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Cholestatic liver diseases

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https://www.readbyqxmd.com/read/27921168/the-2016-diagnostic-criteria-for-primary-sclerosing-cholangitis
#1
Takahiro Nakazawa, Kenji Notohara, Susumu Tazuma, Atsushi Tanaka, Hiroyuki Isayama, Toshio Tsuyuguchi, Toshiyuki Mori, Hajime Takikawa
BACKGROUND: Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease of unknown cause that is characterized pathologically by an inflammatory and fibrotic process centered on the epithelium, leading to diffuse biliary stenosis and increased wall thickness throughout the intra- and extra-hepatic biliary trees. A nationwide survey in Japan revealed several differences in the clinical aspects of PSC between Japan and Western countries. PSC was recently designated an intractable hepatobiliary disease in Japan...
December 5, 2016: Journal of Gastroenterology
https://www.readbyqxmd.com/read/27906097/a-10-year-old-child-presenting-with-syndromic-paucity-of-bile-ducts-alagille-syndrome-a-case-report
#2
Girish Kumar Pati, Ayaskanta Singh, Preetam Nath, Jimmy Narayan, Pradeep Kumar Padhi, Prasanta Kumar Parida, Kaumudee Pattnaik, Chittaranjan Panda, Shivaram Prasad Singh
BACKGROUND: Alagille syndrome, a rare genetic disorder with autosomal dominant transmission, manifests with five major features: paucity of interlobular bile ducts, characteristic facies, posterior embryotoxon, vertebral defects, and peripheral pulmonary stenosis. Globally, only 500 cases have so far been reported, with only five cases reported in the Indian subcontinent. Rarely, Alagille syndrome also presents with skin manifestations and early-onset chronic liver disease, which was found in our case...
November 30, 2016: Journal of Medical Case Reports
https://www.readbyqxmd.com/read/27898169/cross-sectional-analysis-of-progressive-familial-intrahepatic-cholestasis-in-puerto-rican-children
#3
Dellys M Soler, Antonio I Del Valle, David Fernandez-Lube, Benjamin L Shneider
OBJECTIVE: Specific inherited disorders may be more common in island communities. Prior case reports suggest that cholestatic liver diseases may constitute a group of these inherited disorders in Puerto Rico. A cross-sectional survey of liver diseases in children was conducted to assess this hypothesis. METHODS: A cross-sectional analysis was performed in patients with chronic cholestasis at "Hospital Pediátrico Universitario" in San Juan, Puerto Rico. Ten potential participants with high gamma-glutamyl transpeptidase (GGTP) cholestasis were identified...
December 2016: Puerto Rico Health Sciences Journal
https://www.readbyqxmd.com/read/27895309/hepatocyte-specific-expression-of-an-oncogenic-variant-of-%C3%AE-catenin-results-in-cholestatic-liver-disease
#4
Ursula J Lemberger, Claudia Fuchs, Matthias Karer, Stefanie Haas, Tatjana Stojakovic, Christian Schöfer, Hanns-Ulrich Marschall, Fritz Wrba, Makoto M Taketo, Gerda Egger, Michael Trauner, Christoph H Österreicher
BACKGROUND: The Wnt/β-catenin signaling pathway plays a crucial role in embryonic development, tissue homeostasis, wound healing and malignant transformation in different organs including the liver. The consequences of continuous β-catenin signaling in hepatocytes remain elusive. RESULTS: Livers of Ctnnb1CA hep mice were characterized by disturbed liver architecture, proliferating cholangiocytes and biliary type of fibrosis. Serum ALT and bile acid levels were significantly increased in Ctnnb1CA hep mice...
November 23, 2016: Oncotarget
https://www.readbyqxmd.com/read/27889359/elevated-liver-enzymes-emergency-department-focused-management
#5
Eric Sulava, Samuel Bergin, Brit Long, Alex Koyfman
BACKGROUND: Liver function test (LFT) abnormalities are a common problem faced by emergency physicians. This has become more common with the introduction of laboratory panels and automated routine laboratory testing. Fortunately, not all patients with irregularities in liver enzymes possess underlying pathology. This emergency medicine focused review provides a discussion of the various biochemical tests, their underlying biological basis, and an algorithmic approach to the interpretation of abnormalities...
