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Cholestatic liver diseases

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https://www.readbyqxmd.com/read/28427765/easl-clinical-practice-guidelines-the-diagnosis-and-management-of-patients-with-primary-biliary-cholangitis
#1
Gideon M Hirschfield, Ulrich Beuers, Christophe Corpechot, Pietro Invernizzi, David Jones, Marco Marzioni, Christoph Schramm
Primary biliary cholangitis (PBC) is a chronic inflammatory autoimmune cholestatic liver disease, which when untreated will culminate in end-stage biliary cirrhosis. Diagnosis is usually based on the presence of serum liver tests indicative of a cholestatic hepatitis in association with circulating antimitochondrial antibodies. Patient presentation and course can be diverse and risk stratification is important to ensure all patients receive a personalised approach to their care. The goals of treatment and management are the prevention of end-stage liver disease, and the amelioration of associated symptoms...
April 17, 2017: Journal of Hepatology
https://www.readbyqxmd.com/read/28425415/drug-induced-liver-injury-at-a-tertiary-hospital-in-india-etiology-clinical-features-and-predictors-of-mortality
#2
Chetan Rathi, Nirav Pipaliya, Ruchir Patel, Meghraj Ingle, Aniruddha Phadke, Prabha Sawant
INTRODUCTION AND AIMS: Drug-induced liver injury (DILI) is rare; however, it is one of the important causes of acute liver failure which results in significant morbidity or mortality. MATERIAL AND METHODS: Patients with suspected DILI were enrolled based on predefined criteria and followed up for at least 6 months or until normalization of liver tests. Causality assessment was done by applying the Roussel Uclaf Causality Assessment Method model. RESULTS: We collected data from 82 individuals diagnosed with DILI at our hospital from 2014 through 2015 (41 men; median age, 38 years)...
May 2017: Annals of Hepatology
https://www.readbyqxmd.com/read/28417463/pharmacological-interventions-for-primary-sclerosing-cholangitis-an-attempted-network-meta-analysis
#3
REVIEW
Francesca Saffioti, Kurinchi Selvan Gurusamy, Neil Hawkins, Clare D Toon, Emmanuel Tsochatzis, Brian R Davidson, Douglas Thorburn
BACKGROUND: Primary sclerosing cholangitis is a chronic cholestatic liver disease that is associated with both hepatobiliary and colorectal malignancies, which can result in liver cirrhosis and its complications. The optimal pharmacological treatment for patients with primary sclerosing cholangitis remains controversial. OBJECTIVES: To assess the comparative benefits and harms of different pharmacological interventions in people with primary sclerosing cholangitis by performing a network meta-analysis, and to generate rankings of available pharmacological interventions according to their safety and efficacy...
March 28, 2017: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/28405682/apamin-suppresses-biliary-fibrosis-and-activation-of-hepatic-stellate-cells
#4
Jung-Yeon Kim, Hyun-Jin An, Woon-Hae Kim, Yoon-Yub Park, Kyung Duck Park, Kwan-Kyu Park
Cholestatic liver disease is characterized by the progressive destruction of biliary epithelial cells (BECs) followed by fibrosis, cirrhosis and liver failure. Activated hepatic stellate cells (HSCs) and portal fibroblasts are the major cellular effectors of enhanced collagen deposition in biliary fibrosis. Apamin, an 18 amino acid peptide neurotoxin found in apitoxin (bee venom), is known to block Ca2+-activated K+ channels and prevent carbon tetrachloride-induced liver fibrosis. In the present study, we aimed to ascertain whether apamin inhibits biliary fibrosis and the proliferation of HSCs...
May 2017: International Journal of Molecular Medicine
https://www.readbyqxmd.com/read/28401057/intestinal-hypoganglionosis-leading-to-intestinal-failure-and-the-compassionate-use-of-omegaven%C3%A2
#5
Racha Khalaf, Sara Karjoo, Paul Danielson, Michael Wilsey, Fauzia Shakeel
Intestinal hypoganglionosis is a rare innervation disorder that provides numerous nutritional, medical and surgical challenges. In this case report, we present a case of a newborn with intestinal hypoganglionosis leading to intestinal failure and intestinal failure-associated liver disease who responded to Omegaven™, a fat emulsion comprised of omega-3 fatty acids. Omegaven™ has been shown to be beneficial in the management of cholestatic liver injury. Clinical success with Omegaven™ was seen in this patient with a clear decrease in aspartate aminotransferase, alanine aminotransferase, alkaline phosphatase and complete resolution of cholestasis with a direct bilirubin of zero within two weeks of initiation of Omegaven™...
