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https://www.readbyqxmd.com/read/28433104/wilson-disease-symptomatic-liver-therapy
#1
Jan Pfeiffenberger, Karl-Heinz Weiss, Wolfgang Stremmel
Wilson disease leads to symptomatic impairment of liver function or liver cirrhosis. Strict adherence to decoppering agents is essential in these patients. Secondary prevention of additional hepatic damage by avoidance of other toxic substances (e.g., alcohol, drugs) and sufficient calorie intake is recommended. Routine examinations in cirrhotic patients include screening for signs of portal hypertension (esophagus varices), development of ascites, and hepatic encephalopathy. Where varices are present, primary or secondary preventive interventions may include treatment with nonselective beta-blockers or variceal ligation, similar to the approach in patients with liver cirrhosis due to other etiologies...
2017: Handbook of Clinical Neurology
https://www.readbyqxmd.com/read/28433098/wilson-disease-in-children
#2
Eve A Roberts, Piotr Socha
Wilson disease (WD) is an inherited disorder mainly of hepatocellular copper disposition, due to dysfunction of the Wilson ATPase, a P1B-ATPase encoded by the gene ATP7B. In children, as in older age brackets, clinical disease is highly diverse. Although hepatic disease is the common presentation in children/adolescents, neurologic, psychiatric, and hematologic clinical presentations do occur. Very young children may have clinically evident liver disease due to WD. Early diagnosis, preferably when the child/adolescent is asymptomatic, is most likely to result in near-normal longevity with generally good health so long as the patient tolerates effective medication, is adherent to the lifelong treatment regimen, and has consistent access to the medication...
2017: Handbook of Clinical Neurology
https://www.readbyqxmd.com/read/28432731/association-between-progressive-hepatic-morphology-changes-on-serial-mr-imaging-and-clinical-outcome-in-primary-sclerosing-cholangitis
#3
Yu Xuan Kitzing, Siobhan A Whitley, Sara S Upponi, Brijesh Srivastava, Graeme Jm Alexander, David J Lomas
INTRODUCTION: Hepatic morphology changes are well described in Primary Sclerosing Cholangitis and characterised by a combination of atrophy and hypertrophy changes. This study investigates the relationship between progression of these changes over time and clinical outcome in patients with PSC. METHODS: Fifty-three patients with PSC (mean age 44, 28 males and 25 females) who underwent serial MRI liver studies at least one year apart were identified. The first and the last MRI studies were selected for the retrospective analysis...
April 22, 2017: Journal of Medical Imaging and Radiation Oncology
https://www.readbyqxmd.com/read/28428344/vascular-adhesion-protein-1-is-elevated-in-primary-sclerosing-cholangitis-is-predictive-of-clinical-outcome-and-facilitates-recruitment-of-gut-tropic-lymphocytes-to-liver-in-a-substrate-dependent-manner
#4
Palak J Trivedi, Joseph Tickle, Mette Nåmdal Vesterhus, Peter J Eddowes, Tony Bruns, Jani Vainio, Richard Parker, David Smith, Evaggelia Liaskou, Liv Wenche Thorbjørnsen, Gideon M Hirschfield, Kaisa Auvinen, Stefan G Hubscher, Marko Salmi, David H Adams, Chris J Weston
OBJECTIVE: Primary sclerosing cholangitis (PSC) is the classical hepatobiliary manifestation of IBD. This clinical association is linked pathologically to the recruitment of mucosal T cells to the liver, via vascular adhesion protein (VAP)-1-dependent enzyme activity. Our aim was to examine the expression, function and enzymatic activation of the ectoenzyme VAP-1 in patients with PSC. DESIGN: We examined VAP-1 expression in patients with PSC, correlated levels with clinical characteristics and determined the functional consequences of enzyme activation by specific enzyme substrates on hepatic endothelium...
April 20, 2017: Gut
https://www.readbyqxmd.com/read/28426902/hepatic-steatosis-after-pediatric-liver-transplant
#5
Emily R Perito, Tabitha Vase, Rageshree Ramachandran, Andrew Phelps, Kuang-Yu Jen, Robert H Lustig, Vickie A Feldstein, Philip Rosenthal
RATIONALE: Hepatic steatosis develops after liver transplant in 30% of adults, and non-alcoholic fatty liver disease (NAFLD) is the most common chronic liver disease in non-transplanted children. However, post-transplant steatosis has been minimally studied in pediatric liver transplant recipients. We explored the prevalence, persistence, and association with chronic liver damage of hepatic steatosis in these children. RESULTS: In this single-center study of pediatric patients transplanted 1988-2015 (n=318), 31% of those with any post-transplant biopsy (n=271) had ≥1 biopsy with steatosis...
