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https://www.readbyqxmd.com/read/28804516/the-role-of-sebelipase-alfa-in-the-treatment-of-lysosomal-acid-lipase-deficiency
#1
REVIEW
Angelika L Erwin
Lysosomal acid lipase deficiency (LALD) is a lysosomal storage disorder (LSD) characterized either by infantile onset with fulminant clinical course and very poor prognosis or childhood/adult-onset disease with an attenuated phenotype. The disorder is often misdiagnosed or remains undiagnosed in children and adults due to a rather unspecific clinical presentation with dyslipidemia and steatohepatitis. Until recently, no good treatment options were available for LALD. Despite supportive and symptomatic therapies, death occurred before 1 year of age in patients with infantile-onset disease and patients with childhood/adult-onset LALD suffered from significant complications, such as liver cirrhosis, requiring liver transplantation and early-onset cardiovascular disease...
July 2017: Therapeutic Advances in Gastroenterology
https://www.readbyqxmd.com/read/28804513/noncirrhotic-hepatocellular-carcinoma-etiology-and-occult-hepatitis-b-virus-infection-in-a-hepatitis-b-virus-endemic-area
#2
Chang Woo Shim, Joong-Won Park, So Hee Kim, Jin Sook Kim, Bo Hyun Kim, Sung Hoon Kim, Eun Kyung Hong
BACKGROUND: Although hepatocellular carcinoma (HCC) usually develops in cirrhotic livers, a minority of cases occur in noncirrhotic livers (NCLs). We investigated etiology, clinicopathological features, and occult hepatitis B virus (HBV) infection (OBI) in patients with NCL HCC in an HBV-endemic area. METHODS: A total of 710 patients who underwent resection or transplantation for HCC at the National Cancer Center (NCC), Korea, were enrolled. HCC and fibrosis stage were diagnosed pathologically...
July 2017: Therapeutic Advances in Gastroenterology
https://www.readbyqxmd.com/read/28803656/outcomes-of-transcatheter-versus-surgical-aortic-valve-implantation-for-aortic-stenosis-in-patients-with-hepatic-cirrhosis
#3
Fahad Alqahtani, Sami Aljohani, Anas Ghabra, Fares Alahdab, Akram Kawsara, David R Holmes, Mohamad Alkhouli
Current risk prediction tools for transcatheter aortic valve implantation (TAVI) or surgical aortic valve replacement (SAVR) do not include variables associated with clinically significant hepatic disease. Accordingly, outcome data of TAVI or SAVR in patients with liver cirrhosis are limited. We sought to assess contemporary trends and outcomes of TAVI and SAVR in patients with liver cirrhosis using a national database. The Nationwide Inpatient Sample was used to identify patients with liver cirrhosis who underwent TAVI or SAVR between 2003 and 2014...
July 14, 2017: American Journal of Cardiology
https://www.readbyqxmd.com/read/28802816/safety-and-efficacy-of-an-8-week-regimen-of-grazoprevir-plus-ruzasvir-plus-uprifosbuvir-compared-with-grazoprevir-plus-elbasvir-plus-uprifosbuvir-in-participants-without-cirrhosis-infected-with-hepatitis-c-virus-genotypes-1-2-or-3-c-crest-1-and-c-crest-2-part
#4
Edward J Gane, Stephen Pianko, Stuart K Roberts, Alexander J Thompson, Stefan Zeuzem, Eli Zuckerman, Ziv Ben-Ari, Graham R Foster, Kosh Agarwal, Alex L Laursen, Jan Gerstoft, Wei Gao, Hsueh-Cheng Huang, Brian Fitzgerald, Doreen Fernsler, Jerry J Li, Anjana Grandhi, Hong Liu, Feng-Hsiu Su, Shuyan Wan, Zhen Zeng, Huei-Ling Chen, Frank J Dutko, Bach-Yen T Nguyen, Janice Wahl, Michael N Robertson, Eliav Barr, Wendy W Yeh, Rebeca M Plank, Joan R Butterton, Rafael Esteban
BACKGROUND: New hepatitis C virus (HCV) therapies with pan-genotypic efficacy are needed. The goals of part A of C-CREST-1 and C-CREST-2 were to compare the efficacies of two doses (300 mg or 450 mg once daily) of uprifosbuvir (MK-3682; NS5B inhibitor) in an 8-week regimen combined with grazoprevir (NS3/4A inhibitor; 100 mg once daily) and an NS5A inhibitor, either elbasvir (50 mg once daily) or ruzasvir (MK-8408; 60 mg once daily), and to evaluate the safety and tolerability of these combination regimens in individuals infected with genotypes 1, 2, or 3...
