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Cholestatic liver diseases

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https://www.readbyqxmd.com/read/29790196/rituximab-is-ineffective-for-treatment-of-fatigue-in-primary-biliary-cholangitis-a-phase-2-randomised-controlled-trial
#1
Amardeep Khanna, Laura Jopson, Denise Howel, Andrew Bryant, Andrew Blamire, Julia L Newton, David E Jones
Primary Biliary Cholangitis (PBC) is a chronic cholestatic liver disease. Half of patients experience debilitating fatigue which is currently untreatable. Previous studies have shown muscle bioenergetic abnormalities in PBC, including increased muscle acidosis with exercise linked to the anti-mitochondrial antibody (AMA) diagnostic of the disease, and reduced anaerobic threshold. In this study we addressed the hypothesis that fatigue in PBC is driven by muscle bioenergetic abnormality related to AMA, and that AMA reduction with B-cell depletion therapy will improve fatigue...
May 23, 2018: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
https://www.readbyqxmd.com/read/29787781/regulation-of-bile-secretion-by-calcium-signaling-in-health-and-disease
#2
REVIEW
David C Trampert, Michael H Nathanson
Calcium (Ca2+ ) signaling controls secretion in many types of cells and tissues. In the liver, Ca2+ regulates secretion in both hepatocytes, which are responsible for primary formation of bile, and cholangiocytes, which line the biliary tree and further condition the bile before it is secreted. Cholestatic liver diseases, which are characterized by impaired bile secretion, may result from impaired Ca2+ signaling mechanisms in either hepatocytes or cholangiocytes. This review will discuss the Ca2+ signaling machinery and mechanisms responsible for regulation of secretion in both hepatocytes and cholangiocytes, and the pathophysiological changes in Ca2+ signaling that can occur in each of these cell types to result in cholestasis...
May 19, 2018: Biochimica et Biophysica Acta
https://www.readbyqxmd.com/read/29761165/a-real-world-observational-cohort-of-patients-with-primary-biliary-cholangitis-target-primary-biliary-cholangitis-study-design-and-rationale
#3
Cynthia Levy, Christopher L Bowlus, Elizabeth Carey, Julie M Crawford, Karen Deane, Marlyn J Mayo, W Ray Kim, Michael W Fried
Primary biliary cholangitis (PBC) is a rare chronic cholestatic liver disease that may progress to biliary cirrhosis if left untreated. The first-line therapy for PBC is ursodeoxycholic acid (UDCA). Unfortunately, 1 of 3 patients does not respond to UDCA. These patients are at risk for developing clinical events, including cirrhosis, complications of portal hypertension, hepatocellular carcinoma, liver transplant, or death. Recently, the U.S. Food and Drug Administration approved obeticholic acid to be used in certain patients with PBC...
May 2018: Hepatology Communications
https://www.readbyqxmd.com/read/29758112/novel-and-emerging-therapies-for-cholestatic-liver-diseases
#4
Jordan Goldstein, Cynthia Levy
While bile acids are important for both digestion and signaling, hydrophobic bile acids can be harmful especially when in high concentrations. Mechanisms for protection of cholangiocytes against bile acid cytotoxicity include negative feedback loops via farnesoid X nuclear receptor (FXR) activation, the bicarbonate umbrella, cholehepatic shunting and anti-inflammatory signaling, among others. By altering or overwhelming these defense mechanisms, cholestatic diseases such as primary biliary cholangitis (PBC) and primary sclerosing cholangitis (PSC) can further progress to biliary cirrhosis, end-stage liver disease and death or liver transplantation...
May 14, 2018: Liver International: Official Journal of the International Association for the Study of the Liver
https://www.readbyqxmd.com/read/29751877/pathologic-features-of-hereditary-cholestatic-diseases
#5
REVIEW
Andrew D Clouston
The inherited diseases causing conjugated hyperbilirubinemia are diverse, with variability in clinical severity, histologic appearance, and time of onset. The liver biopsy appearances can also vary depending on whether the initial presentation is in the neonatal period or later. Although many of the disorders have specific histologic features in fully developed and classic cases, biopsies taken early in the disease course may be nonspecific, showing either cholestatic hepatitis or an obstructive pattern of injury requiring close correlation with the laboratory and clinical findings to reach the correct diagnosis...
