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https://www.readbyqxmd.com/read/28087208/-child-langerhans-cell-histiocytosis
#1
Jean Donadieu, Sébastien Héritier
DEFINITION: Langerhans cell histiocytosis (LCH) is defined by the association of a clinical and radiological involvement and a biopsy of a pathological tissue. Extension: it can affect any organ or system of the body but most commonly the bone (80% of cases), the skin (33%) and the pituitary (25%). Other organs are concerned such as liver, spleen, hematopoietic system and the lungs (15% each), lymph nodes (5-10%) and central nervous system (CNS) excluding the pituitary (2-4%). Natural history: the natural history of the disease is very heterogeneous, ranging from auto-regressive lesions to a disease affecting multiple organs with fatal consequences, while some lesions may be responsible for permanent sequels...
January 10, 2017: La Presse Médicale
https://www.readbyqxmd.com/read/28086796/prophylaxis-of-post-erc-infectious-complications-in-patients-with-biliary-obstruction-by-adding-antimicrobial-agents-into-erc-contrast-media-a-single-center-retrospective-study
#2
Hella Wobser, Agnetha Gunesch, Frank Klebl
BACKGROUND: Patients with biliary obstruction are at high risk to develop septic complications after endoscopic retrograde cholangiography (ERC). We evaluated the benefits of local application of antimicrobial agents into ERC contrast media in preventing post-ERC infectious complications in a high-risk study population. METHODS: Patients undergoing ERC at our tertiary referral center were retrospectively included. Addition of vancomycin, gentamicin and fluconazol into ERC contrast media was evaluated in a case-control design...
January 13, 2017: BMC Gastroenterology
https://www.readbyqxmd.com/read/28079751/updates-on-primary-sclerosing-cholangitis
#3
Chantal Housset
No abstract text is available yet for this article.
January 12, 2017: Current Opinion in Gastroenterology
https://www.readbyqxmd.com/read/28079297/the-effectiveness-of-diclofenac-sodium-in-the-treatment-of-mondor-s-disease-of-the-breast-the-topical-patch-compared-to-the-oral-capsules
#4
Bader Hamza Shirah, Hamza Assad Shirah, Wedad Salem Alonazie
Mondor's disease of the breast is a rare, benign sclerosing superficial thrombophlebitis of the subcutaneous veins of the anterior or lateral chest wall, which is treated conservatively. We aim in this study to evaluate the outcome and effectiveness of our treatment protocol using oral diclofenac sodium and topical diclofenac sodium patch in 172 patients. A retrospective database analysis of 172 female patients between January 2001 and December 2010 was done. The treatment protocol consisted of group 1: treatment by oral diclofenac sodium 100 mg once daily for 3 weeks...
January 12, 2017: Breast Journal
https://www.readbyqxmd.com/read/28078379/chitosan-sodium-tetradecyl-sulfate-hydrogel-characterization-and-preclinical-evaluation-of-a-novel-sclerosing-embolizing-agent-for-the-treatment-of-endoleaks
#5
Fatemeh Zehtabi, Vincent Dumont-Mackay, Ahmed Fatimi, Antony Bertrand-Grenier, Hélène Héon, Gilles Soulez, Sophie Lerouge
PURPOSE: To compare the efficacy of an embolization agent with sclerosing properties (made of chitosan and sodium tetradecyl sulfate, CH-STS) with a similar embolization agent but without sclerosing properties (made of chitosan, CH) in treating endoleaks in a canine endovascular aneurysm repair model. METHODS: Two chitosan-based radiopaque hydrogels were prepared, one with STS and one without STS. Their rheological, injectability, and embolizing properties were assessed in vitro; afterwards, their efficacy in occluding endoleaks was compared in a canine bilateral aneurysm model reproducing type I endoleaks (n = 9 each)...
January 11, 2017: Cardiovascular and Interventional Radiology
https://www.readbyqxmd.com/read/28077782/serum-and-tissue-markers-in-hepatocellular-carcinoma-and-cholangiocarcinoma-clinical-and-prognostic-implications
#6
REVIEW
Massimiliano Berretta, Carla Cavaliere, Lara Alessandrini, Brigida Stanzione, Gaetano Facchini, Luca Balestreri, Tiziana Perin, Vincenzo Canzonieri
HCC represents the sixth most common cancer worldwide and the second leading cause of cancer-related death. Despite the high incidence, treatment options for advanced HCC remain limited and unsuccessful, resulting in a poor prognosis. Despite the major advances achieved in the diagnostic management of HCC, only one third of the newly diagnosed patients are presently eligible for curative treatments. Advances in technology and an increased understanding of HCC biology have led to the discovery of novel biomarkers...
