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https://www.readbyqxmd.com/read/27921168/the-2016-diagnostic-criteria-for-primary-sclerosing-cholangitis
#1
Takahiro Nakazawa, Kenji Notohara, Susumu Tazuma, Atsushi Tanaka, Hiroyuki Isayama, Toshio Tsuyuguchi, Toshiyuki Mori, Hajime Takikawa
BACKGROUND: Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease of unknown cause that is characterized pathologically by an inflammatory and fibrotic process centered on the epithelium, leading to diffuse biliary stenosis and increased wall thickness throughout the intra- and extra-hepatic biliary trees. A nationwide survey in Japan revealed several differences in the clinical aspects of PSC between Japan and Western countries. PSC was recently designated an intractable hepatobiliary disease in Japan...
December 5, 2016: Journal of Gastroenterology
https://www.readbyqxmd.com/read/27921051/systemic-mastocytosis-causing-refractory-pruritus-in-a-liver-disease-patient
#2
Naga S Addepally, Jagpal S Klair, Mohit Girotra, Johnny Jones, Farshad Aduli
Systemic mastocytosis (SM) results from clonal, neoplastic proliferation of abnormal mast cells. Patients become susceptible to itching, urticaria, and anaphylactic shock, which occurs due to histamine release from mast cells. SM may coexist alongside other systemic diseases, thus confounding the overall clinical presentation. We discuss a 23-year-old woman with refractory pruritus, which was initially attributed to primary sclerosing cholangitis but had a nonresponse to antihistaminics, ursodiol, and cholestyramine...
August 2016: ACG Case Reports Journal
https://www.readbyqxmd.com/read/27920848/a-systematic-investigation-of-sclerosing-mesenteritis-through-ct-and-mri
#3
Sotirios Apostolakis, Argyrios Ioannidis, Garyfalia Tsioga, Konstantina Papageorgiou, Georgios Velimezis
Sclerosing mesenteritis is primarily diagnosed through histologic and radiologic evaluation; however, only a few works provide a systematic description using MRI. This work presents the case of a 68-year-old male, who was admitted for a routine cholecystectomy. Intraoperativly, a large mass was identified dislocating the abdominal viscera. The microscopic examination revealed vascular congestion of the omentum. The contrast-enhanced CT and MRI scans revealed the presence of a heterogenous, lipomatous mass with lesions visible only in T2W and contrast-enhanced T1W MRI...
December 2016: Radiology case reports
https://www.readbyqxmd.com/read/27916678/incidence-risk-factors-and-outcomes-of-colorectal-cancer-in-patients-with-ulcerative-colitis-with-low-grade-dysplasia-a-systematic-review-and-meta-analysis
#4
REVIEW
Mathurin Fumery, Parambir S Dulai, Samir Gupta, Larry J Prokop, Sonia Ramamoorthy, William J Sandborn, Siddharth Singh
BACKGROUND & AIMS: Little is known about outcomes of patients with ulcerative colitis with low-grade dysplasia (UC-LGD). We estimated the incidence of and risk factors for progression to colorectal cancer (CRC) in cohorts of patients with UC-LGD who underwent surveillance (surveillance cohort), and the prevalence of dysplasia-related findings among patients who underwent colectomy for UC-LGD (surgical cohort). METHODS: We performed a systematic literature review through June 1, 2016 to identify cohort studies of adults with UC-LGD...
December 1, 2016: Clinical Gastroenterology and Hepatology
https://www.readbyqxmd.com/read/27912077/nlrp3-activation-induces-il-18-synthesis-and-affects-the-epithelial-barrier-function-in-reactive-cholangiocytes
#5
Luca Maroni, Laura Agostinelli, Stefania Saccomanno, Claudio Pinto, Debora Giordano, Chiara Rychlicki, Samuele De Minicis, Luciano Trozzi, Jesus M Banales, Espen Melum, Tom H Karlsen, Antonio Benedetti, Gianluca Svegliati Baroni, Marco Marzioni
Microbial products are thought to influence the progression of cholangiopathies, in particular primary sclerosing cholangitis (PSC). Inflammasomes are molecular platforms that respond to microbial products through the synthesis of proinflammatory cytokines. We investigated the role of inflammasome activation in cholangiocyte response to injury. Nucleotide-binding oligomerization domain (NOD)-like receptor family, pyrin domain-containing protein 3 (Nlrp3) expression was tested in cholangiocytes of normal and cholestatic livers...
November 29, 2016: American Journal of Pathology
https://www.readbyqxmd.com/read/27911069/-urological-pathology-of-lymphatic-origin
#6
A Domènech, A Serrano, I Forner-Cordero, F Gómez, D Maldonado, C Domínguez
OBJECTIVES: Lymphatic disease is a rarely cause of some very unspecific genitourinary manifestations, assuming a diagnostic challenge in most cases. The aim of this paper is to warn about the possible etiology of these urological lymphatic presentations and discuss its management. METHODS: Retrospective review of clinical data in pediatric patients with urological pathology of lymphatic origin between 2008-2014. Three patients, two boys and a girl, were included...
