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https://www.readbyqxmd.com/read/28922106/igg4-related-disease-a-patient-with-multiple-organ-involvement
#1
Miroslav Průcha, Lenka Sedláčková
IgG4-related diseases represent a heterogeneous group of conditions characterised by elevated serum IgG4 levels and fibrotic or sclerosing changes in the affected organs or systems accompanied by IgG4-positive plasma cells. A disease associated with IgG4 may affect virtually any organ - salivary glands, periorbital tissue, kidneys, lungs, meninges, aorta, prostate, pericardium or skin. Histopathological findings are uniform, characterised by a major lymphoplasmocytic infiltrate and the presence of IgG4-producing plasma cells, irrespective of the affected site...
2017: Prague Medical Report
https://www.readbyqxmd.com/read/28911147/kif5b-ret-rearrangement-in-a-carcinoma-of-the-thyroid-gland-a-case-report-of-a-fatal-disease
#2
David Viola, Carlotta Giani, Salvatore Mazzeo, Clara Ugolini, Raffaele Ciampi, Eleonora Molinaro, Laura Agate, Nicla Borrelli, Antonio Chella, Gabriella Fontanini, Fulvio Basolo, Rossella Elisei
Background: The diffuse sclerosing variant of papillary thyroid cancer (DSV-PTC) is a rare variant of papillary thyroid cancer (PTC) with different clinicopathological features compared with conventional PTC. Case: An advanced DSV-PTC was diagnosed in a 39-year-old man. The radioiodine posttherapeutic whole-body-scan showed only an uptake in the central neck, whereas the computerized tomography showed multiple latero-cervical and mediastinum lymph node metastases, a single and spiculated lung lesion and multiple bilateral cerebellum metastases...
September 1, 2017: Journal of Clinical Endocrinology and Metabolism
https://www.readbyqxmd.com/read/28905689/igg4-related-cholangiopathy-and-its-mimickers-a-case-report-and-review-highlighting-the-importance-of-early-diagnosis
#3
Kyle Geary, Cemal Yazici, Anita Seibold, Grace Guzman
Immunoglobulin (Ig) G4 (IgG4)-related disease is a recently described clinical entity that can involve multiple organs. It is an autoimmune disorder characterized by a dense lymphoplasmacytic infiltrate, storiform fibrosis, and obliterative phlebitis. A key distinguishing factor is its dramatic response to steroid therapy. Although best described in cases of autoimmune pancreatitis, IgG4-related disease has also been implicated in patients with cholangitis and is now commonly referred to as IgG4-related cholangiopathy...
September 1, 2017: International Journal of Surgical Pathology
https://www.readbyqxmd.com/read/28870031/-the-clinical-characteristics-of-346-patients-with-igg4-related-disease
#4
P P Zhang, J Z Zhao, M Wang, R E Feng, X W Liu, X M Lai, X J Li, J G Zeng, H J Shi, H D Zhu, W Xue, H Zhang, Y Y Chen, L Y Fei, X F Peng, F C Zeng, Y M Zhang, W Zhang
Objective: To analyze the clinical characteristics of IgG4-related disease (IgG4-RD)so as to improve the understanding of IgG4-RD in China. Methods: IgG4-RD patients were recruited from Peking Union Medical College Hospital between January 2011 and January 2016. All patients were followed-up for more than 6 months. The demographic characteristics, symptoms, organ involvements, laboratory examinations and treatment efficacy were evaluated and analyzed. Results: A total of 346 patients were finally enrolled, including 230 males (66...
September 1, 2017: Zhonghua Nei Ke za Zhi [Chinese Journal of Internal Medicine]
https://www.readbyqxmd.com/read/28798005/hodgkin-s-lymphoma-as-a-multiloculated-lung-cavity-lesion
#5
Aisha M Al-Osail, Hind S Al-Saif, Mashail M Al-Hajri, Hajer M Al-Zuhair, Deemah A Al-Abdulhadi, Emad M Al-Osail, Sarah M Al-Hajri
BACKGROUND: Pulmonary manifestation of Hodgkin's lymphoma is common, and recently, single and/or multiple lung cavities with air-fluid levels has been reported as a primary manifestation of Hodgkin's lymphoma. However, the unusual presentation of the lung cavity itself has not been previously reported for Hodgkin's lymphoma, especially as the first presentation of the disease. CASE PRESENTATION: We report a 16-year-old male who presented to the emergency department with chief complaints of fever, unintentional 10-kilogram weight loss, a productive cough and pulmonary cavity for investigation...
