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https://www.readbyqxmd.com/read/29147139/insights-into-the-management-of-wilson-s-disease
#1
REVIEW
Mohmadshakil Kathawala, Gideon M Hirschfield
Wilson's disease is a rare, inherited autosomal recessive disease of copper metabolism, in which the causative gene, ATP7B, results in absent or reduced function of the ATP7B transporter important for biliary excretion of copper and incorporation of copper into caeruloplasmin. Affected patients accumulate excessive copper within the liver, brain and other tissues. A disease mainly of children, adolescents and young adults; clinical features vary from the asymptomatic state to chronic liver disease, acute liver failure, and neuropsychiatric manifestations...
November 2017: Therapeutic Advances in Gastroenterology
https://www.readbyqxmd.com/read/29145470/active-immunization-in-patients-transplanted-for-hepatitis-b-virus-related-liver-diseases-a-prospective-study
#2
Anli Yang, Zhiyong Guo, Qingqi Ren, Linwei Wu, Yi Ma, Anbin Hu, Dongping Wang, Haidan Ye, Xiaofeng Zhu, Weiqiang Ju, Xiaoshun He
INTRODUCTION: Prophylactic administration of hepatitis B immunoglobulin (HBIG) and nucleos(t)ide analogues (NAs) is the standard treatment for controlling hepatitis B virus (HBV) recurrence after liver transplantation (LT). Since lifelong use of HBIG is expensive and inconvenient and the antibodies level in anti-hepatitis B surface (HBs) is not sustainable and stable, an alternative strategy is to produce anti-HBs antibodies by active immunization. Our present study aimed to prospectively investigate the efficacy and safety of procedural HBV vaccination in transplanted patients...
2017: PloS One
https://www.readbyqxmd.com/read/29145424/levosimendan-protects-human-hepatocytes-from-ischemia-reperfusion-injury
#3
Stefanie N Brunner, Nicolai V Bogert, Andreas A Schnitzbauer, Eva Juengel, Anton Moritz, Isabella Werner, Angela Kornberger, Andres Beiras-Fernandez
BACKGROUND: Ischemia-reperfusion injury (IRI) is a major challenge in liver transplantation. The mitochondrial pathway plays a pivotal role in hepatic IRI. Levosimendan, a calcium channel sensitizer, was shown to attenuate apoptosis after IRI in animal livers. The aim of this study was to investigate the effect of levosimendan on apoptosis in human hepatocytes. METHODS: Primary human hepatocytes were either exposed to hypoxia or cultured under normoxic conditions...
2017: PloS One
https://www.readbyqxmd.com/read/29142490/postoperative-elevated-resistive-indices-do-not-predict-hepatic-artery-thrombosis-in-extended-criteria-donor-livers
#4
Eric J Siskind, Fauzia Vandermeer, Tamar R Siskind, David A Bruno, Samuel Sultan, Josue Alvarez-Casas, Arielle Stafford, Barton Lane, John C Lamattina, Rolf N Barth, Steven I Hanish
Postoperative transplant liver ultrasounds were analyzed in standard criteria donor (SCD), extended criteria donor (ECD), and donation after cardiac death (DCD) liver allografts to determine if elevated resistive indices (RIs) are consistently present and if they are pathological. Postoperative transplant liver ultrasounds were reviewed from 115 consecutive patients. Hepatic arterial RIs were stratified based on the type of donor: DCD, macrosteatosis (>30%), or standard criteria. In all patients with elevated RI, subsequent ultrasounds were reviewed to demonstrate RI normalization...
December 2017: International Journal of Angiology: Official Publication of the International College of Angiology, Inc
https://www.readbyqxmd.com/read/29142481/rescue-case-of-low-birth-weight-infant-with-acute-hepatic-failure
#5
Noriki Okada, Yukihiro Sanada, Taizen Urahashi, Yoshiyuki Ihara, Naoya Yamada, Yuta Hirata, Takumi Katano, Kentaro Ushijima, Shinya Otomo, Shujiro Fujita, Koichi Mizuta
We report a case involving a rescued low birth weight infant (LBWI) with acute liver failure. CASE: The patient was 1594 g and 32(3/7) gestational wk at birth. At the age of 11 d, she developed acute liver failure due to gestational alloimmune liver disease. Exchange transfusion and high-dose gamma globulin therapy were initiated, and body weight increased with enteral nutrition. Exchange transfusion was performed a total of 33 times prior to living donor liver transplantation (LDLT)...