January 25, 2016: Cirugía Pediátrica: Organo Oficial de la Sociedad Española de Cirugía Pediátrica
https://www.readbyqxmd.com/read/27910944/thyroid-sclerosing-mucoepidermoid-carcinoma-with-eosinophilia-a-clinicopathologic-and-molecular-analysis-of-a-distinct-entity
#7
Akeesha A Shah, Kristin La Fortune, Caitlyn Miller, Stacey E Mills, Zubair Baloch, Virginia LiVolsi, Sanja Dacic, Alyssa L Mahaffey, Marina Nikiforova, Yuri E Nikiforov, Raja R Seethala
Sclerosing mucoepidermoid carcinoma with eosinophilia is a rare thyroid neoplasm of uncertain pathogenesis that resembles salivary gland mucoepidermoid carcinoma. This multi-institutional study characterizes the clinicopathologic and molecular features of this tumor by utilizing next-generation sequencing to assess common mutations and gene fusions involved in thyroid carcinogenesis as well as fluorescence in-situ hybridization for MAML2 translocations typical of salivary gland mucoepidermoid carcinoma. Nine cases (6 females and 3 males, mean age: 59 years, range 30-77 years) were identified...
December 2, 2016: Modern Pathology: An Official Journal of the United States and Canadian Academy of Pathology, Inc
https://www.readbyqxmd.com/read/27909285/-multiple-pulmonary-sclerosing-pneumocytoma-with-abnormal-accumulation-of-fluorodeoxyglucose-positron-emission-tomography-diagnosed-by-surgical-treatment-report-of-a-case
#8
Kantaro Hara, Nobuhiro Izumi, Takuma Tsukioka, Hiroaki Komatsu, Satoshi Okada, Michihito Toda, Ryuichi Ito, Toshihiko Shibata, Noritoshi Nishiyama
A 36-year-old Chinese woman was referred to our hospital for further examination of an abnormal shadow on chest X-ray. Chest computed tomography(CT) revealed a 5 mm circular nodule in diameter in right lung S3 and a 32 mm mass in diameter in right lung S7. The S7 mass showed an abnormal accumulation of SUVmax=4.0 on positron emission tomography( PET)-CT. Transbronchial biopsy was performed, but failed to rule out possible malignancy. Differential diagnoses were multiple lung benign tumor, multiple lung metastases from unknown primary cancer, malignant lymphoma or primary lung cancer, and so on...
December 2016: Kyobu Geka. the Japanese Journal of Thoracic Surgery
https://www.readbyqxmd.com/read/27908204/clinical-implications-of-serial-versus-isolated-biliary-fluorescence-in-situ-hybridization-fish-polysomy-in-primary-sclerosing-cholangitis
#9
Kevin P Quinn, James H Tabibian, Keith D Lindor
BACKGROUND: Cholangiocarcinoma (CCA) is a leading cause of death in patients with primary sclerosing cholangitis (PSC). Biliary polysomy detected by fluorescence in situ hybridization (FISH) helps to identify patients with early CCA, but not all PSC patients with polysomy develop CCA. Here, we examined the features and clinical outcomes of PSC patients with serial versus isolated polysomy. METHODS: All patients with PSC who underwent ≥1 endoscopic retrograde cholangiography (ERC) with FISH testing at Mayo Clinic, Rochester from 2008-2011 were identified...
December 1, 2016: Scandinavian Journal of Gastroenterology
https://www.readbyqxmd.com/read/27904825/can-acetazolamide-be-used-to-treat-diseases-involving-increased-bone-mineral-density
#10
Juan David González-Rodríguez, María Isabel Luis-Yanes, Esther Inglés-Torres, Pedro Arango-Sancho, José Eugenio Cabrera-Sevilla, María Rosario Duque-Fernández, Salvador Gil-Sánchez, Víctor Manuel García-Nieto
Sclerosing bone dysplasias are a series of clinically and genetically heterogeneous diseases characterized by functional failure of the osteoclasts in bone resorption, leading to an excessive amount of bone mineral density (BMD) which could have serious clinical consequences. We treated three children affected with seriously high levels of BMD with acetazolamide, with the intention of inducing metabolic acidosis, thus increasing bone resorption and reducing BMD. All our patients tolerated and followed the treatment well and the clinical response was satisfactory in all cases...