June 2018: Surgical Pathology Clinics
https://www.readbyqxmd.com/read/29751359/bone-morphogenetic-protein-9-as-a-key-regulator-of-liver-progenitor-cells-in-ddc-induced-cholestatic-liver-injury
#6
Annalisa Addante, Cesáreo Roncero, Laura Almalé, Nerea Lazcanoiturburu, María García-Álvaro, Margarita Fernández, Julián Sanz, Seddik Hammad, Zeribe Chike Nwosu, Se-Jin Lee, Isabel Fabregat, Steven Dooley, Peter Ten Dijke, Blanca Herrera, Aránzazu Sánchez
BACKGROUND & AIMS: Bone morphogenetic protein 9 (BMP9) interferes with liver regeneration upon acute injury, while promoting fibrosis upon carbon tetrachloride-induced chronic injury. We have now addressed the role of BMP9 in 3,5 diethoxicarbonyl-1,4 dihydrocollidine (DDC)-induced cholestatic liver injury, a model of liver regeneration mediated by hepatic progenitor cell (known as oval cell), exemplified as ductular reaction and oval cell expansion. METHODS: WT and BMP9KO mice were submitted to DDC diet...
May 11, 2018: Liver International: Official Journal of the International Association for the Study of the Liver
https://www.readbyqxmd.com/read/29738332/anesthetic-care-of-2-siblings-with-neonatal-ichthyosis-and-sclerosing-cholangitis-syndrome-case-reports
#7
Céline Khalifa, Geoffrey Boliaki Botombe, Xavier Stephenne, Francis Veyckemans
We report for the first time the anesthetic management of 2 sisters suffering from neonatal ichthyosis and sclerosing cholangitis syndrome. They both presented with neonatal cholestatic jaundice and ichthyosis. The first was admitted for orthotopic liver transplantation at the age of 1 year, and the second patient underwent open pyeloplasty for a pyeloureteric junction syndrome at the age of 4 years. These 2 case reports highlight that, except for the potential difficulties with securing the catheters, dressings and endotracheal tube to the skin, the anesthetic implications of neonatal ichthyosis and sclerosing cholangitis syndrome are mainly related to the liver disease: cirrhosis and portal hypertension...
May 7, 2018: A&A practice
https://www.readbyqxmd.com/read/29736833/are-clinicians-ready-for-safe-use-of-stratified-therapy-in-primary-biliary-cholangitis-pbc-a-study-of-educational-awareness
#8
Laura Jopson, Amardeep Khanna, Patricia Peterson, Elaine Rudell, Margaret Corrigan, David Jones
BACKGROUND: Primary Biliary Cholangitis (PBC, formerly cirrhosis), is a chronic cholestatic liver disease which until spring 2016 had a single licensed therapy, Ursodeoxycholic acid (UDCA). Approximately 30% of patients do not respond to UDCA, and are high-risk for progressing to end stage liver disease, transplantation or death. A new era of stratified medicine with second-line therapies to treat high-risk disease is emerging, with the first such second-line agent obeticholic acid recently receiving FDA and EMA approval and entering practice...
May 8, 2018: Digestive Diseases and Sciences
https://www.readbyqxmd.com/read/29720662/de-novo-formation-of-the-biliary-system-by-tgf%C3%AE-mediated-hepatocyte-transdifferentiation
#9
Johanna R Schaub, Kari A Huppert, Simone N T Kurial, Bernadette Y Hsu, Ashley E Cast, Bryan Donnelly, Rebekah A Karns, Feng Chen, Milad Rezvani, Hubert Y Luu, Aras N Mattis, Anne-Laure Rougemont, Philip Rosenthal, Stacey S Huppert, Holger Willenbring
Transdifferentiation is a complete and stable change in cell identity that serves as an alternative to stem-cell-mediated organ regeneration. In adult mammals, findings of transdifferentiation have been limited to the replenishment of cells lost from preexisting structures, in the presence of a fully developed scaffold and niche 1 . Here we show that transdifferentiation of hepatocytes in the mouse liver can build a structure that failed to form in development-the biliary system in a mouse model that mimics the hepatic phenotype of human Alagille syndrome (ALGS) 2 ...
May 2, 2018: Nature
https://www.readbyqxmd.com/read/29709217/glycogenic-hepatopathy
#10
Johad Khoury, Yaniv Zohar, Naim Shehadeh, Tarek Saadi
BACKGROUND: Glycogenic hepatopathy (GH) is a disorder associated with uncontrolled diabetes mellitus, most commonly type 1, expressed as right upper quadrant abdominal pain, hepatomegaly and increased liver enzymes. The diagnosis may be difficult, because laboratory and imaging tests are not pathognomonic. Although GH may be suggested based on clinical presentation and imaging studies, the gold standard for diagnosis is a liver biopsy, showing a significant accumulation of glycogen within the hepatocytes...
