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Sclerosing cholangitis

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https://www.readbyqxmd.com/read/28926118/chop-induced-loss-of-intestinal-epithelial-stemness-contributes-to-bile-duct-ligation-induced-cholestatic-liver-injury
#1
Runping Liu, Xiaojiaoyang Li, Zhiming Huang, Derrick Zhao, Bhagyalaxmi Sukka Ganesh, Guanhua Lai, William M Pandak, Phillip B Hylemon, Jasmohan S Bajaj, Arun J Sanyal, Huiping Zhou
Impaired intestinal barrier function promotes the progression of various liver diseases including cholestatic liver disease. The close association of primary sclerosing cholangitis (PSC) with inflammatory bowel disease highlights the importance of the gut-liver axis. It has been reported that bile duct ligation (BDL)-induced liver fibrosis is significantly reduced in C/EBP homologous protein knock out (CHOP(-/-) ) mice. However, the underlying mechanisms remain unclear. In the current study, we demonstrate that BDL induces striking and acute hepatic ER stress responses after 1 day, which return to normal after 3 days...
September 19, 2017: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
https://www.readbyqxmd.com/read/28921801/complications-symptoms-quality-of-life-and-pregnancy-in-cholestatic-liver-disease
#2
Kais Zakharia, Anilga Tabibian, Keith D Lindor, James H Tabibian
Cholestatic liver diseases (CLDs) encompass a variety of disorders of bile formation and/or flow which generally result in progressive hepatobiliary injury and ultimately end-stage liver disease. Many patients with CLD are diagnosed between the ages of 20-50 years, a particularly productive period of life professionally, biologically, and in other respects; it is not surprising, thus, that CLD is often associated with impaired health-related quality of life (HRQOL) and uncertainty regarding implications for and outcomes of pregnancy...
September 18, 2017: Liver International: Official Journal of the International Association for the Study of the Liver
https://www.readbyqxmd.com/read/28919271/-autoimmune-hepatitis-immunological-diagnosis
#3
Imane Brahim, Ikram Brahim, Raja Hazime, Brahim Admou
Autoimmune hepatopathies (AIHT) including autoimmune hepatitis (AIH), primary biliary cirrhosis (PBC), primary sclerosing cholangitis (PSC) and autoimmune cholangitis (AIC), represent an impressive entities in clinical practice. Their pathogenesis is not perfectly elucidated. Several factors are involved in the initiation of hepatic autoimmune and inflammatory phenomena such as genetic predisposition, molecular mimicry and/or abnormalities of T-regulatory lymphocytes. AIHT have a wide spectrum of presentation, ranging from asymptomatic forms to severe acute liver failure...
September 14, 2017: La Presse Médicale
https://www.readbyqxmd.com/read/28913747/primary-sj%C3%A3-gren-s-syndrome-extraglandular-manifestations-and-hydroxychloroquine-therapy
#4
J Demarchi, S Papasidero, M A Medina, D Klajn, R Chaparro Del Moral, O Rillo, V Martiré, G Crespo, A Secco, A Catalan Pellet, C Amitrano, C Crow, C Asnal, P Pucci, F Caeiro, N Benzanquen, J P Pirola, M Mayer, F Zazzetti, S Velez, J Barreira, N Tamborenea, L Santiago, L Raiti
The use of hydroxychloroquine (HCQ) in Primary Sjögren's Syndrome (pSS) has been assessed in different studies over the last years, with conflicting results regarding its efficacy in sicca syndrome and extraglandular manifestations (EGM). The goal of this study was to compare the incidence rate of EGM in pSS patients with and without HCQ therapy.We performed a multicenter retrospective study, including patients with pSS (European classification criteria) with at least 1 year of follow-up. Subjects with concomitant fibromyalgia, autoimmune hepatitis, primary biliary cirrhosis, and primary sclerosing cholangitis were excluded...
September 14, 2017: Clinical Rheumatology
https://www.readbyqxmd.com/read/28913623/updated-strategies-for-the-management-pathogenesis-and-molecular-genetics-of-different-forms-of-ichthyosis-syndromes-with-prominent-hair-abnormalities
#5
REVIEW
Madiha Rasheed, Shaheen Shahzad, Afifa Zaeem, Imran Afzal, Asma Gul, Sumbal Khalid
Syndromic ichthyosis is rare inherited disorders of cornification with varied disease complications. This disorder appears in seventeen subtypes associated with severe systematic manifestations along with medical, cosmetic and social problems. Syndromic ichthyosis with prominent hair abnormalities covers five major subtypes: Netherton syndrome, trichothiodystrophy, ichthyosis hypotrichosis syndrome, ichthyosis hypotrichosis sclerosing cholangitis and ichthyosis follicularis atrichia photophobia syndrome. These syndromes mostly prevail in high consanguinity states, with distinctive clinical features...
