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https://www.readbyqxmd.com/read/28802875/primary-sclerosing-cholangitis-a-comprehensive-review
#1
REVIEW
Tom H Karlsen, Trine Folseraas, Douglas Thorburn, Mette Vesterhus
Primary sclerosing cholangitis (PSC) is a rare disorder characterized by multi-focal bile duct strictures and progressive liver disease. Inflammatory bowel disease (IBD) is usually present and there is a high risk of cholangiocarcinoma and colorectal cancer. With no effective medical therapy, most patients ultimately require liver transplantation, after which disease recurrence may occur. With limited therapeutic options and lack of proven surveillance strategies, patients experience significant unmet needs...
August 9, 2017: Journal of Hepatology
https://www.readbyqxmd.com/read/28791611/chronic-autoimmune-epithelitis-in-sj%C3%A3-gren-s-syndrome-and-primary-biliary-cholangitis-a-comprehensive-review
#2
REVIEW
Carlo Selmi, M Eric Gershwin
Within the spectrum of autoimmune diseases, Sjögren's syndrome and primary biliary cholangitis are exemplary and can be coined as chronic epithelitis based on their frequent coexistence in clinical practice and the highly specific immune-mediated injury of the small bile ducts and the exocrine glands. The pathogenic mechanisms underlying the diseases are similar, with apoptosis being the key element leading to organ-specific immune-mediated injury directed against the small bile ducts and salivary gland epithelia, respectively along with similar epidemiological features, such as female predominance and the age of onset in the fifth decade of life...
August 8, 2017: Rheumatology and Therapy
https://www.readbyqxmd.com/read/28774506/single-stage-operation-for-perforated-choledochal-cyst
#3
Go Ohba, Hiroshi Yamamoto, Masato Nakayama, Shohei Honda, Akinobu Taketomi
BACKGROUND: The option of either single- or two-staged cyst excision has been proposed for perforated choledochal cysts (CCs), but which of the two methods is more effective remains controversial. We examined the complications and short-term outcomes of single-stage excision of perforated and non-perforated CCs. METHODS: The medical records of patients treated for CCs from 2003 to 2016 were retrospectively reviewed. Outcomes were compared between patients with perforated CCs (Group A) and non-perforated CCs (Group B)...
July 21, 2017: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/28752474/secondary-sclerosing-cholangitis-a-review-of-recent-literature
#4
REVIEW
John Brooling, Rolando Leal
PURPOSE OF REVIEW: Secondary sclerosing cholangitis is an emerging entity. Yet, because of the low incidence and varying etiologies of this disease, the literature is not robust. There are mainly case reports and small studies evaluating the disease. Our aim in this review is to bring the reader up to date with recent literature on secondary sclerosing cholangitis including the disease presentation, course, prognosis, and treatment options. RECENT FINDINGS: Numerous case reports have been published the last 5 years on secondary sclerosing cholangitis...
September 2017: Current Gastroenterology Reports
https://www.readbyqxmd.com/read/28743097/less-is-more-an-outcome-assessment-of-patients-operated-for-gallstone-ileus-without-fistula-treatment
#5
Dario Tartaglia, Sohail Bakkar, Lorenzo Piccini, Jessica Bronzoni, Luigi Cobuccio, Andrea Bertolucci, Christian Galatioto, Massimo Chiarugi
BACKGROUND: The treatment of gallstone ileus (GI) consists of surgical removal of the impacted bilestone with or without cholecystectomy and repair of the biliodigestive fistula. The objective of this study was to assess whether sparing patients a definitive biliary procedure adversely influenced the outcome. MATERIALS AND METHODS: Patients with a diagnosis of GI were reviewed. Two groups were identified: patients who underwent a definitive biliary procedure with relieving the intestinal obstruction (group 1/G1) and those who did not have a definitive biliary procedure (group 2/G2)...