April 20, 2017: Liver Transplantation
https://www.readbyqxmd.com/read/28426800/neutrophil-lymphocyte-ratio-predicts-short-term-mortality-in-patients-with-hepatitis-b-virus-related-acute-on-chronic-liver-failure-treated-with-an-artificial-liver-support-system
#6
Zeng Fan, Chen EnQiang, Du Ling Yao, Yan LiBo, Li Hong, Bai Lang, Feng Ping, Tang Hong
AIM: Hepatitis B virus-related acute-on-chronic liver failure has high short-term mortality. Artificial liver support systems (ALSS) may improve outcome and avoid liver transplantation, but predicting short-term prognosis in such patients is difficult. This study aimed to determine whether the neutrophil-lymphocyte ratio (NLR), an inflammation marker, predicted mortality in patients treated with ALSS. METHODS: A total of 560 patients with hepatitis B virus-related acute-on-chronic liver failure were enrolled, 338 were treated with ALSS and the others treated with standard of care(SOC)...
2017: PloS One
https://www.readbyqxmd.com/read/28426468/indwelling-tunneled-pleural-catheters-for-the-management-of-hepatic-hydrothorax
#7
Kevin P Haas, Alexander C Chen
PURPOSE OF REVIEW: Hepatic hydrothorax is a complication of end-stage liver disease that may have significant associated morbidity. Sodium restriction and diuretic therapy are the mainstays of treatment, though up to a quarter of patients will become refractory to this and will require a pleural drainage procedure. Thoracentesis, transjugular intrahepatic portosystemic shunt, and chemical pleurodesis all have variable success rates and associated complications in the management of hepatic hydrothorax...
April 19, 2017: Current Opinion in Pulmonary Medicine
https://www.readbyqxmd.com/read/28425419/a-rare-bsep-mutation-associated-with-a-mild-form-of-progressive-familial-intrahepatic-cholestasis-type-2
#8
Orith Waisbourd-Zinman, Lea F Surrey, Anna E Schwartz, Pierre A Russo, Jessica Wen
Progressive Familial Intrahepatic Cholestasis type 2 (PFIC2) is a rare cholestatic disorder diagnosed in infancy or childhood that can lead to severe hepatic fibrosis and liver failure. Mutations in the ABCB11 gene result in a deficiency of the bile salt export protein (BSEP) and accumulation of bile inside the hepatocytes. Hepatocellular carcinoma is another condition associated with severe forms of deletion mutations in the ABCB11 gene. Treatment options including ursodeoxycholic acid biliary diversion have mixed outcomes and some patients require liver transplantation...
May 2017: Annals of Hepatology
https://www.readbyqxmd.com/read/28425407/persistence-of-virologic-response-after-liver-transplant-in-hepatitis-c-patients-treated-with-ledipasvir-sofosbuvir-plus-ribavirin-pretransplant
#9
Eric M Yoshida, Paul Kwo, Kosh Agarwal, Christophe Duvoux, François Durand, Markus Peck-Radosavljevic, Leslie Lilly, Bernard Willems, Hugo Vargas, Princy Kumar, Robert S Brown, Yves Horsmans, Shampa De-Oertel, Sarah Arterburn, Hadas Dvory-Sobol, Diana M Brainard, John G McHutchison, Norah Terrault, Mario Rizzetto, Beat Mu Llhaupt
INTRODUCTION: Recurrence of HCV infection in patients with chronic hepatitis C virus (HCV) at the time of liver transplantation is nearly universal and reduces the likelihood of graft and patient survival. MATERIALS AND METHODS: We evaluated outcomes of 17 patients (16 with HCV genotype 1 and 1 with genotype 4) who received up to 12 or 24 weeks of ledipasvir/sofosbuvir plus ribavirin prior to or up to the time of liver transplant in the SOLAR-1 and SOLAR-2 trials...
May 2017: Annals of Hepatology
https://www.readbyqxmd.com/read/28425397/persistence-of-virologic-response-after-liver-transplant-in-hepatitis-c-patients-treated-with-ledipasvir-sofosbuvir-plus-ribavirin-pretransplant
#10
Carmen Vinaixa, Marina Berenguer
No abstract text is available yet for this article.