September 14, 2017: Archives of Dermatological Research
https://www.readbyqxmd.com/read/28905689/igg4-related-cholangiopathy-and-its-mimickers-a-case-report-and-review-highlighting-the-importance-of-early-diagnosis
#6
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/28892096/development-and-characterization-of-cholangioids-from-normal-and-diseased-human-cholangiocytes-as-an-in-vitro-model-to-study-primary-sclerosing-cholangitis
#7
Lorena Loarca, Thiago M De Assuncao, Nidhi Jalan-Sakrikar, Steve Bronk, Anuradha Krishnan, Bing Huang, Leslie Morton, Christy Trussoni, Lorena Marcano Bonilla, Eugene Krueger, Steve O'Hara, Patrick Splinter, Guang Shi, María José Lorenzo Pisarello, Gregory J Gores, Robert C Huebert, Nicholas F LaRusso
Primary sclerosing cholangitis (PSC) is an incurable, fibroinflammatory biliary disease for which there is no effective pharmacotherapy. We recently reported cholangiocyte senescence as an important phenotype in PSC while others showed that portal macrophages accumulate in PSC. Unfortunately, our ability to explore cholangiocyte senescence and macrophage accumulation has been hampered by limited in vitro models. Thus, our aim was to develop and characterize a three-dimensional (3D) model of normal and diseased bile ducts (cholangioids) starting with normal human cholangiocytes (NHC), senescent NHC (NHC-sen), and cholangiocytes from PSC patients...
September 11, 2017: Laboratory Investigation; a Journal of Technical Methods and Pathology
https://www.readbyqxmd.com/read/28882625/drug-induced-bile-duct-injury
#8
REVIEW
Michele Visentin, Daniela Lenggenhager, Zhibo Gai, Gerd A Kullak-Ublick
Drug-induced liver injury includes a spectrum of pathologies, some related to the mode of injury, some to the cell type primarily damaged. Among these, drug-induced bile duct injury is characterized by the destruction of the biliary epithelium following exposure to a drug. Most of the drugs associated with bile duct injury cause immune-mediated lesions to the epithelium of interlobular ducts. These share common histopathological features with primary biliary cholangitis, such as inflammation and necrosis at the expense of cholangiocytes and, if the insult persists, bile duct loss and biliary cirrhosis...
September 4, 2017: Biochimica et Biophysica Acta
https://www.readbyqxmd.com/read/28870031/-the-clinical-characteristics-of-346-patients-with-igg4-related-disease
#9
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/28865156/gene-disease-associations-identify-a-connectome-with-shared-molecular-pathways-in-human-cholangiopathies
#10
Zhenhua Luo, Anil G Jegga, Jorge A Bezerra
Cholangiopathies are a diverse group of progressive diseases whose primary cell targets are cholangiocytes. To identify shared pathogenesis and molecular connectivity among the three main human cholangiopathies (biliary atresia [BA], primary biliary cholangitis [PBC] and primary sclerosing cholangitis [PSC]), we built a comprehensive platform of published data on gene variants, gene expression and functional studies, and applied network-based analytics in search for shared molecular circuits. Mining the data platform with largest connected component and interactome analyses, we validated previously reported associations and identified essential- and hub-genes...
September 2, 2017: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
https://www.readbyqxmd.com/read/28856287/acute-on-chronic-hepatitis-a-case-report-of-autoimmune-hepatitis-primary-sclerosing-cholangitis-ulcerative-colitis-overlap-syndrome-in-a-15-year-old-patient
#11
Aneta Nalepa, Małgorzata Woźniak, Joanna Cielecka-Kuszyk, Marek Stefanowicz, Irena Jankowska, Maciej Dądalski, Joanna Pawłowska
Acute-on-chronic liver failure (ACLF) is a disease in which a rapid deterioration of liver function occurs in patients with chronic liver disease, and is usually associated with a precipitating event. We present the case of a boy with autoimmune hepatitis/primary sclerosing cholangitis/ulcerative colitis (AIH/PSC/UC) overlap syndrome, in whom liver function was stable for 4.5 years of treatment. At 15 years of age the patient was hospitalized due to a deterioration of his general condition, severe abdominal pain, diarrhoea, vomiting and weight loss...