March 2017: Pediatric Gastroenterology, Hepatology & Nutrition
https://www.readbyqxmd.com/read/28400821/the-great-impostor-transaminitis-masking-the-coinfection-of-syphilis-and-human-immunodeficiency-virus
#6
Sunit Tolia, Hassan Kassem, Ana Capatina-Rata
Introduction. The incidence of syphilis continues to rise in the United States over the past 15 years. This disease process is classified into stages and may present with a coinfection of Human Immunodeficiency Virus (HIV). Case Report. We present a case of a 32-year-old African American male who presented with cutaneous manifestations of secondary syphilis and transaminitis. A workup revealed that the transaminitis was secondary to underlying syphilitic hepatitis in the presence of HIV coinfection. The patient had a reactive rapid plasma reagin (RPR) of 1 : 64 TU and reactive Treponema pallidum particle agglutination assay (TPPA)...
2017: Case Reports in Medicine
https://www.readbyqxmd.com/read/28397388/risk-of-thyroid-cancer-among-solid-organ-transplant-recipients
#7
Cari M Kitahara, Elizabeth L Yanik, Paul W Ladenson, Brenda Y Hernandez, Charles F Lynch, Karen S Pawlish, Eric A Engels
Solid organ transplant recipients have an elevated incidence of thyroid cancer. We evaluated a wide range of potential risk factors in a cohort of 229,300 U.S. solid organ transplant recipients linked with 15 stage/regional cancer registries (1987-2012). Incidence rate ratios (IRRs) were adjusted for age, sex, race/ethnicity, transplanted organ, year of transplant, and time since transplant. Hazards ratios (HRs) for death and/or graft failure were adjusted for age, sex, race/ethnicity, transplanted organ, and year of transplant...
April 11, 2017: American Journal of Transplantation
https://www.readbyqxmd.com/read/28391876/lipoprotein-x-in-cholestatic-patients-causes-xanthomas-and-promotes-foam-cell-formation-in-human-macrophages
#8
Luka Suzuki, Satoshi Hirayama, Mariko Fukui, Makoto Sasaki, Sadayuki Hiroi, Makoto Ayaori, Shuji Terai, Minoru Tozuka, Hirotaka Watada, Takashi Miida
BACKGROUND: Lipoprotein-X (Lp-X) is an abnormal phospholipid-rich lipoprotein found in patients with cholestatic liver disease. Some patients exhibit skin xanthomas and severe hyperlipidemia. OBJECTIVE: We investigated whether Lp-X induces foam cell formation in human-derived macrophages. METHODS: To compare the atherogenic properties of Lp-X and modified LDL, we isolated Lp-X from 2 patients who had drug-induced cholestasis and xanthoma striata in the interphalangeal folds...
January 2017: Journal of Clinical Lipidology
https://www.readbyqxmd.com/read/28388864/clinical-and-histologic-features-of-azathioprine-induced-hepatotoxicity
#9
Sith Siramolpiwat, Dussadee Sakonlaya
BACKGROUND: Hepatoxicity is a relative uncommon complication related with Azathioprine, however most studies were performed in inflammatory bowel diseases patients. The aim of this study is to report the clinical profile of patients with Azathioprine-induced hepatotoxicity. METHODS: All medical records of patients received Azathioprine from 2010 to 2015 were retrospectively reviewed. Hepatotoxicity was defined as serum alanine aminotransferase (ALT) or aspatate aminotransferase (AST) or total bilirubin >2 times upper limit normal...
April 7, 2017: Scandinavian Journal of Gastroenterology
https://www.readbyqxmd.com/read/28388653/expression-and-localization-of-sterile-alpha-motif-domain-containing-5-is-associated-with-cell-type-and-malignancy-of-biliary-tree
#10
Tomoki Yagai, Satoshi Matsui, Kenichi Harada, Fuyuki F Inagaki, Eiko Saijou, Yasushi Miura, Yasuni Nakanuma, Atsushi Miyajima, Minoru Tanaka
Cholangiocarcinoma (CC) is a type of relatively rare neoplasm in adenocarcinoma. The characteristics of CCs as well as biliary epithelial cells are heterogeneous at the different portion of the biliary tree. There are two candidate stem/progenitor cells of the biliary tree, i.e., biliary tree stem/progenitor cell (BTSC) at the peribiliary gland (PBG) of large bile ducts and liver stem/progenitor cell (LPC) at the canals of Hering of peripheral small bile duct. Although previous reports suggest that intrahepatic CC (ICC) can arise from such stem/progenitor cells, the characteristic difference between BTSC and LPC in pathological process needs further investigation, and the etiology of CC remains poorly understood...
