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https://www.readbyqxmd.com/read/28716013/long-term-renal-survival-of-%C3%AE-3-heavy-chain-deposition-disease-a-case-report
#1
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
#2
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
#3
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
#4
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
#5
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...
July 4, 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
#6
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 a global rise in incidence and high mortality. 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 preoperative stratification strategies. Osteopontin is a secreted extracellular glyco-phosphoprotein that has been associated with inflammation, metabolic disorders and cancer...
June 28, 2017: Journal of Hepatology
https://www.readbyqxmd.com/read/28638005/sclerosing-mesenteritis-causing-chylous-ascites-and-small-bowel-perforation
#7
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
#8
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
#9
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
#10
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
#11
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. Non-invasive differential diagnosis between intrahepatic CCA (iCCA) and hepatocellular carcinoma (HCC) is sometimes difficult. Accurate non-invasive biomarkers for PSC, CCA or HCC are not available. In the search of 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
#12
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
#13
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
#14
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
#15
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
https://www.readbyqxmd.com/read/28479483/coffee-and-autoimmunity-more-than-a-mere-hot-beverage
#16
REVIEW
Kassem Sharif, Abdulla Watad, Nicola Luigi Bragazzi, Mohammad Adawi, Howard Amital, Yehuda Shoenfeld
Coffee is one of the world's most consumed beverage. In the last decades, coffee consumption has attracted a huge body of research due to its impact on health. Recent scientific evidences showed that coffee intake could be associated with decreased mortality from cardiovascular and neurological diseases, diabetes type II, as well as from endometrial and liver cancer, among others. In this review, on the basis of available data in the literature, we aimed to investigate the association between coffee intake and its influence on the immune system and the insurgence of the most relevant autoimmune diseases...
May 4, 2017: Autoimmunity Reviews
https://www.readbyqxmd.com/read/28457384/liver-retransplantation-for-hepatic-abscess-due-to-hepatic-artery-thrombosis-a-case-report
#17
G Zanus, M Romano, M Finotti, E Dalla Bona, D Sgarabotto, D Bassi, C Mescoli, P Angeli, P Burra, E Gringeri, A Vitale, F D'Amico, P Feltracco, U Cillo
INTRODUCTION: Hepatic artery thrombosis (HAT) is a well-recognized complication of liver transplantation (LT). HAT is an important risk factor for infectious, in particular hepatic abscess, which can cause graft loss and increasing morbidity and mortality. CASE REPORT: We present a case report of complicated LT in a 52-year-old Caucasian man with primary sclerosing cholangitis. In 2007 the patient was included on the waiting list in Padua for LT. In 2012 the patient underwent percutaneous transhepatic biliary drainage for bile duct stricture, complicated with acute pancreatitis...
May 2017: Transplantation Proceedings
https://www.readbyqxmd.com/read/28426778/apai-polymorphism-of-vitamin-d-receptor-affects-health-related-quality-of-life-in-patients-with-primary-sclerosing-cholangitis
#18
Agnieszka Kempinska-Podhorodecka, Malgorzata Milkiewicz, Dariusz Jabłonski, Piotr Milkiewicz, Ewa Wunsch
BACKGROUND: Polymorphisms of vitamin D receptor (VDR) contribute to the pathogenesis of multiple autoimmune conditions. METHODS: We investigated the incidence of VDR polymorphisms (rs1544410-BsmI; rs7975232-ApaI; rs731236-TaqI) in a group of patients with primary sclerosing cholangitis (PSC, n = 275) and in healthy controls (n = 376). Additionally, correlations of the VDR polymorphisms with clinical and biochemical factors of the disease were analysed. RESULTS: The genotype and allele distributions of these polymorphisms in PSC patients were similar to those observed in controls...
2017: PloS One
https://www.readbyqxmd.com/read/28426471/advances-in-the-management-of-malignant-pleural-effusion
#19
Labib G Debiane, David E Ost
PURPOSE OF REVIEW: The current review describes the latest evidence in the management of malignant pleural effusions (MPE). RECENT FINDINGS: Daily drainage of indwelling pleural catheters achieved auto-pleurodesis at a higher rate compared with every-other-day drainage [0.47 vs. 0.24; difference in proportion of 0.23; 95% confidence interval (CI) 0.08-0.38; P = 0.003]. In patients with MPE undergoing talc pleurodesis, a large multicenter randomized clinical trial found that pain control with opiates vs...
July 2017: Current Opinion in Pulmonary Medicine
https://www.readbyqxmd.com/read/28407375/h19-promotes-cholestatic-liver-fibrosis-by-preventing-zeb1-mediated-inhibition-of-epcam
#20
Yongfeng Song, Chune Liu, Xia Liu, Jocelyn Trottier, Michele Beaudoin, Li Zhang, Chad Pope, Guangyong Peng, Olivier Barbier, Xiaobo Zhong, Linheng Li, Li Wang
Based on our recent finding that disruption of bile acid (BA) homeostasis in mice results in the induction of hepatic lncRNA H19 expression, we sought to elucidate the role of H19 in cholestatic liver fibrosis. Hepatic overexpression of H19RNA augmented bile duct ligation (BDL)-induced liver fibrosis, which was accompanied by the elevation of serum ALT, AST, bilirubin, and BA levels. Multiple genes related to liver fibrosis, inflammation, and biliary hyperplasia were increased in H19-BDL vs Null-BDL mice, whereas genes in BA synthesis were decreased...
April 13, 2017: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
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