March 2017: Clin Exp Hepatol
https://www.readbyqxmd.com/read/28852270/the-role-of-proinflammatory-pathways-in-the-pathogenesis-of-colitis-associated-colorectal-cancer
#12
REVIEW
Chengxin Luo, Hu Zhang
Patients with inflammatory bowel disease (IBD) are at an increased risk of developing colorectal cancer (CRC). The risk factors of CRC in IBD patients include long disease duration, extensive colitis, severe histological inflammation, and coexistence with primary sclerosing cholangitis (PSC). Several molecular pathways that contribute to sporadic CRC are also involved in the pathogenesis of colitis-associated CRC. It is well established that long-standing chronic inflammation is a key predisposing factor of CRC in IBD...
2017: Mediators of Inflammation
https://www.readbyqxmd.com/read/28851259/does-transient-elastography-correlate-with-liver-fibrosis-in-patients-with-psc-laennec-score-based-analysis-of-explanted-livers
#13
Marcin Krawczyk, Joanna Ligocka, Mariusz Ligocki, Joanna Raszeja-Wyszomirska, Małgorzata Milkiewicz, Grzegorz Szparecki, Tomasz Ilczuk, Barbara Górnicka, Krzysztof Zieniewicz, Marek Krawczyk, Frank Lammert, Piotr Milkiewicz
BACKGROUND AND AIMS: Previous studies demonstrated a close correlation between transient elastography (TE) and liver histology in chronic liver diseases. Data on the accuracy of TE in primary sclerosing cholangitis (PSC) remains scarce. Here, we investigated the association between TE, serum marker of liver injury and histology of explanted livers in PSC patients. METHODS: Thirty patients were prospectively recruited. TE (Fibroscan(®)) and blood sampling were performed during evaluation for liver transplantation (LT); the second blood sampling was performed on the day of LT...
August 29, 2017: Scandinavian Journal of Gastroenterology
https://www.readbyqxmd.com/read/28844960/bile-acid-receptors-in-the-biliary-tree-tgr5-in-physiology-and-disease
#14
REVIEW
Kathleen Deutschmann, Maria Reich, Caroline Klindt, Carola Dröge, Lina Spomer, Dieter Häussinger, Verena Keitel
Bile salts represent signalling molecules with a variety of endocrine functions. Bile salt effects are mediated by different receptor molecules, comprising ligand-activated nuclear transcription factors as well as G protein-coupled membrane-bound receptors. The farnesoid X receptor (FXR) and the plasma membrane-bound G protein-coupled receptor TGR5 (Gpbar-1) are prototypic bile salt receptors of both classes and are highly expressed in the liver including the biliary tree as well as in the intestine. In liver, TGR5 is localized in different non-parenchymal cells such as sinusoidal endothelial cells, Kupffer cells, hepatic stellate cells and small and large cholangiocytes...
August 24, 2017: Biochimica et Biophysica Acta
https://www.readbyqxmd.com/read/28844954/optimising-the-clinical-strategy-for-autoimmune-liver-diseases-principles-of-value-based-medicine
#15
Marco Carbone, Laura Cristoferi, Paolo Angelo Cortesi, Matteo Rota, Antonio Ciaccio, Stefano Okolicsanyi, Marta Gemma, Luciana Scalone, Giancarlo Cesana, Luca Fabris, Michele Colledan, Stefano Fagiuoli, Gaetano Ideo, Luca Saverio Belli, Luca Maria Munari, Lorenzo Mantovani, Mario Strazzabosco
BACKGROUND: Autoimmune hepatitis, primary biliary cholangitis, and primary sclerosing cholangitis represent the three major autoimmune liver diseases (AILDs). Their management is highly specialized, requires a multidisciplinary approach and often relies on expensive, orphan drugs. Unfortunately, their treatment is often unsatisfactory, and the care pathway heterogeneous across different centers. Disease-specific clinical outcome indicators (COIs) able to evaluate the whole cycle of care are needed to assist both clinicians and administrators in improving quality and value of care...