2017: PloS One
https://www.readbyqxmd.com/read/28385542/model-systems-for-studying-the-role-of-canalicular-efflux-transporters-in-drug-induced-cholestatic-liver-disease
#11
REVIEW
Bruno Stieger, Zainab M Mahdi
Bile formation is a key function of the liver. Disturbance of bile flow may lead to liver disease and is called cholestasis. Cholestasis may be inherited such as for example in progressive familial intrahepatic cholestasis or acquired, such as for example by drug-mediated inhibition of bile salt export from hepatocytes into the canaliculi. The key transport system for exporting bile salts into the canaliculi is the bile salt export pump. Inhibition of the bile salt export pump by drugs is a well-established cause of drug-induced cholestasis...
April 3, 2017: Journal of Pharmaceutical Sciences
https://www.readbyqxmd.com/read/28379588/a-focus-on-epidemiology-of-drug-induced-liver-injury-analysis-of-a-prospective-cohort
#12
A Licata, M G Minissale, V Calvaruso, A Craxì
OBJECTIVE: Drug-induced liver injury (DILI) is more often a challenge even for expert clinicians. Presently, there are limited data about the epidemiology, because the real incidence and prevalence of the disorder are underestimated, and further, sometimes the pharmacovigilance chain is unsuccessful as cases are largely underreported. We review available literature data and discuss our clinical experience regarding a prospective cohort of 185 patients with a diagnosis of DILI. MATERIALS AND METHODS: Significant papers were identified by literature search, and selected based on content including the epidemiology of DILI...
March 2017: European Review for Medical and Pharmacological Sciences
https://www.readbyqxmd.com/read/28376623/the-clinical-extremes-of-autoimmune-cholangitis
#13
Sara Campos, Dário Gomes, Maria Augusta Cipriano, Carlos Sofia
Autoimmune cholangitis (AIC) was first described in 1987 as immunocholangitis in three women who presented with signs and symptoms of primary biliary cholangitis (PBC), but who were antimitochondrial (AMA) negative and antinuclear antibodies (ANA) positive, and responded to immunosuppressive therapy with azathioprine and prednisolone (1). AIC is a rare chronic cholestatic inflammatory disease characterized by the presence of high ANA or smooth muscle antibodies (SMA) but AMA seronegativity. Histologically, AIC exhibits bile duct injury (2)...
April 3, 2017: Revista Española de Enfermedades Digestivas
https://www.readbyqxmd.com/read/28376583/inflammatory-bowel-disease-and-primary-sclerosing-cholangitis-a-review-of-the-phenotype-and-associated-specific-features
#14
REVIEW
Carolina Palmela, Farhad Peerani, Daniel Castaneda, Joana Torres, Steven H Itzkowitz
Primary sclerosing cholangitis (PSC) is a chronic, progressive cholestatic disease that is associated with inflammatory bowel disease (IBD) in approximately 70% of cases. Although the pathogenesis is still unknown for both diseases, there is increasing evidence to indicate that they share a common underlying predisposition. Herein, we review the epidemiology, diagnosis, disease pathogenesis, and specific clinical features of the PSC-IBD phenotype. Patients with PSC-IBD have a distinct IBD phenotype with an increased incidence of pancolitis, backwash ileitis, and rectal sparing...
April 6, 2017: Gut and Liver
https://www.readbyqxmd.com/read/28371374/vitamin-a-deficiency-in-chronic-cholestatic-liver-disease-is-vitamin-a-therapy-beneficial
#15
Cora Freund, Daniel Nils Gotthardt
Chronic cholestatic diseases are progressive diseases of the biliary tract that cause hepatic fibrosis and ultimately lead to liver failure. Liver transplantation is the sole curative option currently available, and because of high morbidity and mortality rates of these diseases, new therapeutic approaches are needed. Vitamin A is a nutrient essential for health as it regulates many processes, including epithelial growth and immunological processes. Vitamin A is primarily stored in hepatic stellate cells, and during liver injury, through an unknown mechanism, these cells lose vitamin A and convert into collagen-producing myofibroblasts, which contributes to hepatic fibrosis...