November 23, 2016: Journal of Emergency Medicine
https://www.readbyqxmd.com/read/27882046/the-role-of-genetic-and-immune-factors-for-the-pathogenesis-of-primary-sclerosing-cholangitis-in-childhood
#6
REVIEW
Priscila Menezes Ferri, Ana Cristina Simões E Silva, Soraya Luiza Campos Silva, Diego Junior Queiroga de Aquino, Eleonora Druve Tavares Fagundes, Débora Marques de Miranda, Alexandre Rodrigues Ferreira
Primary sclerosing cholangitis (PSC) is a rare cholestatic liver disease characterized by chronic inflammation of the biliary tree resulting in liver fibrosis. PSC is more common in male less than 40 years of age. The diagnosis of PSC is based on clinical, laboratory, image, and histological findings. A biochemical profile of mild to severe chronic cholestasis can be observed. Endoscopic retrograde cholangiography is the golden standard method for diagnosis, but magnetic resonance cholangiography is currently also considered a first-line method of investigation...
2016: Gastroenterology Research and Practice
https://www.readbyqxmd.com/read/27874998/factors-predictive-of-the-efficacy-of-bezafibrate-therapy-in-patients-with-primary-sclerosing-cholangitis
#7
Suguru Mizuno, Hiroyuki Isayama, Kenji Hirano, Takeo Watanabe, Naminatsu Takahara, Hirofumi Kogure, Saburo Matsubara, Yousuke Nakai, Minoru Tada, Kazuhiko Koike
AIM: Primary sclerosing cholangitis (PSC) is a rare cholestatic disease. We previously reported the effects of bezafibrate on elevated hepatobiliary enzyme levels in patients with this disease both retrospectively and prospectively. In this study, we assessed factors predictive of bezafibrate efficacy. METHODS: Twenty-five patients with PSC, who underwent bezafibrate therapy (400 mg per day) from November 2006 to June 2015, were evaluated. Treatment was judged as being effective if the levels of all of the hepatobiliary enzymes decreased after 12 weeks...
November 22, 2016: Hepatology Research: the Official Journal of the Japan Society of Hepatology
https://www.readbyqxmd.com/read/27855457/-cholestatic-liver-disease
#8
Christine Pichler, Tobias Boettler, Robert Thimme
Common cholestatic liver diseases are primary biliary cholangitis (PBC) and primary sclerosing cholangitis (PSC). Both diseases are considered autoimmune disorders; however, the precise pathogenesis remains elusive. Patients usually show no symptoms or present with pruritus and fatigue. High alkaline phosphatase and the presence of antimitochondrial antibodies are sufficient to diagnose PBC. Ursodeoxycholic acid is approved and recommended for the treatment of PBC. The diagnosis of PSC is established by elevated alkaline phosphatase-levels and typical cholangiographic findings...
November 2016: Deutsche Medizinische Wochenschrift
https://www.readbyqxmd.com/read/27852811/adrenal-function-in-cats-with-cholestatic-liver-disease
#9
Faith I Buckley, Orla Mahony, Cynthia R L Webster
Cats with cholestatic liver disease experience significant morbidity and mortality when they undergo invasive procedures under anesthesia. Although inadequate adrenal response might account for these outcomes, adrenal function in cats with cholestatic liver disease has not been documented, to our knowledge. The goal of our study was to describe adrenal function in these cats. Twenty-seven cats with a serum bilirubin >230 µmol/L (3 mg/dL) and serum alanine aminotransferase >2 times the upper limit of normal had pre- and 60-min post-adrenocorticotropic hormone (ACTH) cortisol analysis after administration of 5 µg/kg cosyntropin intravenously...
November 15, 2016: Journal of Veterinary Diagnostic Investigation
https://www.readbyqxmd.com/read/27834858/impact-of-microbes-on-the-pathogenesis-of-primary-biliary-cirrhosis-pbc-and-primary-sclerosing-cholangitis-psc
#10
REVIEW
Jochen Mattner
Primary biliary cirrhosis (PBC) and primary sclerosing cholangitis (PSC) represent the major clinical entities of chronic cholestatic liver diseases. Both disorders are characterized by portal inflammation and slowly progress to obliterative fibrosis and eventually liver cirrhosis. Although immune-pathogenic mechanisms have been implicated in the pathogenesis of PBC and PSC, neither disorder is considered to be a classical autoimmune disease, as PSC and PBC patients do not respond to immune-suppressants. Furthermore, the decreased bile flow resulting from the immune-mediated tissue assault and the subsequent accumulation of toxic bile products in PBC and PSC not only perpetuates biliary epithelial damage, but also alters the composition of the intestinal and biliary microbiota and its mutual interactions with the host...
