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Frank Lammert, Nahum Méndez-Sánchez
No abstract text is available yet for this article.
November 2016: Annals of Hepatology
Shumyla Jabeen, Irfan Robbani, Naseer A Choh, Obaid Ashraf, Feroze Shaheen, Tariq Gojwari, Sabeeha Gul
OBJECTIVE: To characterize biliary abnormalities seen in portal cavernoma cholangiopathy (PCC) on Magnetic Resonance Cholangiopancreaticography(MRCP) and elucidate certain salient features of the disease by collaborating our findings with those of previous studies. METHODS: We prospectively enrolled 52 patients with portal cavernoma secondary to idiopathic Extrahepatic portal vein obstruction(EHPVO) who underwent a standard MRCP protocol. Images were analysed for abnormalities involving the entire biliary tree...
October 12, 2016: British Journal of Radiology
Kazuo Imai, Kazuhisa Misawa, Takahiro Matsumura, Yuji Fujikura, Kei Mikita, Masaharu Tokoro, Takuya Maeda, Akihiko Kawana
We herein describe a case of progressive human immunodeficiency virus (HIV)-associated cholangiopathy despite normalization of laboratory parameters, which had indicated liver dysfunction, after the initiation of combined anti-retroviral therapy (cART). HIV-associated cholangiopathy remains important as a differential diagnosis of bile duct disorders, although it is considered to be a rare disease in the era of cART. Magnetic resonance cholangiopancreatography could thus be a powerful tool for the diagnosis and follow-up of this disease...
2016: Internal Medicine
Sophie Restellini, Olivier Chazouillères, Jean-Louis Frossard
Inflammatory bowel diseases (IBD) are associated with various hepatobiliary disorders, reported both in Crohn's disease (CD) and ulcerative colitis (UC). They may occur at any moment in the natural course of the disease. The prevalence of liver dysfunction rises from 3% to 50% accordingly to definitions. Fatty liver is considered as the most common hepatobiliary complication in IBD whilst primary sclerosing cholangitis (PSC) the most specific one. Less frequently, IBD-associated hepatobiliary disorders include: autoimmune hepatitis/PSC overlap syndrome, IgG4-associated cholangiopathy, primary biliary cholangitis (PBC), hepatic amyloidosis, granulomatous hepatitis, cholelithiasis, portal vein thrombosis, and liver abscess...
October 6, 2016: Liver International: Official Journal of the International Association for the Study of the Liver
Adriana Cavași, Voicu Mercea, Ofelia Anton, Ion Cosmin Puia
Although transjugular intrahepatic portosystemic shunts are most frequently used for the management of portal hypertension, the surgical approach is preferred for symptomatic portal cavernoma cholangiopathy. We present the case of a 25-year old female patient with a portal cavernoma secondary to catheterization of the umbilical vein at birth. She had had two episodes of esophageal variceal bleeding, successfully treated by endoscopic banding. and an episode of acute cholangitis secondary to portal cavernoma cholangiopathy...
September 2016: Journal of Gastrointestinal and Liver Diseases: JGLD
Hyoung-Chul Oh, Fatih Akisik, Stuart Sherman
No abstract text is available yet for this article.
September 14, 2016: Clinical Gastroenterology and Hepatology
Romina Fiorotto, Ambra Villani, Antonis Kourtidis, Roberto Scirpo, Mariangela Amenduni, Peter J Geibel, Massimilano Cadamuro, Carlo Spirli, Panos Z Anastasiadis, Mario Strazzabosco
: In the liver, CFTR regulates bile secretion and other functions at the apical membrane of biliary epithelial cells (i.e cholangiocytes). CF-related liver disease (CFLD) is a major cause of death in patients with CF. CFTR dysfunction affects innate immune pathways, generating a para-inflammatory status in the liver, and other epithelia. This study investigates the mechanisms linking CFTR to TLR4 activity. We found that CFTR is associated in a multi-protein complex at the apical membrane of normal mouse cholangiocytes, with proteins that negatively control Src activity...