March 29, 2017: Liver International: Official Journal of the International Association for the Study of the Liver
https://www.readbyqxmd.com/read/28371104/old-and-new-treatments-for-primary-biliary-cholangitis
#16
David Chascsa, Elizabeth J Carey, Keith D Lindor
Primary biliary cholangitis (formerly primary biliary cirrhosis) is a rare progressive cholestatic liver disease, whose hallmark features include a persistently elevated alkaline phosphatase level, presence of anti-mitochondrial antibodies and characteristic histology. Since 1998, ursodeoxycholic acid (UDCA), a bile acid, has been the only available therapeutic agent. Primary biliary cholangitis is associated with the development of end-stage liver disease, increased morbidity and mortality. UDCA has been shown to improve serum biochemistries, histology and delay the need for liver transplantation...
April 2017: Liver International: Official Journal of the International Association for the Study of the Liver
https://www.readbyqxmd.com/read/28364821/cholestatic-liver-diseases-after-liver-transplant
#17
REVIEW
Nathalie A Pena Polanco, Cynthia Levy, Eric F Martin
Primary sclerosing cholangitis (PSC) and primary biliary cholangitis (PBC) are the most common cholestatic liver diseases (CLD) in adults. Liver transplant (LT) is desirable for those who progress to end-stage liver disease. CLD have become an uncommon indication for LT. PSC and PBC accounted for 7.1% of all adult LT in 2015. CLD have the best post-LT outcomes compared with other indications for LT. Disease recurrence of PSC and PBC after LT is reported in up to 37% and 43% of LT recipients, respectively. Although recurrent PBC does not affect post-LT outcomes, recurrent PSC is associated with worse post-LT survival...
May 2017: Clinics in Liver Disease
https://www.readbyqxmd.com/read/28348902/atypical-complications-of-graves-disease-a-case-report-and-literature-review
#18
Khaled Ahmed Baagar, Mashhood Ahmed Siddique, Shaimaa Ahmed Arroub, Ahmed Hamdi Ebrahim, Amin Ahmed Jayyousi
Graves' disease (GD) may display uncommon manifestations. We report a patient with rare complications of GD and present a comprehensive literature review. A 35-year-old woman presented with a two-week history of dyspnea, palpitations, and edema. She had a raised jugular venous pressure, goiter, and exophthalmos. Laboratory tests showed pancytopenia, a raised alkaline phosphatase level, hyperbilirubinemia (mainly direct bilirubin), and hyperthyroidism [TSH: <0.01 mIU/L (reference values: 0.45-4.5), fT4: 54...
2017: Case Reports in Endocrinology
https://www.readbyqxmd.com/read/28338458/hepatitis-b-in-solid-organ-transplant-procedures-other-than-liver
#19
Dina Halegoua-De Marzio, Jonathan M Fenkel, Cataldo Doria
Transplant is often the best treatment available for patients with end-stage organ failure. Hepatitis B virus infection in transplant procedures other than liver is a major concern because it can be a significant cause of morbidity and mortality after transplant. Due to the increased risk of hepatic complications, such as fibrosing cholestatic hepatitis or histologic deterioration after transplant, systematic use of nucleoside or nucleotide analogues shortly before or at the time of transplant is recommended (tenofovir or entecavir are preferable to lamivudine) in all patients, whatever the baseline histologic evaluation...
April 2017: Experimental and Clinical Transplantation
https://www.readbyqxmd.com/read/28330614/a-predictive-3d-multi-scale-model-of-biliary-fluid-dynamics-in-the-liver-lobule
#20
Kirstin Meyer, Oleksandr Ostrenko, Georgios Bourantas, Hernan Morales-Navarrete, Natalie Porat-Shliom, Fabian Segovia-Miranda, Hidenori Nonaka, Ali Ghaemi, Jean-Marc Verbavatz, Lutz Brusch, Ivo Sbalzarini, Yannis Kalaidzidis, Roberto Weigert, Marino Zerial
Bile, the central metabolic product of the liver, is transported by the bile canaliculi network. The impairment of bile flow in cholestatic liver diseases has urged a demand for insights into its regulation. Here, we developed a predictive 3D multi-scale model that simulates fluid dynamic properties successively from the subcellular to the tissue level. The model integrates the structure of the bile canalicular network in the mouse liver lobule, as determined by high-resolution confocal and serial block-face scanning electron microscopy, with measurements of bile transport by intravital microscopy...
March 15, 2017: Cell Systems
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