November 9, 2016: International Journal of Molecular Sciences
https://www.readbyqxmd.com/read/27832649/bile-acids-and-the-potential-role-in-primary-biliary-cirrhosis
#11
Hang Yang, Zhijun Duan
BACKGROUND: Bile acids (BAs) play a potential role in regulating the whole-body metabolic homeostasis via the interaction with gut microbiome and the signal transduction as messengers, which establish a link between the primary biliary cirrhosis (PBC) and gut microbiome in many aspects, particularly with regard to the immune system of the body. PBC, as a chronic cholestatic liver disease characterised by the destruction of small intrahepatic bile ducts, causes fibrosis and potential cirrhosis without efficient therapies...
November 11, 2016: Digestion
https://www.readbyqxmd.com/read/27826954/critical-and-diverse-in-vivo-roles-of-apoptosis-signal-regulating-kinase-1-in-animal-models-of-atherosclerosis-and-cholestatic-liver-injury
#12
REVIEW
Sohsuke Yamada, Hirotsugu Noguchi, Akihide Tanimoto
Apoptosis plays pivotal in vivo roles in not only vital processes, such as cell turnover and embryonic development, but also various inflammatory disorders. However, the role of apoptosis by vascular and hepatic cells in the respective progression of atherosclerosis and liver injury remains controversial. Apoptosis signal-regulating kinase 1 (ASK1) is a mitogen-activated protein kinase kinase kinase family member that is activated through distinct mechanisms in response to various cytotoxic stressors. ASK1, ubiquitously expressed, is situated in an important upstream position for many signal transduction pathways, which subsequently induce inflammation and/or apoptosis...
November 9, 2016: Histology and Histopathology
https://www.readbyqxmd.com/read/27824421/-elevated-liver-enzymes
#13
Axel Holstege
Elevated liver enzymes are a frequent finding in both symptomatic and asymptomatic patients necessitating further evaluation to clarify the underlying disease. Three different patterns of increased liver enzymes can be defined to allow for a more precise and rational further diagnostic approach. A predominant increase in transaminase activities reflects a disturbance of hepatocellular integrity which can be found in patients with viral hepatitis, genetic liver diseases like Wilson`s disease or hemochromatosis, and drug-induced liver diseases...
October 2016: Deutsche Medizinische Wochenschrift
https://www.readbyqxmd.com/read/27824131/infliximab-and-dexamethasone-attenuate-the-ductular-reaction-in-mice
#14
Stefaan Verhulst, Jan Best, Wing-Kin Syn, Hendrik Reynaert, Karine H Hellemans, Ali Canbay, Laurent Dolle, Leo A van Grunsven
Chronic hepatic injury is accompanied by a ductular response that is strongly correlated with disease severity and progression of fibrosis. To investigate whether anti-inflammatory drugs can modulate the ductular response, we treated mice suffering from a steatotic or cholestatic injury with anti-TNF-α antibodies (Infliximab) or glucocorticoids (Dexamethasone). We discovered that Dexamethasone and Infliximab can both modulate the adaptive remodeling of the biliary architecture that occurs upon liver injury and limit extracellular matrix deposition...
November 8, 2016: Scientific Reports
https://www.readbyqxmd.com/read/27806633/surveillance-of-primary-sclerosing-cholangitis-with-erc-and-brush-cytology-risk-factors-for-cholangiocarcinoma
#15
Sonja Boyd, Harri Mustonen, Andrea Tenca, Kalle Jokelainen, Johanna Arola, Martti A Färkkilä
OBJECTIVE: Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease leading to bile duct strictures and fibrosis, and predisposing to cholangiocarcinoma (CCA). Biliary dysplasia is a known precursor of CCA. In our unit, PSC patients undergo regular surveillance with ERC and brush cytology (BC), and liver transplantation is an option in case with biliary dysplasia. We evaluated the risk factors for biliary dysplasia and CCA based on ERC imaging, BC and liver function tests...
November 3, 2016: Scandinavian Journal of Gastroenterology
https://www.readbyqxmd.com/read/27802897/evidence-for-the-involvement-of-nitric-oxide-in-cholestasis-induced-itch-associated-response-in-mice
#16
Sattar Ostadhadi, Arash Foroutan, Majid Momeny, Abbas Norouzi-Javidan, Ehsan Azimi, Nastaran Kordjazy, Ahmad Reza Dehpour
Cholestasis is a major systemic disorder associated with distressing pruritus (itch). Nitric oxide (NO) is a neurotransmitter, assumed to be involved in pruritus. Based on over-production of NO in cholestatic liver diseases, this project aimed to investigate involvement of NO in cholestasis-related itch in mice. To achieve this, cholestasis was induced by bile duct ligation (BDL). Our results showed that BDL mice elicited significant itch on fifth and seventh day after the procedure. This scratching behavior was inhibited by intraperitoneal (IP) treatment of mice with non-selective NOS inhibitor N-nitro-l-arginine methyl ester (l-NAME; 3mg/kg) and inducible NOS (iNOS) inhibitor aminoguanidine (AG; 100mg/kg)...