September 15, 2016: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
Petra Hirsova, Samar H Ibrahim, Vikas K Verma, Leslie A Morton, Vijay H Shah, Nicholas F LaRusso, Gregory J Gores, Harmeet Malhi
Extracellular vesicles (EVs) are nanometer-sized, membrane-bound vesicles released by cells into the extracellular milieu. EVs are now recognized to play a critical role in cell-to-cell communication. EVs contain important cargo in the form of proteins, lipids and nucleic acids and serve as vectors for delivering this cargo from donor to acceptor or target cell. The EVs are released both under physiologic and pathologic conditions, including liver diseases, and exert a wide range of effects on target cells...
September 15, 2016: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
Motoko Sasaki, Yasuni Nakanuma
Various cellular responses including apoptosis, necrosis, autophagy and cellular senescence are involved in the pathogenesis of inflammatory cholangiopathies, such as primary biliary cholangitis (PBC), primary sclerosing cholangitis (PSC) and biliary atresia (BA). For example, dysregulated autophagy may play a role in abnormal expression of mitochondrial antigens and following autoimmune pathogenesis in bile duct lesions in PBC. Recently, new types of regulated cell death including necroptosis, parthanatos, pyroptosis, immunogenic cell death are the subject of numerous reports and they may play roles in pathogenesis of liver diseases, such as nonalcoholic steatohepatitis...
September 8, 2016: Clinics and Research in Hepatology and Gastroenterology
Jan Best, Stefaan Verhulst, Wing-Kin Syn, Kimberly Lagaisse, Noemi van Hul, Femke Heindryckx, Jan-Peter Sowa, Liesbeth Peeters, Hans Van Vlierberghe, Isabelle A Leclercq, Ali Canbay, Laurent Dollé, Leo A van Grunsven
Chronic cholangiopathies, such as primary and secondary sclerosing cholangitis, are progressive disease entities, associated with periportal accumulation of inflammatory cells, encompassing monocytes and macrophages, peribiliary extracellular matrix (ECM) deposition and ductular reaction (DR). This study aimed to elucidate the relevance of macrophages in the progression of chronic cholangiopathies through macrophage depletion in a 3,5-diethoxycarbonyl-1,4-dihydrocollidine (DDC) mouse model. One group of mice received a single i...
2016: PloS One
İsmail Şerifoğlu, İbrahim İlker Öz, Yücel Üstündağ, Sevil Uygun İlikhan, Özlem Tokgöz
BACKGROUND: Immunoglobulin G4 (IgG4)-associated diseases mostly involve the pancreaticobiliary tree and pancreatic parenchyma. This disease complex is characterized by marked response to corticosteroid therapy and response to steroids is incorporated in the diagnostic algorithm of IgG4 associated diseases. However, there is much unknown about the sequences and duration of healing during the corticosteroid therapy in the literature. CASE REPORT: In this case report, we report a young male patient with IgG4 associated extrahepatic biliary stricture and autoimmune pancreatitis successfully treated with corticosteroids...
July 2016: Balkan Medical Journal
Kyo-Sang Yoo, Woo Taek Lim, Ho Soon Choi
Cholangiocytes, the lining epithelial cells in bile ducts, are an important subset of liver cells. They are activated by endogenous and exogenous stimuli and are involved in the modification of bile volume and composition. They are also involved in damaging and repairing the liver. Cholangiocytes have many functions including bile production. They are also involved in transport processes that regulate the volume and composition of bile. Cholangiocytes undergo proliferation and cell death under a variety of conditions...
September 15, 2016: Gut and Liver
Vivian Tang, Zenobia C Cofer, Shuang Cui, Valerie Sapp, Kathleen M Loomes, Randolph P Matthews
OBJECTIVES: Biliary atresia (BA) is a progressive fibro-inflammatory cholangiopathy affecting the bile ducts of neonates. Although BA is the leading indication for pediatric liver transplantation, the etiology remains elusive. Adducin 3 (ADD3) and X-prolyl aminopeptidase 1 (XPNPEP1) are two genes previously identified in genome-wide association studies (GWAS) as potential BA susceptibility genes. Using zebrafish, we investigated the importance of ADD3 and XPNPEP1 in functional studies...
August 12, 2016: Journal of Pediatric Gastroenterology and Nutrition
Mark E Sharafinski, Arthur B Meyers, Gregory Vorona
Pediatric gallbladder and bile duct disease encompasses a broad spectrum of processes, from congenital to developmental to neoplastic. We describe normal pediatric biliary anatomy and summarize the most common pathologic entities, with a focus on non-invasive imaging techniques and findings. Ultrasound is the first-line imaging modality in children with suspected biliary pathology based on its widespread availability, cost effectiveness, and lack of ionizing radiation. MRI and MRCP are often used for further evaluation in cases of diagnostic uncertainty and for surgical planning...