October 28, 2017: World Journal of Gastroenterology: WJG
https://www.readbyqxmd.com/read/29142462/a-national-report-from-china-liver-transplant-registry-steroid-avoidance-after-liver-transplantation-for-hepatocellular-carcinoma
#6
Qiang Wei, Feng Gao, Runzhou Zhuang, Qi Ling, Qinghong Ke, Jian Wu, Tian Shen, Mangli Zhang, Min Zhang, Xiao Xu, Shusen Zheng
Objective: We aimed to evaluate the efficacy and safety of steroid-free immunosuppression after liver transplantation (LT) for hepatocellular carcinoma (HCC). Methods: We retrospectively analyzed HCC recipients without steroids after LT (SF group, n=368) based on the China Liver Transplant Registry (CLTR) database. These recipients were matched 1:2 with patients using steroids (S group, n=736) for the same period after LT for HCC, according to propensity scores...
October 2017: Chinese Journal of Cancer Research, Chung-kuo Yen Cheng Yen Chiu
https://www.readbyqxmd.com/read/29142366/nested-stromal-epithelial-tumor-of-liver-with-recurrent-extrahepatic-metastasis-role-of-fluorodeoxyglucose-positron-emission-tomography-computed-tomography
#7
Ishan Garg, Maria J Baladron Zenetti, Ayse T Kendi
Nested stromal-epithelial tumor (NSET) is a very rare nonhepatocytic and nonbiliary primary tumor of the liver. An 8-year-old boy was incidentally detected with hepatic lesions, involving both lobes of the liver for which he later underwent orthotopic liver transplant. The hepatic lesions were confirmed to be NSET following histopathological examination of explant liver specimen. He later developed recurrence with multiple metastatic lesions, including multi-station nodal and right talar bone involvement. We here present the case highlighting the importance of (18)F-fludeoxyglucose positron emission tomography/computed tomography in the management of this rare tumor, in particular for monitoring disease progression and/or recurrence...
October 2017: Indian Journal of Nuclear Medicine: IJNM: the Official Journal of the Society of Nuclear Medicine, India
https://www.readbyqxmd.com/read/29141010/multiple-effects-of-toxins-isolated-from-crotalus-durissus-terrificus-on-the-hepatitis-c-virus-life-cycle
#8
Jacqueline Farinha Shimizu, Carina Machado Pereira, Cintia Bittar, Mariana Nogueira Batista, Guilherme Rodrigues Fernandes Campos, Suely da Silva, Adélia Cristina Oliveira Cintra, Carsten Zothner, Mark Harris, Suely Vilela Sampaio, Victor Hugo Aquino, Paula Rahal, Ana Carolina Gomes Jardim
Hepatitis C virus (HCV) is one of the main causes of liver disease and transplantation worldwide. Current therapy is expensive, presents additional side effects and viral resistance has been described. Therefore, studies for developing more efficient antivirals against HCV are needed. Compounds isolated from animal venoms have shown antiviral activity against some viruses such as Dengue virus, Yellow fever virus and Measles virus. In this study, we evaluated the effect of the complex crotoxin (CX) and its subunits crotapotin (CP) and phospholipase A2 (PLA2-CB) isolated from the venom of Crotalus durissus terrificus on HCV life cycle...
2017: PloS One
https://www.readbyqxmd.com/read/29139181/direct-antiviral-treatment-of-chronic-hepatitis-c-in-heart-transplant-recipients
#9
Martina Vitrone, Roberto Andini, Irene Mattucci, Ciro Maiello, Luigi Atripaldi, Emanuele Durante-Mangoni, Rosa Zampino
Direct-acting antiviral agents (DAAs) are a safe and effective treatment for chronic hepatitis C (CHC). This may be particularly valuable for patients with severe comorbidities or baseline conditions, including non-liver solid organ transplant. We report cases of two heart transplant recipients with CHC treated with DAA (sofosbuvir and daclatasvir) achieving sustained virological response. Treatment was well tolerated and no relevant side effects were observed. The drug-drug interactions and graft function were carefully monitored...
November 15, 2017: Transplant Infectious Disease: An Official Journal of the Transplantation Society
https://www.readbyqxmd.com/read/29138589/clinical-characteristics-and-outcome-of-toxicity-from-amanita-mushroom-poisoning
#10
Satariya Trakulsrichai, Charuwan Sriapha, Achara Tongpoo, Umaporn Udomsubpayakul, Sunun Wongvisavakorn, Sahaphume Srisuma, Winai Wananukul
Objective: To describe and analyze the clinical characteristics and outcome of amatoxin poisoning cases. Methods: We performed a retrospective cohort study of amatoxin poisoning cases from Ramathibodi Poison Center Toxic Exposure Surveillance System, from May 2013 to August 2015. Results: There were 30 consultations with a total of 55 poisoning cases. Most cases were male and from the north-east region. Hepatitis, acute kidney injury, jaundice, and coagulopathy accounted for 74%, 46...