May 2017: Annals of Hepatology
https://www.readbyqxmd.com/read/28425187/significance-of-measured-intraoperative-portal-vein-flows-after-thrombendvenectomy-in-deceased-donor-liver-transplants-with-portal-vein-thrombosis
#11
Mark Draoua, Nicole Titze, Amar Gupta, Hoylan T Fernandez, Michael Ramsay, Giovanna Saracino, Gregory McKenna, Testa Giuliano, Goran B Klintmalm, Peter T W Kim
BACKGROUND: Adequate portal vein (PV) flow in liver transplantation is essential for a good outcome, and it may be compromised in patients with portal vein thrombosis (PVT). This study evaluated the impact of intraoperatively measured PV flow after PV thrombendvenectomy on outcomes after deceased donor liver transplantation. STUDY DESIGN: The study included 77 patients over a 16-year period who underwent PV thrombendvenectomy with complete flow data. Patients were classified into two groups: high PV flow (>1300 mL/min, N = 55) and low PV flow (≤1300 mL/min, N = 22)...
April 20, 2017: Liver Transplantation
https://www.readbyqxmd.com/read/28422962/docosahexaenoic-acid-blocks-progression-of-western-diet-induced-nonalcoholic-steatohepatitis-in-obese-ldlr-mice
#12
Kelli A Lytle, Carmen P Wong, Donald B Jump
BACKGROUND: Nonalcoholic fatty liver disease (NAFLD) is a major public health concern in western societies. Nonalcoholic steatohepatitis (NASH), the progressive form of NAFLD, is characterized by hepatic steatosis, inflammation, oxidative stress and fibrosis. NASH is a risk factor for cirrhosis and hepatocellular carcinoma. NASH is predicted to be the leading cause of liver transplants by 2020. Despite this growing public health concern, there remain no Food and Drug Administration (FDA) approved NASH treatments...
2017: PloS One
https://www.readbyqxmd.com/read/28422425/negativization-of-viremia-prior-to-liver-transplant-reduces-early-allograft-dysfunction-in-hepatitis-c-recipients
#13
Silvia Martini, Francesco Tandoi, Lodovico Terzi di Bergamo, Silvia Strona, Bruna Lavezzo, Marco Sacco, Francesca Maione, Federica Gonella, Paolo Strignano, Dominic Dell Olio, Mauro Salizzoni, Giorgio Maria Saracco, Renato Romagnoli
Although early allograft dysfunction (EAD) negatively impacts on survival from the first months following liver transplantation (LT), direct-acting antivirals (DAAs) have revolutionized HCV therapy. We investigated the EAD definition best predicting 90-day graft loss and identified EAD risk factors in HCV positive recipients. From 11-2002 to 06-2016, 603 HCV-positive patients (hepatocellular carcinoma 53.4%) underwent a first LT with HCV-negative donors. Median recipient MELD score 15, median donor age 63 years...
April 19, 2017: Liver Transplantation
https://www.readbyqxmd.com/read/28422392/hepatic-artery-reconstruction-in-living-donor-liver-transplantation-using-surgical-loupes-achieving-low-rate-of-hepatic-arterial-thrombosis-in-consecutive-741-recipients-tips-and-tricks-to-overcome-the-poor-hepatic-arterial-flow
#14
Ping-Chun Li, Ashok Thorat, Long-Bin Jeng, Horng-Ren Yang, Ming-Li Li, Chun-Chieh Yeh, Te-Hung Chen, Shih-Chao Hsu, Kin-Shing Poon
BACKGROUND: The reconstruction of hepatic artery (HA) is most complex step in living donor liver transplantation (LDLT) due to smaller diameter of the artery and increased risk of HA related complications. Due to smaller diameter of the HA, many centres use microsurgical technique with interrupted sutures for arterial anastomosis. Aim of our study was to retrospectively investigate the outcomes after HA reconstruction performed under magnifying loupes using "parachute technique". MATERIALS AND METHODS: From August 2002 to 31(st) August 2016, LDLT was performed in 766 recipients...
April 19, 2017: Liver Transplantation
https://www.readbyqxmd.com/read/28422386/corticosteroid-could-possibly-reduce-serum-levels-of-pro-inflammatory-cytokines-in-fulminant-hepatitis-a-case-series
#15
Keiichi Fujiwara, Seiji Hida, Shin Yasui, Osamu Yokosuka, Shigeto Oda
AIM: There are no beneficial therapies except for emergency liver transplantation for acute liver failure (ALF). However, in Japan where a serious problem of a shortage of donor livers still remains, therapies other than transplantation must be further investigated for patients with ALF. Pro-inflammatory cytokines promoting tissue destruction are predominant at an early phase of ALF. Corticosteroid (CS) influences monocyte/macrophage differentiation, by suppressing pro-inflammatory genes, indicating CS treatment might be beneficial during the early phase of ALF...