May 2017: Gulf Journal of Oncology
https://www.readbyqxmd.com/read/28716013/long-term-renal-survival-of-%C3%AE-3-heavy-chain-deposition-disease-a-case-report
#6
Takayuki Katsuno, Shige Mizuno, Masatsuna Mabuchi, Naotake Tsuboi, Atsushi Komatsuda, Shoichi Maruyama
BACKGROUND: Monoclonal immunoglobulin deposition disease (MIDD) is characterized by the non-amyloid deposition of monoclonal immunoglobulin fragments in the basement membranes. Heavy chain deposition disease (HCDD) is a type of MIDD. HCDD is an extremely rare disease, and only three cases have been reported in Japan up to the present. The prognosis of HCDD is very poor, and optimal treatment has not been established. Only a few cases of HCDD with favorable long-term renal prognosis have been reported to date...
July 17, 2017: BMC Nephrology
https://www.readbyqxmd.com/read/28711597/micrornas-and-extracellular-vesicles-in-cholangiopathies
#7
REVIEW
P Olaizola, P Y Lee-Law, A Arbelaiz, A Lapitz, M J Perugorria, L Bujanda, J M Banales
Cholangiopathies encompass a heterogeneous group of disorders affecting biliary epithelial cells (i.e. cholangiocytes). Early diagnosis, prognosis and treatment still remain clinically challenging for most of these diseases and are critical for adequate patient care. In the past decade, extensive research has emphasized microRNAs (miRs) as potential non-invasive biomarkers and tools to accurately identify, predict and treat cholangiopathies. MiRs can be released extracellularly conjugated with lipoproteins or encapsulated in extracellular vesicles (EVs)...
July 12, 2017: Biochimica et Biophysica Acta
https://www.readbyqxmd.com/read/28695657/hla-haplotypes-in-primary-sclerosing-cholangitis-patients-of-admixed-and-non-european-ancestry
#8
E K K Henriksen, M K Viken, M Wittig, K Holm, T Folseraas, S Mucha, E Melum, J R Hov, K N Lazaridis, B D Juran, O Chazouillères, M Färkkilä, D N Gotthardt, P Invernizzi, M Carbone, G M Hirschfield, S M Rushbrook, E Goode, C Y Ponsioen, R K Weersma, B Eksteen, K K Yimam, S C Gordon, D Goldberg, L Yu, C L Bowlus, A Franke, B A Lie, T H Karlsen
Primary sclerosing cholangitis (PSC) is strongly associated with several human leukocyte antigen (HLA) haplotypes. Due to extensive linkage disequilibrium and multiple polymorphic candidate genes in the HLA complex, identifying the alleles responsible for these associations has proven difficult. We aimed to evaluate whether studying populations of admixed or non-European descent could help in defining the causative HLA alleles. When assessing haplotypes carrying HLA-DRB1*13:01 (hypothesized to specifically increase the susceptibility to chronic cholangitis), we observed that every haplotype in the Scandinavian PSC population carried HLA-DQB1*06:03...
July 11, 2017: HLA
https://www.readbyqxmd.com/read/28690845/diagnostic-autoantibodies-for-autoimmune-liver-diseases
#9
REVIEW
Ban-Hock Toh
Autoimmune liver diseases are conditions of low prevalence that comprise the triad of autoimmune hepatitis, primary biliary cholangitis (cirrhosis) and primary sclerosing cholangitis and their poorly characterised overlapping syndromes. Diagnostic autoantibodies are associated with autoimmune hepatitis and primary biliary cholangitis but not with primary sclerosing cholangitis. Autoantibodies are useful disease markers that facilitate early diagnosis of autoimmune hepatitis and primary biliary cholangitis and allow for therapeutic intervention to prevent progression to liver cirrhosis and associated complications...
May 2017: Clinical & Translational Immunology
https://www.readbyqxmd.com/read/28676968/melorheostosis-a-rare-sclerosing-bone-dysplasia
#10
REVIEW
Anupam Kotwal, Bart L Clarke
PURPOSE OF REVIEW: Melorheostosis is a rare sclerosing bone dysplasia that affects both cortical bone and adjacent soft tissue structures in a sclerotomal distribution. In this review, we describe the natural history, radiological features, proposed pathogenesis, and management options for this debilitating condition. RECENT FINDINGS: Since its first description in 1922, about 400 cases of melorheostosis have been reported, either as single reports or in small case series...