December 14, 2016: Oncotarget
https://www.readbyqxmd.com/read/28077023/hiv-associated-sub-acute-sclerosing-panencephalitis-an-emerging-threat
#7
Abi Manesh, Mahesh Moorthy, Rini Bandopadhyay, Priscilla Rupali
Earlier age of measles virus infection predisposes to development of sub-acute sclerosing panencephalitis (SSPE) and this risk is heightened in HIV-infected children. We describe a HIV-infected young adult on antiretroviral therapy, presenting with a non-classical, fulminant form of SSPE to highlight the unpredictable nature of measles presentation. The recent spate of measles outbreaks due to virus introduction in populations with sub-optimal vaccine coverage or waning immunity and co-existing paediatric HIV cohorts is a cause for concern...
January 1, 2017: International Journal of STD & AIDS
https://www.readbyqxmd.com/read/28074536/use-of-an-intravenous-sclerosing-foam-3-sodium-tetradecyl-sulfate-for-treatment-of-orbital-varix-in-a-dog
#8
Rebecca S Saunders, Brian A Scansen, Stephen S Jung, Hooman Khabiri, David A Wilkie
A 6-month-old intact male Standard Dachshund was referred for evaluation of a soft tissue swelling above the right eye. Examination of the right eye revealed mild lateral deviation of the globe, normal vision, and a dorsonasal soft tissue swelling. Examination of the posterior segment was normal. Dual-phase computed tomography angiography was consistent with an orbital varix of the angularis oculi and right dorsal external ophthalmic veins with no evidence of arterial involvement. Treatment involved fluoroscopically guided coil embolization of the venous outflow with nine platinum microcoils, followed by sclerotherapy of the varix using 1...
January 10, 2017: Veterinary Ophthalmology
https://www.readbyqxmd.com/read/28073151/ccbe1-mutation-causing-sclerosing-cholangitis-expanding-the-spectrum-of-lymphedema-cholestasis-syndrome
#9
André Viveiros, Marion Reiterer, Benedikt Schaefer, Armin Finkenstedt, Stefan Schneeberger, Hubert Schwaighofer, Patrizia Moser, Rudolf Sprenger, Bernhard Glodny, Wolfgang Vogel, Andreas R Janecke, Heinz Zoller
No abstract text is available yet for this article.
January 10, 2017: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
https://www.readbyqxmd.com/read/28070877/idiopathic-retroperitoneal-fibrosis-and-its-overlap-with-igg4-related-disease
#10
REVIEW
Giovanni Maria Rossi, Rossana Rocco, Eugenia Accorsi Buttini, Chiara Marvisi, Augusto Vaglio
Retroperitoneal fibrosis (RPF) is a rare disease characterised by fibrous tissue proliferation in the retroperitoneum, with encasement of the ureters and large vessels of the abdomen as the most destructive of potentially severe complications. It can either be idiopathic, or secondary to infections, malignancies, or the use of certain drugs. The idiopathic form accounts for approximately 75% of the cases, and is usually responsive to immunosuppressive therapy. In recent years, the emergence of a new clinical entity, IgG4-related disease (IgG4-RD), shed light on many fibro-inflammatory disorders once thought to be separate clinical entities, although frequently associated in the so-called multifocal fibrosclerosis...
January 9, 2017: Internal and Emergency Medicine
https://www.readbyqxmd.com/read/28065639/imaging-features-and-diagnosis-of-tuberculosis-of%C3%A2-the-breast
#11
C F Longman, T Campion, B Butler, T D Suaris, A Khanam, H Kunst, S Tiberi, S A O'Keeffe
AIM: To outline the pathophysiology, clinical presentation, imaging features, and relevant investigations of the different subtypes of breast tuberculosis (TB). MATERIALS AND METHODS: A review was undertaken of all cases (33 in total) of breast TB presenting to Barts Health NHS Trust within a 10-year period, including patient demographics, imaging features, and route of diagnosis. RESULTS: Thirty-three cases of proven granulomatous TB of the breast were identified (11 mastitis obliterans, 10 nodular caseous form, five sclerosing form, four disseminated disease, and three abnormal axillary lymph nodes)...