August 9, 2017: Lancet. Gastroenterology & Hepatology
https://www.readbyqxmd.com/read/28802814/safety-and-efficacy-of-a-fixed-dose-combination-regimen-of-grazoprevir-ruzasvir-and-uprifosbuvir-with-or-without-ribavirin-in-participants-with-and-without-cirrhosis-with-chronic-hepatitis-c-virus-genotype-1-2-or-3-infection-c-crest-1-and-c-crest-2-part-b-two
#5
Eric Lawitz, Maria Buti, John M Vierling, Piero L Almasio, Savino Bruno, Peter J Ruane, Tarek I Hassanein, Beat Muellhaupt, Brian Pearlman, Ligita Jancoriene, Wei Gao, Hsueh-Cheng Huang, Aimee Shepherd, Brynne Tannenbaum, Doreen Fernsler, Jerry J Li, Anjana Grandhi, Hong Liu, Feng-Hsiu Su, Shuyan Wan, Frank J Dutko, Bach-Yen T Nguyen, Janice Wahl, Michael N Robertson, Eliav Barr, Wendy W Yeh, Rebeca M Plank, Joan R Butterton, Eric M Yoshida
BACKGROUND: There is a need for hepatitis C virus (HCV) therapies with excellent efficacy across genotypes and in diverse populations. Part A of the C-CREST-1 and C-CREST-2 trials led to the selection of a three-drug regimen of grazoprevir (MK-5172; an HCV NS3/4A protease inhibitor; 100 mg/day) plus ruzasvir (MK-8408; an NS5A inhibitor; 60 mg/day) plus uprifosbuvir (MK-3682; an HCV NS5B polymerase inhibitor; 450 mg/day). Part B of the studies tested this combination as a single formulation in different treatment durations in a broader population...
August 9, 2017: Lancet. Gastroenterology & Hepatology
https://www.readbyqxmd.com/read/28802065/quercetin-reverses-experimental-cirrhosis-by-immunomodulation-of-the-proinflammatory-and-profibrotic-processes
#6
Sael Casas-Grajales, Luis F Vázquez-Flores, Erika Ramos-Tovar, Erika Hernández-Aquino, Rosa E Flores-Beltrán, Carlos M Cerda-García-Rojas, Javier Camacho, Mineko Shibayama, Víctor Tsutsumi, Pablo Muriel
The ability of quercetin to reverse an established cirrhosis has not yet been investigated. Therefore, the aim of the present study was to examine the efficacy of this flavonoid in reversing experimental cirrhosis. Cirrhosis was induced by intraperitoneal administration of TAA (200 mg/kg of body weight) three times per week for 8 weeks or by intraperitoneal petrolatum-CCl4 (400 mg/kg of body weight) administration three times per week for 8 weeks. To determine the capacity of quercetin to prevent liver fibrosis, the flavonoid (50 mg/kg of body weight, p...
August 12, 2017: Fundamental & Clinical Pharmacology
https://www.readbyqxmd.com/read/28801610/creb3l2-mediated-expression-of-sec23a-sec24d-is-involved-in-hepatic-stellate-cell-activation-through-er-golgi-transport
#7
Shotaro Tomoishi, Shinichi Fukushima, Kentaro Shinohara, Toshiaki Katada, Kota Saito
Hepatic fibrosis is caused by exaggerated wound healing response to chronic injury, which eventually leads to hepatic cirrhosis. Differentiation of hepatic stellate cells (HSCs) to myofibroblast-like cells by inflammatory cytokines is the critical step in fibrosis. This step is accompanied by enlargement of the endoplasmic reticulum (ER) and Golgi apparatus, suggesting that protein synthesis and secretion are augmented in the activated HSCs. However, the process of rearrangement of secretory organelles and their functions remain to be fully elucidated...