August 2017: Current Osteoporosis Reports
https://www.readbyqxmd.com/read/28668580/elevated-levels-of-circulating-osteopontin-are-associated-with-a-poor-survival-after-resection-of-cholangiocarcinoma
#11
Sven H Loosen, Christoph Roderburg, Katja L Kauertz, Inês Pombeiro, Catherine Leyh, Fabian Benz, Mihael Vucur, Thomas Longerich, Alexander Koch, Till Braunschweig, Tom F Ulmer, Christoph Heidenhain, Frank Tacke, Marcel Binnebösel, Maximilian Schmeding, Christian Trautwein, Ulf P Neumann, Tom Luedde
BACKGROUND & AIMS: Cholangiocarcinoma (CCA) represents a primary hepatic malignancy with incidence and mortality rising globally. Surgical treatment has remained the only potentially curative treatment option, but it is still unclear which patients benefit most from extended liver surgery, highlighting the need for new pre-operative stratification strategies. Osteopontin is a secreted extracellular glyco-phosphoprotein that has been associated with inflammation, metabolic disorders and cancer...
June 29, 2017: Journal of Hepatology
https://www.readbyqxmd.com/read/28638005/sclerosing-mesenteritis-causing-chylous-ascites-and-small-bowel-perforation
#12
Huei-Wen Lim, Keith S Sultan
BACKGROUND Sclerosing mesenteritis (SM) is a rare idiopathic inflammation and fibrosis of the mesentery. Its etiology and pathogenesis remain unclear. It has been linked to abdominal trauma related to peritoneal dialysis, recent abdominal surgery, idiopathic inflammatory disorder, paraneoplastic syndrome, ischemia/infection, and autoimmunity. Abdominal CT is the most sensitive imaging modality, and diagnosis is usually confirmed by surgical biopsy. Patients most often present with abdominal pain, nausea, vomiting, diarrhea, and weight loss, and less commonly with chylous ascites and small bowel obstruction...
June 22, 2017: American Journal of Case Reports
https://www.readbyqxmd.com/read/28634929/factors-associated-with-multiple-recurrences-of-nonspecific-orbital-inflammation-aka-orbital-pseudotumor
#13
Puneet S Braich, Robin K Kuriakose, Naveen S Khokhar, Jared C Donaldson, Timothy J McCulley
OBJECTIVE: To assess clinical characteristics of patients at risk for recurrent nonspecific orbital inflammation (NSOI). METHODS: This was a retrospective comparison study with a 24-month follow-up, in a clinic-based population in rural India. The medical records at two eye care centers were searched from March 2001-2014. A total of 153 patients with recurrent NSOI, 107 of whom had a single recurrence (Group 1) and 46 of whom had multiple recurrences (Group 2). Clinical and demographic data were compared between both groups...
June 20, 2017: International Ophthalmology
https://www.readbyqxmd.com/read/28610559/sclerosing-mesenteritis-and-mesenteric-panniculitis-clinical-experience-and-radiological-features
#14
Lisa Nyberg, Jan Björk, Peter Björkdahl, Olle Ekberg, Klas Sjöberg, Lina Vigren
BACKGROUND: Sclerosing mesenteritis (SM) is sometimes used as an umbrella-term for idiopathic inflammatory conditions in the mesentery. Mesenteric panniculitis (MP) is a radiological finding and its relation to clinical SM is not fully understood. The aims of this study were to determine whether any correlation could be found between the radiological findings and the clinical disease course. METHODS: Patients observed due to idiopathic inflammation of the mesentery were identified...
June 13, 2017: BMC Gastroenterology
https://www.readbyqxmd.com/read/28569045/a-novel-ews-creb3l3-gene-fusion-in-a-mesenteric-sclerosing-epithelioid-fibrosarcoma
#15
Barbara Dewaele, Louis Libbrecht, Gabriel Levy, Benedicte Brichard, Vanessa Vanspauwen, Raf Sciot, Maria Debiec-Rychter
Sclerosing epithelioid fibrosarcoma (SEF) is a rare, malignant fibroblastic neoplasm, morphologically composed of cords, nests or sheets of monotonous epithelioid cells within a collagenous matrix. It has been recently characterized by recurrent pathogenic EWS-CREB3L1/2 or FUS-CREB3L2 fusions and common MUC4 protein expression by immunohistochemistry. Typically SEF occur in middle-aged adults and rarely have been reported within the abdominal cavity. Here we report an 18-year-old man with intraabdominal tumor and multiple disseminated liver metastases, presenting pure SEF histologic and immunophenotypic features...