October 29, 2016: Biomedicine & Pharmacotherapy, Biomédecine & Pharmacothérapie
https://www.readbyqxmd.com/read/27788700/-progress-in-diagnosis-and-therapy-of-extrahepatic-manifestations-of-cholestatic-liver-diseases
#17
L G Lu
Extra-hepatic complications of cholestasis mainly include pruritus, fatigue and metabolic bone diseases, and their molecular mechanisms have not yet been fully identified so far. Though therapeutic regimens targeted at the pathogenesis alleviate the symptoms temporarily, the therapeutic effects are limited, and therefore expounding the pathogenesis will help find more appropriate therapeutic approaches. Pruritus may be caused by nerve conduction block resulting from accumulation of some substances in the accumulated bile, existing recommended methods like drug therapy with cholestyramine and blood purification can markedly alleviate cholestasis-induced pruritus...
August 20, 2016: Zhonghua Gan Zang Bing za Zhi, Zhonghua Ganzangbing Zazhi, Chinese Journal of Hepatology
https://www.readbyqxmd.com/read/27785310/hepatitis-c-in-liver-allograft-recipients-utility-of-one-year-post-transplantation-biopsy-as-an-indicator-of-antiviral-therapy
#18
Shahid Habib, Shahid Malik, Bo Fu, Joyce Chang, Michael Nalesnik, Abhinav Humar, Obaid S Shaikh
BACKGROUND: All-oral interferon-free regimens for hepatitis C viral (HCV) infection are highly efficacious; however, high cost is a barrier to applicability. Liver allograft recipients are particularly likely to benefit from therapy as HCV often leads to graft dysfunction and loss. In this study, we aimed to establish the utility of allograft biopsy at 1 year post-transplant as an indicator of treatment. METHODS AND RESULTS: Among 252 liver recipients enrolled, 136 (54%) developed severe disease (fibrosing cholestatic hepatitis (FCH) or fibrosis stage ≥ 2 at 1 year post-transplant)...
December 2015: Gastroenterology Research
https://www.readbyqxmd.com/read/27784437/-environmental-factors-and-primary-biliary-cirrhosis
#19
L P Chen, H Zhao, B Lyu, J L Cheng
The complex interplay between immune factors and genetic susceptibility plays an essential role in autoimmune diseases. This is especially true for primary biliary cirrhosis (PBC). PBC is an autoimmune cholestatic liver disease characterized by the destruction of the small intrahepatic bile ducts and the presence of high-titer antimitochondrial antibodies (AMA). Among the currently known risk factors, genetic predisposition remains to be the dominant one. However, it is insufficient to explain the different geographic distributions of PBC and the incomplete concordance in identical twins...
July 20, 2016: Zhonghua Gan Zang Bing za Zhi, Zhonghua Ganzangbing Zazhi, Chinese Journal of Hepatology
https://www.readbyqxmd.com/read/27779646/heparin-binding-epidermal-growth-factor-like-growth-factor-and-hepatocyte-growth-factor-inhibit-cholestatic-liver-injury-in-mice-through-different-mechanisms
#20
Kouichi Sakamoto, Ngin Cin Khai, Yuqing Wang, Rie Irie, Hideo Takamatsu, Hiroshi Matsufuji, Ken-Ichiro Kosai
In contrast to hepatocyte growth factor (HGF), the therapeutic potential and pathophysiologic roles of heparin-binding epidermal growth factor-like growth factor (HB-EGF) in liver diseases remain relatively unknown. To address the lack of effective pharmacologic treatments for cholestatic liver injuries, as well as to clarify the biologic features of these growth factors, we explored the effects of HB-EGF and HGF in mice with cholestatic liver injury induced by bile duct ligation (BDL). The mice were assessed 3, 5 and/or 14 days after BDL (acute, subacute and/or chronic phases, respectively) and intravenous injection of adenoviral vector expressing LacZ (control), HB-EGF, HGF, or HB-EGF and HGF...
October 20, 2016: International Journal of Molecular Medicine
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