2017: International Journal of Surgery Case Reports
https://www.readbyqxmd.com/read/28742721/hepatic-portocholecystostomy-97-cases-from-a-single-institution
#6
Géraldine Héry, Emmanuel Gonzales, Olivier Bernard, Virginie Fouquet, Frédéric Gauthier, Sophie Branchereau
OBJECTIVES: About 20% of cases of biliary atresia (BA) do not involve the gallbladder, the cystic duct and the common bile duct. In these cases, a hepatoportocholecystostomy (HPC) may be performed instead of the classical hepatoportoenterostomy (HPE). METHODS: We reviewed our cohort to investigate the efficacy of HPC as well as the associated surgical complications and clinical problems. RESULTS: From 1984 to 2009, 97 patients underwent HPC in our institution...
July 22, 2017: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/28735981/autoimmune-liver-disease-in-children-with-sickle-cell-disease
#7
Suttiruk Jitraruch, Emer Fitzpatrick, Maesha Deheragoda, Annamaria Deganello, Giorgina Mieli-Vergani, Susan Height, David Rees, Nedim Hadzic, Marianne Samyn
OBJECTIVE: To assess the incidence, clinical features, and outcome of autoimmune liver disease (AILD) in patients with sickle cell disease (SCD). STUDY DESIGN: Single center retrospective review of patients with SCD with AILD referred between 1999 and 2015. RESULTS: Thirteen of 77 (17%) patients with SCD with hepatic dysfunction were diagnosed with AILD (median age 11, range, 3.4-16 years) with a female preponderance (77%). Acute hepatitis and insidious onset were the commonest presentations...
July 20, 2017: Journal of Pediatrics
https://www.readbyqxmd.com/read/28733732/predictive-risk-factors-associated-with-cholangitis-following-ercp
#8
Joshua Tierney, Neal Bhutiani, Bryce Stamp, John S Richey, Michael H Bahr, Gary C Vitale
BACKGROUND: Endoscopic retrograde cholangiopancreatography (ERCP) with stent placement is used for the management of many pancreaticobiliary disorders. It is generally safe with a few short-term complications. The risk factors for the development of post-ERCP cholangitis due to stent occlusion have not been previously described. This study identified such risk factors among patients undergoing ERCP and stent placement for pancreatic or biliary obstruction. METHODS: 3648 ERCPs performed at the University of Louisville from 2008 to 2016 were reviewed...
July 21, 2017: Surgical Endoscopy
https://www.readbyqxmd.com/read/28733178/clinical-usefulness-of-serum-antibodies-as-biomarkers-of-gastrointestinal-and-liver-diseases
#9
REVIEW
Antonio Di Sabatino, Federico Biagi, Marco Lenzi, Luca Frulloni, Marco Vincenzo Lenti, Paolo Giuffrida, Gino Roberto Corazza
The progressively growing knowledge of the pathophysiology of a number of immune-mediated gastrointestinal and liver disorders, including autoimmune atrophic gastritis, coeliac disease, autoimmune enteropathy, inflammatory bowel disease, autoimmune hepatitis, primary sclerosing cholangitis, primary biliary cholangitis and autoimmune pancreatitis, together with the improvement of their detection methods have increased the diagnostic power of serum antibodies. In some cases - coeliac disease and autoimmune atrophic gastritis - they have radically changed gastroenterologists' diagnostic ability, while in others - autoimmune hepatitis, inflammatory bowel disease and autoimmune pancreatitis - their diagnostic performance is still inadequate...
June 23, 2017: Digestive and Liver Disease
https://www.readbyqxmd.com/read/28731850/the-diagnostic-accuracy-of-biomarkers-for-diagnosis-of-primary-biliary-cholangitis-pbc-in-anti-mitochondrial-antibody-ama-negative-pbc-patients-a-review-of-literature
#10
Federica de Liso, Caterina Matinato, Mariangela Ronchi, Rita Maiavacca
Primary biliary cholangitis (PBC), also known as primary biliary cirrhosis, is an autoimmune disease of the liver characterized by anti-mitochondrial antibodies (AMA) in 90%-95% of patients. The aim of this study was to evaluate the diagnostic value of several serum biomarkers in patients with PBC but negative for AMA. Some antinuclear antibodies (ANA) pattern, detected by indirect immunofluorescence (IIF), such as multiple nuclear dot (MND) and rim-like patterns are well-known to be specific for PBC. The corresponding nuclear antigens are the components of the nuclear pore complex (Gp210 for rim-like pattern) and Sp100, PML proteins (for MND pattern) detectable by immunoblotting and ELISA methods...