August 11, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28801369/hepatitis-b-virus-upregulates-the-expression-of-c-reactive-protein-both-in-vivo-and-in-vitro
#8
Shiyong Hao, Yan Wang, Guosheng Gao, Zhishan Li
Hepatitis B virus (HBV) infection induces immune-mediated inflammatory responses. The C-reactive protein (CRP) is an inflammatory marker that is synthesized by the liver. The aim of the present study was to investigate the effect of HBV infection on the expression of CRP and its clinical significance. A reverse transcription-polymerase chain reaction (RT-PCR) analysis was employed to examine the expression of CRP mRNA in the human hepatocellular carcinoma (HCC) cell lines HepG2 and HepG2.2.15. A fully automatic biochemical analyzer was used to examine the serum levels of CRP in patients with chronic HBV infections and in healthy controls...
August 2017: Annals of Clinical and Laboratory Science
https://www.readbyqxmd.com/read/28801286/-value-of-detection-of-serum-glypican-3-level-in-diagnosis-and-therapeutic-effect-evaluation-of-primary-hepatocellular-carcinoma
#9
Yuan-Yuan Wang, Chen-Jie Zhou, Jing Li, Ling Zhou, Ming-Song Li, Bing Xiao
OBJECTIVE: To explore the clinical value of detecting serum glypican-3 in the diagnosis and therapeutic effect evaluation of primary hepatocellular carcinoma (PHC). METHODS: Using sandwich ELISA, we detected serum glypican-3 levels in 60 patients with PHC, 60 with metastatic liver cancer, 50 with liver cirrhosis, 50 with chronic viral hepatitis, 20 with hepatic cyst, 20 with fatty liver, 20 with hepatic hemangioma and 20 with drug-induced hepatitis as well as in 40 healthy subjects (control)...
August 20, 2017: Nan Fang Yi Ke da Xue Xue Bao, Journal of Southern Medical University
https://www.readbyqxmd.com/read/28800720/jc-virus-related-progressive-multifocal-leukoencephalopathy-after-living-donor-liver-transplant-a-rare-case
#10
Archana Rastogi, Natasha Gulati, Chhagan Bihari, Ashok Chaudhary, Kalpana Bansal, Shridhar Sasturkar, Shalini Thapar, Viniyendra Pamecha
Progressive multifocal leukoencephalopathy caused by polyoma JC virus is a rare and severe demyelinating disease of the central nervous system. It occurs in immunocompromised patients and is scarcely reported in liver transplant recipients. Brain biopsy demonstrating demyelination with presence of foamy macrophages, relative preservation of axons, astrogliosis, and typical polyomavirus inclusions in the enlarged oligodendroglial nuclei is essential for diagnosis. Here, we report a case of JC virus-associated progressive multifocal leukoencephalopathy in a living-donor liver transplant recipient who was transplanted for hepatitis C virus-related decompensated cirrhosis...
August 12, 2017: Experimental and Clinical Transplantation
https://www.readbyqxmd.com/read/28800087/imaging-features-of-non-alcoholic-fatty-liver-disease-in-children-and-adolescents
#11
REVIEW
Michele Di Martino, Kameliya Koryukova, Mario Bezzi, Carlo Catalano
Non-invasive diagnosis and quantification of liver steatosis is important to overcome limits of liver biopsy, in order to follow up patients during their therapy and to establish a reference standard that can be used in clinical trials and longitudinal studies. Imaging offers several methods in this setting: ultrasound, which is the cheapest technique and easy to perform; magnetic resonance spectroscopy (MRS), which reflects the real content of triglycerides in a specific volume; and proton density fat fraction (PDFF) magnetic resonance, which is a simple method that reflects the distribution of the fat in the whole liver...