November 2016: Intractable & Rare Diseases Research
https://www.readbyqxmd.com/read/27904822/targeted-sequencing-approach-to-identify-genetic-mutations-in-nasu-hakola-disease
#11
Jun-Ichi Satoh, Motoaki Yanaizu, Youhei Tosaki, Kenji Sakai, Yoshihiro Kino
Nasu-Hakola disease (NHD) is a rare autosomal recessive disorder characterized by sclerosing leukoencephalopathy and multifocal bone cysts, caused by a loss-of-function mutation of either TYROBP (DAP12) or TREM2. TREM2 and DAP12 constitute a receptor/adaptor signaling complex expressed exclusively on osteoclasts, dendritic cells, macrophages, and microglia. Premortem molecular diagnosis of NHD requires genetic analysis of both TYROBP and TREM2, in which 20 distinct NHD-causing mutations have been reported. Due to genetic heterogeneity, it is often difficult to identify the exact mutation responsible for NHD...
November 2016: Intractable & Rare Diseases Research
https://www.readbyqxmd.com/read/27904625/an-intriguing-case-of-gallstone-ileus-after-hepaticojejunostomy-caused-by-a-stone-on-a-suture
#12
Mahir Gachabayov, Petr Mityushin
Gallstone ileus (GI) is a mechanical obstruction of small or large bowel caused by gallstone passed to the intestinal lumen through spontaneous or postoperative biliodigestive fistula. A 42-year-old female patient was admitted with the clinical presentation of small bowel obstruction. She underwent hepaticojejunostomy 4 years prior to admission for primary sclerosing cholangitis. Barium meal follows through revealed Rigler's triad. The patient underwent laparotomy which revealed GI. A "stone on a suture" was removed through enterotomy...
2016: Journal of Research in Medical Sciences: the Official Journal of Isfahan University of Medical Sciences
https://www.readbyqxmd.com/read/27902879/imprint-cytologic-and-immunocytochemical-findings-of-sclerosing-pneumocytoma
#13
Naoto Kuroda, Masahiko Ohara, Keiko Mizuno, Kenji Yorita
To the best of our knowledge, there is no description on immunocytochemical study of sclerosing pneumocytoma (SP) using a cell transfer method. In this article, we report imprint cytological and immunocytochemical findings of SP. Three cases of SP possessing both imprint cytological and histological materials were examined. All tumors showed well-defined margin grossly. On microscopic examination, the tumor consisted of surface and stromal round cells. Cytologically, surface and stromal round cells showed fragments or clusters of papillary configuration with low N/C ratio and cell clusters of small to medium-sized cells with high N/C ration...
November 30, 2016: Diagnostic Cytopathology
https://www.readbyqxmd.com/read/27896649/the-micropapillary-hobnail-variant-of-papillary-thyroid-carcinoma-a-review-of-series-described-in-the-literature-compared-to-a-series-from-one-southern-italy-pathology-institution
#14
REVIEW
Antonio Ieni, Valeria Barresi, Roberta Cardia, Luana Licata, Flavia Di Bari, Salvatore Benvenga, Giovanni Tuccari
Papillary thyroid carcinoma (PTC) has a good prognosis with a 10-yr survival greater than 90%. Recently, a micro-papillary pattern with hobnail appearance (MPHC) in PTC has been indicated as associated with poor prognosis, but this suggestion is based only on a few cases from geographical areas different from ours. Two-hundred ninety-nine consecutive PTC cases were collected between the years of 1992 and 2014 at our institution. The corresponding histologic sections (at least 6 for each case) were stained with hematoxylin and eosin and reviewed independently by two pathologists to reach a consensus on the identification and quantification of the MPHC...
November 28, 2016: Reviews in Endocrine & Metabolic Disorders
https://www.readbyqxmd.com/read/27894790/venous-malformations-of-the-head-and-neck-current-concepts-in-management
#15
REVIEW
C Fowell, C Verea Linares, R Jones, H Nishikawa, A Monaghan
Low-flow venous malformations are congenital lesions and they are the third most common vascular anomaly in the head and neck. In this paper, the third in a series of three educational reviews, we discuss current trends in their management, and include a summary of common sclerosant agents used in their control.
November 25, 2016: British Journal of Oral & Maxillofacial Surgery
https://www.readbyqxmd.com/read/27891750/epidemiology-and-outcomes-of-primary-sclerosing-cholangitis-with-and-without-inflammatory-bowel-disease-in-an-australian-cohort
#16
Ken Liu, Ruoxi Wang, Viraj Kariyawasam, Mark Wells, Simone I Strasser, Geoff McCaughan, Crispin Corte, Rupert W Leong
BACKGROUND&AIMS: Epidemiological data on primary sclerosing cholangitis (PSC) outside the Northern hemisphere is limited. Similarly, the impact of inflammatory bowel disease (IBD) on PSC outcomes remains unclear. We aimed to study the epidemiology and outcomes of PSC patients with and without IBD in an Australian cohort. METHODS: We retrospectively studied PSC patients attending two tertiary referral hospitals over 20years. Diagnosis of PSC was made according to international guidelines by positive cholangiography and/or liver biopsy (for small duct PSC) with supporting clinical and laboratory evidence RESULTS: 208 PSC patients (61% male) were studied (2,271patient-years follow-up)...