2017: International Journal of General Medicine
https://www.readbyqxmd.com/read/29137590/a-closing-chapter-hepatitis-c-genotype-3-elimination-in-liver-transplant-sofosbuvir-daclatasvir-in-a-hard-to-treat-population
#11
Eva Maria Teegen, Brigitta Globke, Eckart Schott, Johann Pratschke, Dennis Eurich
OBJECTIVES: Historically, hepatitis C virus genotype 3 infection has not been as hard to treat as genotype 1 using interferon-based therapy. Now, genotype 3 infection can be treated using interferon-free regimes such as the combination of sofosbuvir and daclatasvir, which is a highly successful and reliable therapeutic option before liver transplant. However, real world data are rather limited regarding the use of antivirals (sofosbuvir/daclatasvir) for hepatitis C virus genotype 3 recurrence after liver transplant...
November 15, 2017: Experimental and Clinical Transplantation
https://www.readbyqxmd.com/read/29136329/follow-up-of-sustained-virologic-responders-with-hepatitis-c-and-advanced-liver-disease-after-interferon-ribavirin-free-treatment
#12
Karin Kozbial, Stephan Moser, Ramona Al-Zoairy, Remy Schwarzer, Christian Datz, Rudolf Stauber, Hermann Laferl, Michael Strasser, Sandra Beinhardt, Albert Friedrich Stättermayer, Michael Gschwantler, Heinz Zoller, Andreas Maieron, Ivo Graziadei, Michael Trauner, Petra Steindl-Munda, Harald Hofer, Peter Ferenci
BACKGROUND: The introduction of direct-acting antivirals (DAA) has increased sustained virological response (SVR) rates in patients with advanced liver disease and chronic hepatitis C(CHC)infection. At present, data on clinical outcome and long-term durability of viral eradication after successful DAA therapy are scarce. AIM: To evaluate the long-term success of viral eradication in patients with advanced fibrosis or cirrhosis treated with DAAs. METHODS: Five hundred and fifty one patients with advanced fibrosis (n=158) or liver cirrhosis (CPS-A:317,CPS-B/C:76) and SVR after interferon and ribavirin-free DAA therapy treated between October 2013 and April 2016 were studied with a median follow-up of 65...
November 14, 2017: Liver International: Official Journal of the International Association for the Study of the Liver
https://www.readbyqxmd.com/read/29136322/heme-oxygenase-1-regulates-sirtuin-1-autophagy-pathway-in-liver-transplantation-from-mouse-to-human
#13
Kojiro Nakamura, Shoichi Kageyama, Shi Yue, Jing Huang, Takehiro Fujii, Bibo Ke, Rebecca A Sosa, Elaine F Reed, Nakul Datta, Ali Zarrinpar, Ronald W Busuttil, Jerzy W Kupiec-Weglinski
Liver ischemia-reperfusion injury (IRI) represents a major risk factor of early graft dysfunction and a key obstacle to expanding the donor pool in orthotopic liver transplantation (OLT). Although graft autophagy is essential for resistance against hepatic IRI, its significance in clinical OLT remains unknown. Despite recent data identifying heme oxygenase-1 (HO-1) as a putative autophagy inducer, its role in OLT and interactions with Sirtuin-1 (SIRT1), a key autophagy regulator, have not been studied. We aimed to examine HO-1 mediated autophagy induction in human OLT and in a murine OLT model with extended (20h) cold storage, as well as to analyze the requirement for SIRT1 in autophagy regulation by HO-1...
November 14, 2017: American Journal of Transplantation
https://www.readbyqxmd.com/read/29135887/ischemia-reperfusion-injury-in-aged-livers-the-energy-metabolism-inflammatory-response-and-autophagy
#14
Chunyi Kan, Luisa Ungelenk, Amelie Lupp, Olaf Dirsch, Uta Dahmen
Due to the lack of adequate organs, the number of patients with end-stage liver diseases, acute liver failure or hepatic malignancies waiting for liver transplantation is constantly increasing. Accepting aged liver grafts is one of the strategies expanding the donor pool to ease the discrepancy between the growing demand and the limited supply of donor organs. However, recipients of organs from old donors may show an increased post-transplantation morbidity and mortality due to enhanced ischemia reperfusion injury...
November 11, 2017: Transplantation
https://www.readbyqxmd.com/read/29132522/nonalcoholic-fatty-liver-disease
#15
REVIEW
Patrick H Sweet, Teresa Khoo, Steven Nguyen
Nonalcoholic fatty liver disease (NAFLD) defines a condition of hepatic steatosis with or without hepatic injury. NAFLD is increasing in prevalence worldwide and presents a public health burden. Most patients are asymptomatic, although some present with fatigue and right upper quadrant pain. NAFLD is discovered incidentally when patients have elevated liver enzymes or fatty liver is seen on imaging modalities. Imaging studies can confirm fatty deposits in the liver, but needle biopsy is needed to determine degree of inflammation...
