immunohistochemical staining was positive for CK7,CEA, and CAM5...
January 15, 2018: Dermatology Online Journal
Pelvender S Gill, Newton A C S Wong
AIMS: The currently recognised subtypes of anal canal/perianal adenocarcinoma are those arising from low rectal mucosa or columnar cuff, fistula-related tumours and anal gland carcinoma. This following report presents two examples of a hitherto undescribed subtype of perianal adenocarcinoma with an intestinal phenotype. METHODS AND RESULTS: A 74 year old man had a perianal tumour locally excised whereas a 73 year old female underwent an abdominoperineal resection for perianal Paget's disease with an underlying carcinoma...
February 21, 2018: Histopathology
Jason Singh, David Row, Tim Schaub, Nathalie C Zeitouni
No abstract text is available yet for this article.
February 15, 2018: Dermatologic Surgery: Official Publication for American Society for Dermatologic Surgery [et Al.]
YouJin Lee, Per Harald Jonson, Jaakko Sarparanta, Johanna Palmio, Mohona Sarkar, Anna Vihola, Anni Evilä, Tiina Suominen, Sini Penttilä, Marco Savarese, Mridul Johari, Marie-Christine Minot, David Hilton-Jones, Paul Maddison, Patrick Chinnery, Jens Reimann, Cornelia Kornblum, Torsten Kraya, Stephan Zierz, Carolyn Sue, Hans Goebel, Asim Azfer, Stuart H Ralston, Peter Hackman, Robert C Bucelli, J Paul Taylor, Conrad C Weihl, Bjarne Udd
Multisystem proteinopathy (MSP) involves disturbances of stress granule (SG) dynamics and autophagic protein degradation that underlie the pathogenesis of a spectrum of degenerative diseases that affect muscle, brain, and bone. Specifically, identical mutations in the autophagic adaptor SQSTM1 can cause varied penetrance of 4 distinct phenotypes: amyotrophic lateral sclerosis (ALS), frontotemporal dementia, Paget's disease of the bone, and distal myopathy. It has been hypothesized that clinical pleiotropy relates to additional genetic determinants, but thus far, evidence has been lacking...
February 19, 2018: Journal of Clinical Investigation
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