July 21, 2017: Clinical Chemistry and Laboratory Medicine: CCLM
https://www.readbyqxmd.com/read/28712945/intestinal-and-extraintestinal-cancers-associated-with-inflammatory-bowel-disease
#11
REVIEW
Minna Chang, Liisa Chang, Hanna M Chang, Fuju Chang
Inflammatory bowel disease (IBD) with its 2 most common entities, ulcerative colitis and Crohn's disease, causes an increased risk of developing intestinal cancers. In fact, malignancies are the second most common cause of death after cardiovascular diseases in both sexes of patients with IBD. Risk factors for colorectal cancer in IBD correlate with the duration of the disease, extent of disease, the association with primary sclerosing cholangitis, family history, and early age at onset. Patients with IBD also have an increased risk for developing a variety of extraintestinal malignancies...
June 27, 2017: Clinical Colorectal Cancer
https://www.readbyqxmd.com/read/28711597/micrornas-and-extracellular-vesicles-in-cholangiopathies
#12
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/28706774/are-dominant-strictures-in-primary-sclerosing-cholangitis-a-risk-factor-for-cholangiocarcinoma
#13
REVIEW
Roger W Chapman, Kate D Williamson
PURPOSE OF REVIEW: Cholangiocarcinoma is a devastating, unpredictable complication of large duct primary sclerosing cholangitis (PSC), which occurs in 5-15% of patients. The aim of this review is to discuss whether dominant strictures (DS) occurring in the larger bile ducts in PSC are a risk factor for the development of cholangiocarcinoma. RECENT FINDINGS: The development of DS is related to specific genetic polymorphisms affecting the innate immune system and the microbiome...
2017: Current Hepatology Reports
https://www.readbyqxmd.com/read/28706773/why-doesn-t-primary-biliary-cholangitis-respond-to-immunosuppressive-medications
#14
REVIEW
Antonio Molinaro, Hanns-Ulrich Marschall
PURPOSE OF REVIEW: The purpose of this review is to discuss reasons why immunosuppressive therapy so far failed in Primary Biliry Cholangitis. RECENT FINDINGS: Even targeted immunosuppressive therapy seems ineffective or potentially harmful. SUMMARY: Bile acid-mediated cholangiocyte damage, facilitated by insufficient bicarbonate secretion, seems to attenuate the anti-inflammatory and anti-fibrotic actions of immunosuppressant and immunomodulatory drugs in a clinically significant way...
2017: Current Hepatology Reports
https://www.readbyqxmd.com/read/28698093/bile-acids-and-intestinal-microbiota-in-autoimmune-cholestatic-liver-diseases
#15
REVIEW
You Li, Ruqi Tang, Patrick S C Leung, M Eric Gershwin, Xiong Ma
Autoimmune cholestatic liver diseases, including primary biliary cholangitis (PBC) and primary sclerosing cholangitis (PSC), are manifested as an impairment of normal bile flow and excessive accumulation of potentially toxic bile acids. Endogenous bile acids are involved in the pathogenesis and progression of cholestasis. Consequently, chronic cholestasis affects the expression of bile acid transporters and nuclear receptors, and results in liver injury. Several lines of evidence suggest that intestinal microbiota plays an important role in the etiopathogenesis of cholestatic liver diseases by regulating metabolism and immune responses...
July 8, 2017: Autoimmunity Reviews
https://www.readbyqxmd.com/read/28684726/hepatic-artery-pseudoaneurysm-following-orthotopic-liver-transplantation-increasing-clinical-suspicion-for-a-rare-but-lethal-pathology
#16
Jon Harrison, Meredith Harrison, Cataldo Doria
BACKGROUND Despite an incidence of between 1% and 2%, the mortality rate in ruptured hepatic artery pseudoaneurysm after orthotopic liver transplantation approaches 69%. Our aim is to report operative and outcomes data for 7 patients with pseudoaneurysm following transplant at 1 institution, with emphasis on suspected risk factors for aneurysm formation. From these risk factors, we performed a systematic review to assess their clinical saliency. MATERIAL AND METHODS Using PRISMA guidelines, we completed a PubMed and online database review to gather studies addressing risk factors for pseudoaneurysm following transplant...