May 31, 2017: Genes, Chromosomes & Cancer
https://www.readbyqxmd.com/read/28555885/serum-extracellular-vesicles-contain-protein-biomarkers-for-primary-sclerosing-cholangitis-and-cholangiocarcinoma
#16
Ander Arbelaiz, Mikel Azkargorta, Marcin Krawczyk, Alvaro Santos-Laso, Ainhoa Lapitz, Maria J Perugorria, Oihane Erice, Esperanza Gonzalez, Raul Jimenez-Agüero, Adelaida Lacasta, Cesar Ibarra, Alberto Sanchez-Campos, Juan P Jimeno, Frank Lammert, Piotr Milkiewicz, Marco Marzioni, Rocio I R Macias, Jose J G Marin, Tushar Patel, Gregory J Gores, Ibon Martinez, Félix Elortza, Juan M Falcon-Perez, Luis Bujanda, Jesus M Banales
Cholangiocarcinoma (CCA) includes a heterogeneous group of biliary cancers with poor prognosis. Several conditions, such as primary sclerosing cholangitis (PSC), are risk factors. Noninvasive differential diagnosis between intrahepatic CCA and hepatocellular carcinoma (HCC) is sometimes difficult. Accurate noninvasive biomarkers for PSC, CCA, and HCC are not available. In the search for novel biomarkers, serum extracellular vesicles (EV) were isolated from CCA (n = 43), PSC (n = 30), or HCC (n = 29) patients and healthy individuals (control, n = 32); and their protein content was characterized...
May 26, 2017: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
https://www.readbyqxmd.com/read/28546695/retinal-vascular-occlusion-secondary-to-retrobulbar-injection-case-report-and-literature-review
#17
REVIEW
Dhaivat Vasavada, Prabu Baskaran, Seema Ramakrishnan
Retrobulbar injection has been widely practiced as a technique of ocular anesthesia for many decades. Nevertheless, the technique is not free from complications. Vascular occlusion secondary to retrobulbar injection is rare but can be vision threatening. We report a case series of two such patients who presented with poor vision following retrobulbar injection. Fundus showed pale retina with cherry red spot suggestive of central retinal artery occlusion in case 1 and pale disc with sclerosed vessels and multiple superficial hemorrhages suggestive of a combined occlusion of retinal artery and vein in case 2...
January 2017: Middle East African Journal of Ophthalmology
https://www.readbyqxmd.com/read/28543841/role-of-fluorescence-in-situ-hybridization-in-diagnosing-cholangiocarcinoma-in-indeterminate-biliary-strictures
#18
Z H Liew, T J Z Loh, T K H Lim, T H Lim, C J L Khor, S J Mesenas, C S C Kong, W C Ong, D M Y Tan
BACKGROUND: Brush cytology, the conventional method to diagnose cholangiocarcinoma, has been plagued by low diagnostic sensitivity and false-negative results. We aim to study the clinical utility of fluorescence in situ hybridization (FISH) in enhancing identification of malignant biliary strictures. METHODS: Brush cytologic specimens collected from endoscopic retrograde cholangiopancreatography (ERCP) for biliary strictures in a tertiary hospital in Singapore from March 2013 to July 2015 were examined by FISH technique using UroVysion probe set in this study...
May 19, 2017: Journal of Gastroenterology and Hepatology
https://www.readbyqxmd.com/read/28520586/clinical-course-of-ulcerative-colitis-after-liver-transplantation-in-patients-with-concomitant-primary-sclerosing-cholangitis-and-ulcerative-colitis
#19
Mohammad Reza Fattahi, Seyyed Ali Malek-Hosseini, Gholam Reza Sivandzadeh, Ali Reza Safarpour, Kamran Bagheri Lankarani, Ali Reza Taghavi, Fardad Ejtehadi
BACKGROUND: The natural history of ulcerative colitis (UC) after liver transplantation (LT) for primary sclerosing cholangitis (PSC) remains ill defined. This study aimed to evaluate the course of UC after LT for PSC. METHODS: The course of UC, including the clinical colitis severity index, was evaluated in patients with concomitant PSC and UC who received LT for PSC-induced end-stage liver disease. A total of 167 (55.4%) patients with PSC had concurrent inflammatory bowel disease (IBD)...
July 2017: Inflammatory Bowel Diseases
https://www.readbyqxmd.com/read/28496240/beyond-onj-a-review-of-the-potential-uses-of-bisphosphonates-in-dentistry
#20
N P Shah, S Nayee, M Pazianas, C Sproat
There is evidence, although limited, for beneficial effects of bisphophonates (BPs) across multiple dental specialties. Within implant dentistry BP coatings have been shown to significantly increase pull out forces and bone density in animal models, and significantly increase implant stability whilst reducing marginal bone loss in humans. Adjunctive topical and systemic application of BPs during conventional periodontal treatment have shown significant improvements in probing depth and clinical attachment level in various forms of periodontal disease...
May 12, 2017: British Dental Journal
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