April 2018: Hepatobiliary & Pancreatic Diseases International: HBPD INT
https://www.readbyqxmd.com/read/29704003/pilot-study-with-ibat-inhibitor-a4250-for-the-treatment-of-cholestatic-pruritus-in-primary-biliary-cholangitis
#11
Samer Al-Dury, Annika Wahlström, Staffan Wahlin, Jacqueline Langedijk, Ronald Oude Elferink, Marcus Ståhlman, Hanns-Ulrich Marschall
Pruritus is a common complication of cholestatic liver diseases. Inhibition of the ileal bile acid transporter (IBAT/ASBT) may emerge as treatment option. Our aim was to assess tolerability and effect on pruritus of the selective IBAT inhibitor A4250 in patients with primary biliary cholangitis (PBC). Ten patients with PBC and bile acid sequestrant treatment of cholestatic pruritus were after a two-week wash out of the bile acid sequestrant treated with either 0.75 mg (n = 4) or 1.5 mg (n = 5) of A4250 for four weeks...
April 27, 2018: Scientific Reports
https://www.readbyqxmd.com/read/29702116/a-rare-but-recognizable-cholestatic-liver-disease
#12
Iranzu Ezcurra, José Ignacio Fortea, Javier Crespo
No abstract text is available yet for this article.
April 24, 2018: Gastroenterology
https://www.readbyqxmd.com/read/29701277/review-article-therapeutic-bile-acids-and-the-risks-for-hepatotoxicity
#13
REVIEW
K Ashby, E E Navarro Almario, W Tong, J Borlak, R Mehta, M Chen
BACKGROUND: Bile acids play important roles in cholesterol metabolism and signal through farnesoid X receptor and G protein-coupled receptors. Given their importance in liver biology, bile acid therapy enables therapeutic applications beyond the treatment of cholestatic liver disease. However, predicting hepatotoxicity of bile acids in humans is obscured due to inconsistent extrapolations of animal data to humans. AIM: To review the evidence that could explain discordant bile acids hepatotoxicity observed in humans and animals...
April 27, 2018: Alimentary Pharmacology & Therapeutics
https://www.readbyqxmd.com/read/29700559/-primary-sclerosing-cholangitis-current-diagnostics-and-treatment
#14
REVIEW
T Liwinski, C Schramm
Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease of unknown etiology. Characteristic features are multifocal strictures and dilatations of the bile ducts. In 60-80 % of cases the PSC is strongly associated with chronic inflammatory bowel disease, mostly in the form of pancolitis. The diagnosis is established based on detection of typical cholangiographic lesions of the bile ducts and exclusion of secondary causes of sclerosing cholangitis. There is no approved medical treatment, but in Germany ursodeoxycholic acid is frequently used...
April 26, 2018: Der Internist
https://www.readbyqxmd.com/read/29693762/growth-body-composition-and-bone-density-following-pediatric-liver-transplantation
#15
Amin Sheikh, Tim Cundy, Helen Maria Evans
Patients transplanted for cholestatic liver disease are often significantly fat-soluble vitamin deficient and malnourished pretransplant, with significant corticosteroid exposure post-transplant, with increasing evidence of obesity and metabolic syndrome post-LT. Our study aimed to assess growth, body composition, and BMD in patients post-pediatric LT. Body composition and bone densitometry scans were performed on 21 patients. Pre- and post-transplant anthropometric data were analyzed. Bone health was assessed using serum ALP, calcium, phosphate, and procollagen-1-N-peptide levels...
April 24, 2018: Pediatric Transplantation
https://www.readbyqxmd.com/read/29685617/validation-of-transient-elastography-cut-points-to-assess-advanced-liver-fibrosis-in-children-and-young-adults-the-boston-children-s-hospital-experience
#16
Christine K Lee, Paul D Mitchell, Roshan Raza, Sarah Harney, Shanna M Wiggins, Maureen M Jonas
OBJECTIVE: To derive an optimal liver stiffness measurement cut point to discriminate METAVIR fibrosis stage F4 and to validate both METAVIR fibrosis stage F3-F4 and F4 cut points in a separate cohort. STUDY DESIGN: Patients at Boston Children's Hospital with liver stiffness measurement from 2006 to 2016 and liver biopsy ≤12 months before screening were eligible. Patients enrolled 2006-2011 were used to calibrate liver stiffness measurement cut points and those enrolled 2011-2016 for validation...