August 11, 2017: Children
https://www.readbyqxmd.com/read/28800033/predictors-of-early-discontinuation-of-interferon-free-direct-antiviral-agents-in-patients-with-hepatitis-c-virus-and-advanced-liver-fibrosis-results-of-a-real-life-cohort
#12
Noelle Miotto, Leandro C Mendes, Letícia P Zanaga, Eduardo S L Goncales, Maria S K Lazarini, Marcelo N Pedro, Fernando L Gonçales, Raquel S B Stucchi, Aline G Vigani
AIM: The aim of this study was to determine risk factors for premature treatment discontinuation among patients with hepatitis C and advanced fibrosis with advanced fibrosis treated with interferon (IFN)-free direct antiviral agents (DAA)-based therapy. PATIENTS AND METHODS: We included all patients with chronic hepatitis C virus infection and advanced liver fibrosis in whom treatment was initiated with IFN-free DAA therapy at a university hospital from December 2015 through June 2016...
August 10, 2017: European Journal of Gastroenterology & Hepatology
https://www.readbyqxmd.com/read/28798943/a-fatal-case-of-erdheim-chester-disease-with-hepatic-involvement
#13
Gokulakrishnan Balasubramanian, Alexandra Modiri, Marina Affi, Catherine E Hagen, Bjorn Batdorf, Kiyoko Oshima, Laura Michaelis, Kia Saeian
Erdheim-Chester disease (ECD) is a rare form of systemic histiocytosis, typically presenting with striking osseous involvement characterized by bilateral osteosclerosis and involvement of organs such as the lung, pituitary gland, heart, and brain. Liver involvement with ECD is extremely uncommon. We report a 56-year-old woman presenting with newly diagnosed cirrhosis and signs concerning for intra-abdominal malignancy, including omental caking and peritoneal thickening. Liver biopsy demonstrated xanthogranulomatous infiltration from ECD...
2017: ACG Case Reports Journal
https://www.readbyqxmd.com/read/28798283/healthcare-costs-for-chronic-hepatitis-c-in-south-korea-from-2009-to-2013-an-analysis-of-the-national-health-insurance-claims-data
#14
Moran Ki, Hwa Young Choi, Kyung-Ah Kim, Eun Sun Jang, Sook-Hyang Jeong
Background/Aims: The introduction of direct-acting antivirals (DAA) in 2013 revolutionized hepatitis C virus (HCV) treatment, offering a cure rate >90%. However, this therapy is expensive, and estimations of the number of chronic HCV-infected (CHC) patients and their treatment costs pre-2013 are therefore essential for creating policies and expanding drug access. Herein, we aimed to investigate the number of HCV-related liver disease patients, their healthcare utilization, their annual direct medical costs, and the interferon-based antiviral treatment rates and costs from 2009 to 2013 in South Korea...
August 14, 2017: Gut and Liver
https://www.readbyqxmd.com/read/28797326/severe-liver-disease-related-to-chronic-hepatitis-c-virus-infection-in-treatment-naive-patients-epidemiological-characteristics-and-associated-factors-at-first-expert-centre-visit-france-2000-to-2007-and-2010-to-2014
#15
Alice Sanna, Yann Le Strat, Françoise Roudot-Thoraval, Sylvie Deuffic Burban, Patrizia Carrieri, Elisabeth Delarocque-Astagneau, Christine Larsen
Given recent profound improvements in the effectiveness of antiviral treatment for chronic Hepatitis C virus (HCV) infection, we aimed to describe the characteristics of patients referred to hepatology expert centres in France from 2000 to 2007 and from 2010 to 2014, and to identify factors associated with severe liver disease at their first visit for evaluation. We analysed data from two sources covering all of France: the former hepatitis C surveillance network, which included patients between 2000 and 2007, and the ANRS CO22 HEPATHER multi-centre cohort, which included patients between 2012 and 2014...
July 27, 2017: Euro Surveillance: Bulletin Européen sur les Maladies Transmissibles, European Communicable Disease Bulletin
https://www.readbyqxmd.com/read/28797220/a-review-of-ultrasound-tissue-characterization-with-mean-scatterer-spacing
#16
Zhuhuang Zhou, Weiwei Wu, Shuicai Wu, Kebin Jia, Po-Hsiang Tsui
Tissues exhibiting quasi-periodic structures can be modeled as a collection of diffuse scatterers and coherent scatterers. The mean scatterer spacing (MSS) of coherent and quasi-periodic components is directly related to tissue microstructure and has become an important quantitative ultrasound (QUS) parameter in the characterization of quasi-periodic tissues. In this paper, a review of the literature on the development of MSS as a QUS parameter was conducted. First, a unified theoretical background of MSS estimates was provided...