January 6, 2017: Clinical Radiology
https://www.readbyqxmd.com/read/28060780/18f-fdg-pet-ct-features-of-chronic-sclerosing-sialadenitis-presenting-as-localized-igg4-related-disease
#12
Nobuo Kashiwagi, Eisuke Enoki, Chisa Hosokawa, Kenta Sakaguchi, Takamichi Murakami
Chronic sclerosing sialadenitis is a benign inflammatory condition that most commonly affects the submandibular gland in elderly individuals. It is currently known to belong to the spectrum of IgG4-related systemic diseases, which is reflected by systemic involvement on F-FDG PET/CT images. We presented a case of a 73-year-old man with histologically proven IgG4-related chronic sclerosing sialadenitis, unilateral localized form, on whole-body F-FDG PET/CT images that mimicked submandibular carcinoma with lymph node metastasis...
February 2017: Clinical Nuclear Medicine
https://www.readbyqxmd.com/read/28060365/newly-described-salivary-gland-tumors
#13
Alena Skalova, Michal Michal, Roderick Hw Simpson
This review concentrates on three salivary gland tumors that have been accepted in the recent literature as new neoplastic entities: mammary analog secretory carcinoma (MASC), sclerosing polycystic adenoma (SPA) and cribriform adenocarcinoma of tongue and other minor salivary glands (CAMSGs). MASC is a distinctive low-grade malignant salivary cancer that harbors a characteristic chromosomal translocation, t(12;15) (p13;q25) resulting in an ETV6-NTRK3 fusion. SPA is a rare lesion often mistaken histologically for low-grade salivary carcinoma...
January 2017: Modern Pathology: An Official Journal of the United States and Canadian Academy of Pathology, Inc
https://www.readbyqxmd.com/read/28056976/a-novel-approach-to-genome-wide-association-analysis-identifies-genetic-associations-with-primary-biliary-cholangitis-and-primary-sclerosing-cholangitis-in-polish-patients
#14
Agnieszka Paziewska, Andrzej Habior, Agnieszka Rogowska, Włodzimierz Zych, Krzysztof Goryca, Jakub Karczmarski, Michalina Dabrowska, Filip Ambrozkiewicz, Bozena Walewska-Zielecka, Marek Krawczyk, Halina Cichoz-Lach, Piotr Milkiewicz, Agnieszka Kowalik, Krzysztof Mucha, Joanna Raczynska, Joanna Musialik, Grzegorz Boryczka, Michal Wasilewicz, Irena Ciecko-Michalska, Malgorzata Ferenc, Maria Janiak, Alina Kanikowska, Rafal Stankiewicz, Marek Hartleb, Tomasz Mach, Marian Grzymislawski, Joanna Raszeja-Wyszomirska, Ewa Wunsch, Tomasz Bobinski, Michal Mikula, Jerzy Ostrowski
BACKGROUND: Primary biliary cholangitis (PBC) and primary sclerosing cholangitis (PSC) are forms of hepatic autoimmunity, and risk for both diseases has a strong genetic component. This study aimed to define the genetic architecture of PBC and PSC within the Polish population. METHODS: Subjects were 443 women with PBC, 120 patients with PSC, and 934 healthy controls recruited from Gastroenterology Departments in various Polish hospitals. Allelotyping employed a pooled-DNA sample-based genome-wide association study (GWAS) approach, using Illumina Human Omni2...
January 6, 2017: BMC Medical Genomics
https://www.readbyqxmd.com/read/28056490/knockout-of-the-primary-sclerosing-cholangitis-risk-gene-fut2-causes-liver-disease-in-mice
#15
Luca Maroni, Simon D Hohenester, Stan F J van de Graaf, Dagmar Tolenaars, Krijn van Lienden, Joanne Verheij, Marco Marzioni, Tom H Karlsen, Ronald P J Oude Elferink, Ulrich Beuers
: The etiopathogenesis of primary sclerosing cholangitis (PSC) is unknown. Genetic variants of fucosyltransferase 2 (FUT2) have been identified in genome-wide association studies as risk factor for PSC. We investigated the role of Fut2 in murine liver pathophysiology by studying Fut2(-/-) mice. Fut2(-/-) mice were viable and fertile, had lower body weight than wild type (wt) littermates and grey fur, as described earlier. Half of the Fut2(-/-) mice showed serum bile salt levels 40 times higher than wt (Fut2(-/-)(high) ), whereas the remainder were normocholanemic (Fut2(-/-)(low) )...