April 20, 2018: Journal of Pediatrics
https://www.readbyqxmd.com/read/29678444/biliary-epithelium-a-neuroendocrine-compartment-in-cholestatic-liver-disease
#17
REVIEW
Laurent Ehrlich, Marinda Scrushy, Fanyin Meng, Terry C Lairmore, Gianfranco Alpini, Shannon Glaser
Hepatic fibrosis is characterized by abnormal accumulation of extracellular matrix (ECM) that can lead to ductopenia, cirrhosis, and even malignant transformation. In this review, we examine cholestatic liver diseases characterized by extensive biliary fibrosis such as primary sclerosing cholangitis (PSC), primary biliary cholangitis (PBC), polycystic liver disease (PLD), and MDR2-/- and BDL mouse models. Following biliary injury, cholangiocytes, the epithelial cells that line the bile ducts, become reactive and adopt a neuroendocrine phenotype in which they secrete and respond to neurohormones and neuropeptides in an autocrine and paracrine fashion...
April 17, 2018: Clinics and Research in Hepatology and Gastroenterology
https://www.readbyqxmd.com/read/29668551/fetal-onset-congenital-dyserythropoietic-anemia-type-1-due-to-a-novel-mutation-with-severe-iron-overload-and-severe-cholestatic-liver-disease
#18
Hui-Lin Chin, Le Ye Lee, Pei Lin Koh
We report a rare case of severe congenital dyserythropoietic anemia type 1 with fetal onset. Our patient presented with fetal hydrops from 19 weeks of gestation, requiring multiple intrauterine transfusions. At birth, she had severe hemolytic anemia with severe jaundice, and was subsequently transfusion dependent. She eventually developed severe iron overload and fulminant liver failure before her demise at 5 months of age. Genetic testing revealed a novel mutation in CDAN1.
April 17, 2018: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/29660435/yangonin-protects-against-cholestasis-and-hepatotoxity-via-activation-of-farnesoid-x-receptor-in-vivo-and-in-vitro
#19
Xiaoguang Gao, Ting Fu, Changyuan Wang, Chenqing Ning, Kexin Liu, Zhihao Liu, Huijun Sun, Xiaodong Ma, Xiaokui Huo, Xiaobo Yang, Ming Zou, Qiang Meng
Cholestasis is a clinical syndrome with systemic and intrahepatic accumulation of excessive toxic bile acids that ultimately cause hepatobiliary injury. Recently obeticholic acid (OCA) which is a farnesoid X receptor (FXR) agonist was approved by FDA to treat cholestatic liver diseases, which provided us a newly therapeutic strategy against cholestasis. The purpose of the current study is to screen novel FXR agonists and verify the anti-cholestasis effect of yangonin in vivo and in vitro. The computational strategy of two-dimensional virtual screening was used to search for new FXR agonists, and dual-luciferase reporter gene assay was used to further demonstrate FXR activation by yangonin...
April 13, 2018: Toxicology and Applied Pharmacology
https://www.readbyqxmd.com/read/29658451/-clinical-and-genetic-analysis-of-a-pediatric-patient-with-sodium-taurocholate-cotransporting-polypeptide-deficiency
#20
Hua Li, Jian-Wu Qiu, Gui-Zhi Lin, Mei Deng, Wei-Xia Lin, Ying Cheng, Yuan-Zong Song
Sodium taurocholate cotransporting polypeptide (NTCP) deficiency is an inborn error of bile acid metabolism caused by mutations of SLC10A1 gene. This paper reports the clinical and genetic features of a patient with this disease. A 3.3-month-old male infant was referred to the hospital with the complaint of jaundiced skin and sclera over 3 months. Physical examination revealed moderate jaundice of the skin and sclera. The liver was palpable 3.5 cm below the right subcostal margin with a medium texture. Serum biochemistry analysis revealed markedly elevated bilirubin (predominantly direct bilirubin) and total bile acids (TBA), as well as decreased 25-OH-VitD level...
April 2018: Zhongguo Dang Dai Er Ke za Zhi, Chinese Journal of Contemporary Pediatrics
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