September 2017: Ultrasonic Imaging
https://www.readbyqxmd.com/read/28796283/flumazenil-versus-placebo-or-no-intervention-for-people-with-cirrhosis-and-hepatic-encephalopathy
#17
REVIEW
Ee Teng Goh, Mette L Andersen, Marsha Y Morgan, Lise Lotte Gluud
BACKGROUND: Hepatic encephalopathy is a common complication of cirrhosis which results in poor brain functioning. The spectrum of changes associated with hepatic encephalopathy ranges from the clinically 'indiscernible' or minimal hepatic encephalopathy to the clinically 'obvious' or overt hepatic encephalopathy. Flumazenil is a synthetic benzodiazepine antagonist with high affinity for the central benzodiazepine recognition site. Flumazenil may benefit people with hepatic encephalopathy through an indirect negative allosteric modulatory effect on gamma-aminobutyric acid receptor function...
August 10, 2017: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/28796076/factors-affecting-mortality-and-resource-use-for-hospitalized-patients-with-cirrhosis-a-population-based-study
#18
Phunchai Charatcharoenwitthaya, Ngamphol Soonthornworasiri, Khemajira Karaketklang, Kittiyod Poovorawan, Wirichada Pan-Ngum, Watcharasak Chotiyaputta, Tawesak Tanwandee, Kamthorn Phaosawasdi
Hospitalizations for advanced liver disease are costly and associated with significant mortality. This population-based study aimed to evaluate factors associated with in-hospital mortality and resource use for the management of hospitalized patients with cirrhosis.Mortality records and resource utilization for 52,027 patients hospitalized with cirrhosis and/or complications of portal hypertension (ascites, hepatic encephalopathy, variceal bleeding, spontaneous bacterial peritonitis, or hepatorenal syndrome) were extracted from a nationally representative sample of Thai inpatients covered by Universal Coverage Scheme during 2009 to 2013...
August 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28796071/overexpression-of-microrna-let-7-correlates-with-disease-progression-and-poor-prognosis-in-hepatocellular-carcinoma
#19
Wenxia Shi, Zili Zhang, Bin Yang, Hua Guo, Li Jing, Tong Liu, Ying Luo, Hui Liu, Yayue Li, Yingtang Gao
The aim of the study was to explore the clinical significance of let-7 expression in hepatocellular carcinoma (HCC).A PCR array was conducted to screen for let-7 expression in early-stage HCC. Next, the deregulation of let-7 was confirmed by quantitative real-time RT-PCR (qRT-PCR) in another set of liver tissues, including normal control (NC), chronic hepatitis (CH), liver cirrhosis (LC), HCC, and adjacent nontumor (NT) tissues. In addition, as the potential target mRNA of let-7, alpha 2(I) collagen (COL1A2) mRNA was also quantified in the above liver tissues...
August 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28795982/treatment-of-patients-with-hepatitis-c-virus-infection-genotype-4-with-ledipasvir-sofosbuvir-in-the-liver-transplant-setting
#20
Faisal Abaalkhail, Hussein Elsiesy, Hany Elbeshbeshy, Mohamed Shawkat, Sarra Yousif, Waheed Ullah, Saleh Alabbad, Ahmed Al-Jedai, Aziza Ajlan, Dieter Broering, Sammy Saab, Mohammed Al Sebayel, Waleed Al-Hamoudi
BACKGROUND: Hepatitis C virus infection is a major cause of liver cirrhosis and hepatocellular carcinoma and the leading indication for liver transplantation. In the Middle East, genotype 4 HCV infection is the most common genotype. However, limited data exists on the treatment of genotype-4 in the liver transplant setting. We evaluated the safety and efficacy of ledipasvir-sofosbuvir (LDV/SOF) in treating HCV genotype-4 infected patients with cirrhosis or postliver transplantation. METHODS: This prospective, single-arm, observational study includes cohort of patients with cirrhosis before liver transplantation (Cohort A) and a cohort of postliver transplantation patients (Cohort B)...
August 9, 2017: Transplantation
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