August 24, 2017: Biochimica et Biophysica Acta
https://www.readbyqxmd.com/read/28844953/how-the-biliary-tree-maintains-immune-tolerance
#16
REVIEW
Haiyan Zhang, Patrick S C Leung, M Eric Gershwin, Xiong Ma
The liver is a vital organ with distinctive anatomy, histology and heterogeneous cell populations. These characteristics are of particular importance in maintaining immune homeostasis within the liver microenvironments, notably the biliary tree. Cholangiocytes are the first line of defense of the biliary tree against foreign substances, and are equipped to participate through various immunological pathways. Indeed, cholangiocytes protect against pathogens by TLRs-related signaling; maintain tolerance by expression of IRAK-M and PPARγ; limit immune response by inducing apoptosis of leukocytes; present antigen by expressing human leukocyte antigen molecules and costimulatory molecules; recruit leukocytes to the target site by expressing cytokines and chemokines...
August 24, 2017: Biochimica et Biophysica Acta
https://www.readbyqxmd.com/read/28844951/cholangiocytes-in-the-pathogenesis-of-primary-sclerosing-cholangitis-and-development-of-cholangiocarcinoma
#17
REVIEW
Brian K Chung, Tom H Karlsen, Trine Folseraas
Primary sclerosing cholangitis (PSC) is an idiopathic cholangiopathy strongly associated with inflammatory bowel disease (IBD) and characterized by cholestasis, chronic immune infiltration and progressive fibrosis of the intrahepatic and extrahepatic bile ducts. PSC confers a high risk of cholangiocarcinoma (CCA) with PSC-CCA representing the leading cause of PSC-associated mortality. PSC-CCA is derived from cholangiocytes and associated progenitor cells - a heterogeneous group of dynamic epithelial cells lining the biliary tree that modulate the composition and volume of bile production by the liver...
August 24, 2017: Biochimica et Biophysica Acta
https://www.readbyqxmd.com/read/28840045/hemoperitoneum-secondary-to-spontaneous-rupture-of-a-retroperitoneal-varix
#18
Derrick D Eichele
Hemoperitoneum due to a ruptured retroperitoneal varix is an exceedingly rare condition and a poor prognostic sign with catastrophic and life-threatening complication of portal hypertension. We present a unique case of a 56-year-old female with cirrhosis secondary to primary sclerosing cholangitis who presented with acute abdominal pain and hypovolemic shock prior to a cardiac arrest following a ruptured retroperitoneal varix without prior esophageal varices and a newly identified intrahepatic cholangiocarcinoma...
2017: Case Reports in Hepatology
https://www.readbyqxmd.com/read/28839868/evolving-strategies-to-reduce-colectomy-rates-in-primary-sclerosing-cholangitis-inflammatory-bowel-disease-clinical-remission-of-corticosteroid-refractory-colitis-post-liver-transplant-with-vedolizumab
#19
Saqib Mumtaz, Jason Goh, Gideon M Hirschfield, James Ferguson, Sheldon C Cooper
Primary sclerosing cholangitis (PSC) is associated with inflammatory bowel disease (IBD) in approximately 70% of patients, with ulcerative colitis (UC) being the most common subtype of IBD identified. There is a paucity of data on the optimum management strategy for IBD flares in the post-liver transplant patient, particularly when refractory to conventional treatments. Vedolizumab is a novel gut-specific monoclonal antibody, which has recently been approved for use by National Institute for Health and Care Excellence for moderate-to-severe UC...
October 2016: Frontline Gastroenterology
https://www.readbyqxmd.com/read/28839442/gut-barrier-failure-biomarkers-are-associated-with-poor-disease-outcome-in-patients-with-primary-sclerosing-cholangitis
#20
Tamas Tornai, Eszter Palyu, Zsuzsanna Vitalis, Istvan Tornai, David Tornai, Peter Antal-Szalmas, Gary L Norman, Zakera Shums, Gabor Veres, Antal Dezsofi, Gabriella Par, Alajos Par, Peter Orosz, Ferenc Szalay, Peter Laszlo Lakatos, Maria Papp
AIM: To assess the prevalence of a panel of serologic markers that reflect gut barrier dysfunction in a mixed cohort of pediatric and adult primary sclerosing cholangitis (PSC) patients. METHODS: Sera of 67 PSC patients [median age (range): 32 (5-79) years, concomitant IBD: 67% and cirrhosis: 20%] were assayed for the presence of antibodies against to F-actin (AAA IgA/IgG) and gliadin (AGA IgA/IgG)] and for serum level of intestinal fatty acid-binding protein (I-FABP) by ELISA...
August 7, 2017: World Journal of Gastroenterology: WJG
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