April 19, 2017: Hepatology Research: the Official Journal of the Japan Society of Hepatology
https://www.readbyqxmd.com/read/28421681/early-clinical-xenotransplantation-experiences-an-interview-with-thomas-e-starzl-md-phd
#16
REVIEW
David K C Cooper
Dr Thomas E. Starzl, who died on March 4, 2017, was one of the great pioneers of organ transplantation. He was also a pioneer in the field of xenotransplantation. In 1964, he carried out baboon kidney transplants in six patients with terminal renal disease for whom no living or deceased donor became available; graft survival was for 19-60 days, the grafts being lost largely through continuous complement activation. Between 1966 and 1974, he carried out one ex vivo liver perfusion and three orthotopic liver transplants using chimpanzees as sources of organs; graft survival was for <14 days...
March 2017: Xenotransplantation
https://www.readbyqxmd.com/read/28420441/autologous-bone-marrow-derived-cell-transplantation-in-decompensated-alcoholic-liver-disease-what-is-the-impact-on-liver-histology-and-gene-expression-patterns
#17
Nicolas Lanthier, Nathalie Lin-Marq, Laura Rubbia-Brandt, Sophie Clément, Nicolas Goossens, Laurent Spahr
BACKGROUND: Liver stem cell therapy (SCT) has been suggested as a promising means to improve liver regeneration in advanced liver disease. However, data from trials are heterogeneous, with no systematic histological evaluation. The aim of this study is to specifically analyze the effect of autologous SCT on liver regeneration and on gene expression changes. METHODS: Individuals in the randomized controlled trial of SCT in alcoholic hepatitis with paired liver biopsies were included (n = 58)...
April 18, 2017: Stem Cell Research & Therapy
https://www.readbyqxmd.com/read/28419938/direct-acting-antivirals-the-endgame-for-hepatitis-c
#18
REVIEW
Roberta D'Ambrosio, Elisabetta Degasperi, Massimo Colombo, Alessio Aghemo
Directly-acting antivirals (DAA) have finally allowed all patients to be potentially cured from chronic hepatitis C (HCV) infection. All-oral, Interferon (IFN)-free regimens are based upon the combination of molecules targeting different sites of the HCV replication process. Three classes of DAA exist: protease inhibitors (anti-NS3/4A), RNA-dependent polymerase inhibitors (anti-NS5B) and anti-NS5A inhibitors, which are characterized by different antiviral potency and barrier to resistance and therefore are usually combined in different treatment schedules...
April 15, 2017: Current Opinion in Virology
https://www.readbyqxmd.com/read/28418953/pulmonary-perfusion-scan-mimicking-hepatobiliary-scintigraphy-in-a-patient-with-an-uncommon-manifestation-of-granulomatosis-with-polyangiitis
#19
Michelle D Hopstone, Evan A Raff, Patrick M Colletti
A 36-year-old man with known granulomatosis with polyangiitis underwent quantitative ventilation-perfusion pulmonary scintigraphy as part of an initial evaluation for lung transplant candidacy. Radiotracer uptake on the perfusion study has the appearance of the hepatic silhouette, drawing initial concern that there may have been a misadministration of Tc with a hepatobiliary agent. Comparison CT reveals extensive cystic bronchiectasis, parenchymal destruction, and volume loss involving the entire left lung and basal portions of the right lung, with the residual portions of functional lung parenchyma conforming to a shape similar to the liver...
April 17, 2017: Clinical Nuclear Medicine
https://www.readbyqxmd.com/read/28416360/primary-hepatic-angiosarcoma-and-liver-transplantation-radiological-surgical-histological-findings-and-clinical-outcome
#20
M Tran Minh, A Mazzola, F Perdigao, F Charlotte, G Rousseau, F Conti
Angiosarcoma is a rare type of soft tissue sarcoma that accounts for less than 1% of all sarcomas and only 2% of all primary hepatic tumours. Thorotrast, arsenic, and vinyl chloride monomer are frequently listed as occupational exposure risks. The estimated latency is long (10-40 years) in occupational cases and very long (60 years or more) in non-occupational cases. The symptoms and CT-scan appearance of hepatic angiosarcoma (HAS) are non-specific. We present a case of a 65-year-old Caucasian male with history of cryptogenic cirrhosis, low alpha-foetoprotein levels and a single, 4-cm nodule of potential atypical hepatocellular carcinoma (no washout at MRI and CT-scan) in segment VIII...
April 14, 2017: Clinics and Research in Hepatology and Gastroenterology
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