December 2017: Primary Care
https://www.readbyqxmd.com/read/29127105/epor2-%C3%A3-cr2-independendent-effects-of-low-dose-epoetin-a-in-porcine-liver-transplantation
#16
Linus Kebschull, Leon Franz Christoph Theilmann, Annika Mohr, Wencke Uennigmann, Sandra Stoeppeler, Barbara Heitplatz, Hans-Ullrich Spiegel, Ralf Bahde, Daniel Michael Palmes, Felix Becker
Ischemia-reperfusion injury (IRI) remains the key component of graft damage during transplantation. Erythropoietin (EPO) induces anti-inflammatory and anti-apoptotic effects via the EPOR2/ßcR2-complex, with the potential risk of thrombosis. Previous work indicates that EPO has EPOR2/ßcR2-independent protective effects via direct effects on the endothelium. As the EPOR2/ßcR2-receptor has a very low affinity for EPO, we aimed to test the hypothesis that EPO doses below the level that stimulate this receptor elicit cytoprotective effects via endothelial stimulation in a porcine liver transplantation model...
November 10, 2017: Bioscience Reports
https://www.readbyqxmd.com/read/29126693/characterization-of-patients-with-autoimmune-hepatitis-at-an-university-hospital-in-medell%C3%A3-n-colombia-cohort-study
#17
Gabriel Sebastián Díaz-Ramírez, Juan Ignacio Marín-Zuluaga, Jorge Hernando Donado-Gómez, Octavio Muñoz-Maya, Óscar Santos-Sánchez, Juan Carlos Restrepo-Gutiérrez
INTRODUCTION: Autoimmune hepatitis is a chronic liver disease that impacts on morbidity and mortality of patients. Few epidemiological data exist of this in Latin America and Colombia. OBJECTIVES: The aim of this study is to describe the demographic, clinical and laboratory characteristics of the patients; the treatment and the response to it, the evolution and course of the disease, requirement of liver transplantation and mortality. METHODS: Historical cohort study that include patients attended at an University Hospital in Medellin, Colombia between January 2010 and December 2016 with ≥16 years age at the time of diagnosis of autoimmune hepatitis...
November 7, 2017: Gastroenterología y Hepatología
https://www.readbyqxmd.com/read/29125676/decreasing-mortality-and-disease-severity-in-hepatitis-c-patients-awaiting-liver-transplantation-in-the-united-states
#18
Allison Kwong, W Ray Kim, Ajitha Mannalithara, Nae-Yun Heo, Prowpanga Udompap, Donghee Kim
BACKGROUND: Hepatitis C virus (HCV) infection has been the leading indication for liver transplantation (LT) in the United States. Since 2013, interferon-free antiviral therapy has led to sustained virologic response in many LT candidates. We compared the waitlist mortality of HCV patients with that of patients with other chronic liver diseases. METHODS: Data for primary LT candidates were obtained from the Organ Procurement and Transplantation Network database...
November 10, 2017: Liver Transplantation
https://www.readbyqxmd.com/read/29125670/use-of-a-hepatitis-c-virus-hcv-rna-positive-donor-in-a-treated-hcv-rna-negative-liver-transplant-recipient
#19
Isabel Campos-Varela, Eliana Z Agudelo, Monika Sarkar, John P Roberts, Norah A Terrault
The shortage of livers has led most transplant centers to use extended criteria donors. Hepatitis C virus (HCV) RNA-positive donors are typically not given to patients who have cleared HCV. A 64-year-old male with chronic hepatitis C, genotype 1b was listed for LT with hepatocellular carcinoma. While on the waiting list, the patient was treated with sofosbuvir, ledipasvir, and ribavirin and achieved an HCV RNA <15 IU/mL by week 10. At week 18 of a planned 24-week treatment course, the patient underwent deceased-donor LT and received an organ from an anti-HCV-positive donor...
November 10, 2017: Transplant Infectious Disease: An Official Journal of the Transplantation Society
https://www.readbyqxmd.com/read/29125261/hepatitis-b-and-liver-transplantation
#20
Alberto Ferrarese, Alberto Zanetto, Francesco P Russo
Liver transplantation (LT) is the only effective treatment for hepatitis B-virus (HBV) related end stage liver disease, even if the outcome of these patients, has significantly improved after introduction of effective and well tolerated nucleos/tide analogues (NUC). Pre-transplant therapy has been initially based on lamivudine, but entecavir and tenofovir represent the currently recommended first-line therapeutic option in patients with HBV decompensated cirrhosis. After LT, the development of hepatitis B immunoglobulin (HBIG) in the early 1990s change dramatically the prognosis of these patients by reducing the incidence of HBV recurrence and increasing survival rate...
November 10, 2017: Minerva Gastroenterologica e Dietologica
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