August 12, 2016: Abdominal Radiology
Tassos Grammatikopoulos, Melissa Sambrotta, Sandra Strautnieks, Pierre Foskett, A S Knisely, Bart Wagner, Maesha Deheragoda, Chris Starling, Giorgina Mieli-Vergani, Joshua Smith, University Of Washington Center For Mendelian Genomics, Laura Bull, Richard J Thompson
BACKGROUND & AIMS: Neonatal sclerosing cholangitis (NSC) is a severe neonatal-onset cholangiopathy commonly leading to liver transplantation (LT) for end-stage liver disease in childhood. Liver-biopsy findings histopathologically resemble those in biliary atresia (BA); however, in NSC extrahepatic bile ducts are patent, whilst in BA their lumina are obliterated. NSC is commonly seen in consanguineous kindreds, suggesting autosomal recessive inheritance. METHODS: From 29 NSC patients (24 families) identified, DNA was available in 24 (21 families)...
July 25, 2016: Journal of Hepatology
Saikiran Raghavapuram, Mohit Girotra, Jeffrey D Robertson, Tarun Pandey, Benjamin Tharian
No abstract text is available yet for this article.
July 20, 2016: Gastrointestinal Endoscopy
Chad Hall, Keisaku Sato, Nan Wu, Tianhao Zhou, Tina Kyritsi, Fanyin Meng, Shannon Glaser, Gianfranco Alpini
BACKGROUND: Cholangiocytes, a small population of cells within the normal liver, have been the focus of a significant amount of research over the past two decades because of their involvement in cholangiopathies such as primary sclerosing cholangitis and primary biliary cholangitis. METHODS: This manuscript summarizes landmark studies in the field of cholangiocyte physiology and aims to provide an updated review of biliary pathogenesis. RESULTS: The historical approach of rodent extrahepatic bile duct ligation and the relatively recent utilization of transgenic mice have led to significant discoveries in cholangiocyte pathophysiology...
July 12, 2016: Gene Expression
Sudheer S Pargewar, Saloni N Desai, S Rajesh, Vaibhav P Singh, Ankur Arora, Amar Mukund
Extrahepatic portal vein obstruction (EHPVO) is a primary vascular condition characterized by chronic long standing blockage and cavernous transformation of portal vein with or without additional involvement of intrahepatic branches, splenic or superior mesenteric vein. Patients generally present in childhood with multiple episodes of variceal bleed and EHPVO is the predominant cause of paediatric portal hypertension (PHT) in developing countries. It is a pre-hepatic type of PHT in which liver functions and morphology are preserved till late...
June 28, 2016: World Journal of Radiology
Bhanumathi Lakshminarayanan, Mark Davenport
Biliary atresia presents as an obliterative cholangiopathy with neonatal jaundice and pale stools. The disease exhibits aetiological heterogeneity with a multiplicity of potential causative factors, both developmental and environmental. A number of clinical variants making up a minority of all cases can be defined relatively precisely which match suggested aetiology better although in most it still remains speculative. These include the syndromic form (BASM), the cystic form and those associated with CMV IgM antibodies...
September 2016: Journal of Autoimmunity
Safi Dokmak, Béatrice Aussilhou, Alain Sauvanet, Philippe Lévy, Aurélie Plessier, Fadhel S Ftériche, Olivier Farges, Valérie Vilgrain, Dominique C Valla, Jacques Belghiti
BACKGROUND: Supra-mesocolic surgery (SMS) is complicated in patients with portal vein cavernoma (PC) and portal decompression is recommended. The aim of this study was to report a large single centre of SMS in patients with PC without portal decompression. METHODS: Between 2006 and 2013, all patients who met inclusion criteria were analyzed retrospectively. The primary endpoint was the feasibility rate, surgical and postoperative outcome. The secondary endpoints were the long-term outcome of patients who underwent biliary bypass for cholangitis...
July 2016: HPB: the Official Journal of the International Hepato Pancreato Biliary Association
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