January 5, 2017: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
https://www.readbyqxmd.com/read/28055006/biliary-tract-instillation-of-a-smac-mimetic-induces-trail-dependent-acute-sclerosing-cholangitis-like-injury-in-mice
#16
Maria Eugenia Guicciardi, Anuradha Krishnan, Steven F Bronk, Petra Hirsova, Thomas S Griffith, Gregory J Gores
Primary sclerosing cholangitis (PSC) is a cholestatic liver disease of unknown etiopathogenesis characterized by fibrous cholangiopathy of large and small bile ducts. Systemic administration of a murine TNF-related apoptosis-inducing ligand (TRAIL) receptor agonist induces a sclerosing cholangitis injury in C57BL/6 mice, suggesting endogenous TRAIL may contribute to sclerosing cholangitis syndromes. Cellular inhibitor of apoptosis proteins (cIAP-1 and cIAP-2) are negative regulators of inflammation and TRAIL receptor signaling...
January 5, 2017: Cell Death & Disease
https://www.readbyqxmd.com/read/28052628/management-of-cholestatic-disease-in-2017
#17
REVIEW
Elsemieke de Vries, Ulrich Beuers
Primary biliary cholangitis (PBC) and primary sclerosing cholangitis (PSC) are the most frequent chronic cholestatic liver diseases and serve as model diseases to discuss the management of cholestasis in 2017 in the lecture that is summarized in this report. PBC and PSC are characterized by inflammation and fibrosis of small intrahepatic (PBC) or larger intra- and/or extrahepatic (PSC) bile ducts. Bile duct damage leads to cholestasis and can progress to liver fibrosis and even cirrhosis. Various genetic, environmental and endogenous factors may contribute to the development of chronic cholestatic liver diseases, but the exact pathogenesis of PBC and PSC has not been clarified...
January 2017: Liver International: Official Journal of the International Association for the Study of the Liver
https://www.readbyqxmd.com/read/28050782/colorectal-cancer-in-inflammatory-bowel-diseases-a-population-based-study-in-utah
#18
N Jewel Samadder, John F Valentine, Stephen Guthery, Harminder Singh, Charles N Bernstein, Yuan Wan, Jathine Wong, Kenneth Boucher, Lisa Pappas, Kerry Rowe, Mary Bronner, Cornelia M Ulrich, Randall W Burt, Karen Curtin, Ken R Smith
BACKGROUND AND AIMS: The molecular, endoscopic, and histological features of IBD-associated CRC differ from sporadic CRC. The objective of this study was to describe the prevalence, clinical features, and prognosis of IBD-associated CRC compared to patients with sporadic CRC in a US statewide population-based cohort. METHODS: All newly diagnosed cases of CRC between 1996 and 2011 were obtained from Utah Cancer Registry. IBD was identified using a previously validated algorithm, from statewide databases of Intermountain Healthcare, University of Utah Health Sciences, and the Utah Population Database...
January 3, 2017: Digestive Diseases and Sciences
https://www.readbyqxmd.com/read/28050378/large-cell-calcifying-sertoli-cell-tumour-of-testis-a-rare-case-report
#19
Harresh Kumar, Nadeem Tanveer, Natasha Gupta, Kiran Mishra
Sertoli cell tumours of testes are classified into sertoli cell tumour NOS (not otherwise specified), sclerosing variant and large cell calcifying variant. So far, 90 cases of the large cell calcifying variant have been reported in literature. We describe a rare case of inhibin negative locally invasive large cell calcifying sertoli cell tumour of testis. A 62-year-old man presented with complaints of pain and swelling in right scrotum for 8 months. Ultrasound revealed a right testicular mass with internal vascularity and calcification...
November 2016: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/28050039/impact-of-crohn-s-disease-duration-on-the-risk-of-cholangiocarcinoma-in-patients-with-primary-sclerosing-cholangitis
#20
De-Jun Cui, Xun Zhao, Mei Hu
No abstract text is available yet for this article.
January 2017: American Journal of Gastroenterology
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