July 7, 2017: Annals of Transplantation: Quarterly of the Polish Transplantation Society
https://www.readbyqxmd.com/read/28674140/investigating-the-safety-and-activity-of-the-use-of-btt1023-timolumab-in-the-treatment-of-patients-with-primary-sclerosing-cholangitis-buteo-a-single-arm-two-stage-open-label-multi-centre-phase-ii-clinical-trial-protocol
#17
Katherine Arndtz, Margaret Corrigan, Anna Rowe, Amanda Kirkham, Darren Barton, Richard P Fox, Laura Llewellyn, Amrita Athwal, Manpreet Wilkhu, Yung-Yi Chen, Chris Weston, Amisha Desai, David H Adams, Gideon M Hirschfield
INTRODUCTION: Primary sclerosing cholangitis (PSC) is a progressive inflammatory liver disease characterised by relentless liver fibrosis and a high unmet need for new therapies. Preventing fibrosis represents an important area of interest in the development of vital new drugs. Vascular adhesion protein-1 (VAP-1) drives inflammation in liver disease, and provision of an antibody against VAP-1 blunts fibrosis in murine models of liver injury. METHODS AND ANALYSIS: BUTEO is a single-arm, two-stage, open-label, multi-centre, phase II clinical trial...
July 3, 2017: BMJ Open
https://www.readbyqxmd.com/read/28671339/role-of-erbb-her-family-of-receptor-tyrosine-kinases-in-cholangiocyte-biology
#18
REVIEW
Anna Pellat, Javier Vaquero, Laura Fouassier
The ErbB/HER family comprises four distinct tyrosine kinase receptors, EGFR/ErbB1/HER1, ErbB2/HER2, ErbB3/HER3 and ErbB4/HER4, which trigger intracellular signals at the origin of essential cellular functions including differentiation, proliferation, survival and migration. Epithelial cells, named cholangiocytes, that line intrahepatic and extrahepatic bile ducts, contribute substantially to biliary secretory functions and bile transport. Although ErbB receptors have been widely studied in cholangiocarcinoma (CCA), a malignancy of the biliary tract, knowledge of these receptors in biliary epithelium physiology and in non-malignant cholangiopathies is far from completed...
July 3, 2017: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
https://www.readbyqxmd.com/read/28667665/the-relationship-between-portopulmonary-hypertension-and-splenectomy-mayo-clinic-experience-and-literature-review
#19
Justin M Segraves, Rodrigo Cartin-Ceba, Michael D Leise, Michael J Krowka
BACKGROUND & AIMS: Portopulmonary hypertension is a serious complication of portal hypertension that can lead to right heart failure and death. To our knowledge, an association between portopulmonary hypertension and prior splenectomy has not been described previously. The goals of this study were to describe the frequency of splenectomy in portopulmonary hypertension and compare selected parameters between portopulmonary hypertension subgroups. METHODS: Retrospective analysis of patients diagnosed with portopulmonary hypertension between 01/01/1988 - 06/30/2015 at Mayo Clinic, Rochester, MN...
July 1, 2017: Hepatology Research: the Official Journal of the Japan Society of Hepatology
https://www.readbyqxmd.com/read/28657946/reconstruction-repair-of-iatrogenic-biliary-injuries-is-the-robot-offering-a-new-option-short-clinical-report
#20
Pier Cristoforo Giulianotti, Pablo Quadri, Samarth Durgam, Francesco Maria Bianco
OBJECTIVE: The aim of this study is to analyze perioperative outcomes of robotic reconstruction of iatrogenic biliary injuries and describe the surgical technique in detail. BACKGROUND: Iatrogenic bile duct injuries (BDIs) continue to be a major concern in open and laparoscopic cholecystectomy. In the past decade, robotic surgery has been applied to many different procedures showing technical advantages, especially in microsurgical fields. Few cases of robotic BDI reconstructions have been described in the literature so far...
June 27, 2017: Annals of Surgery
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