November 28, 2016: Liver International: Official Journal of the International Association for the Study of the Liver
https://www.readbyqxmd.com/read/27888671/sclerosing-polycystic-adenosis-of-the-parotid-gland-a-systematic-review-and-report-of-2-new-cases
#17
Carlos A Espinosa, Laura Rua, Héctor E Torres, Álvaro Fernández Del Valle, Rui P Fernandes, Juan C Devicente
PURPOSE: We sought to evaluate clinical-epidemiologic aspects, pathologic characteristics, and treatment of sclerosing polycystic adenosis (SPA) of the parotid gland and to report 2 new cases. MATERIALS AND METHODS: We conducted a systematic review following PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-Analyses) guidelines for studies published in or before June 2016. The inclusion criteria were as follows: final diagnosis of SPA affecting the parotid gland and articles published in Spanish or English...
November 2, 2016: Journal of Oral and Maxillofacial Surgery
https://www.readbyqxmd.com/read/27885065/effectiveness-of-mycophenolate-mofetil-as-first-line-therapy-in-aqp4-igg-mog-igg-and-seronegative-neuromyelitis-optica-spectrum-disorders
#18
Alexis Montcuquet, Nicolas Collongues, Caroline Papeix, Helene Zephir, Bertrand Audoin, David Laplaud, Bertrand Bourre, Bruno Brochet, Jean-Philippe Camdessanche, Pierre Labauge, Thibault Moreau, David Brassat, Bruno Stankoff, Jerome de Seze, Sandra Vukusic, Romain Marignier
OBJECTIVE: To evaluate the effectiveness and tolerance of mycophenolate mofetil (MMF) as a first-line treatment in neuromyelitis optica spectrum disorder (NMOSD). METHODS: In all, 67 NMOSD patients treated by MMF as first-line therapy, from the NOMADMUS cohort were included. A total of 65 fulfilled 2015 NMOSD criteria, and 5 were myelin oligodendrocyte glycoprotein (MOG)-immunoglobulin G (IgG) positive. Effectiveness was evaluated on percentage of patients continuing MMF, percentage of patients free of relapse, pre- and post-treatment change in the annualized relapse rate (ARR), and Expanded Disability Status Scale (EDSS)...
November 24, 2016: Multiple Sclerosis: Clinical and Laboratory Research
https://www.readbyqxmd.com/read/27882046/the-role-of-genetic-and-immune-factors-for-the-pathogenesis-of-primary-sclerosing-cholangitis-in-childhood
#19
REVIEW
Priscila Menezes Ferri, Ana Cristina Simões E Silva, Soraya Luiza Campos Silva, Diego Junior Queiroga de Aquino, Eleonora Druve Tavares Fagundes, Débora Marques de Miranda, Alexandre Rodrigues Ferreira
Primary sclerosing cholangitis (PSC) is a rare cholestatic liver disease characterized by chronic inflammation of the biliary tree resulting in liver fibrosis. PSC is more common in male less than 40 years of age. The diagnosis of PSC is based on clinical, laboratory, image, and histological findings. A biochemical profile of mild to severe chronic cholestasis can be observed. Endoscopic retrograde cholangiography is the golden standard method for diagnosis, but magnetic resonance cholangiography is currently also considered a first-line method of investigation...
2016: Gastroenterology Research and Practice
https://www.readbyqxmd.com/read/27880989/validation-of-the-prognostic-value-of-histologic-scoring-systems-in-primary-sclerosing-cholangitis-an-international-cohort-study
#20
Elisabeth M G De Vries, Manon de Krijger, Martti Farkkila, Johanna Arola, Peter Schirmacher, Daniel Gotthardt, Benjamin Goeppert, Palak J Trivedi, Gideon M Hirschfield, Henriette Ytting, Ben Vainer, Henk R van Buuren, Katharina Biermann, Maren H Harms, Olivier Chazouilleres, Dominique Wendum, Astrid Donald Kemgang, Roger W Chapman, Lai Mun Wang, Kate D Williamson, Annette S H Gouw, Valerie Paradis, Christine Sempoux, Ulrich Beuers, Stefan Hubscher, Joanne Verheij, Cyriel Ponsioen
: Histologic scoring systems specific for primary sclerosing cholangitis (PSC) are not validated. We recently determined the applicability and prognostic value of three histological scoring systems in a single PSC cohort. The aim of this study was to validate their prognostic utility and reproducibility, across a multicenter PSC cohort. Liver biopsies from PSC patients were collected across 7 European centers. Histologic scoring was performed using the Nakanuma, Ishak, and Ludwig scoring systems...
November 23, 2016: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
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