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https://www.readbyqxmd.com/read/28335488/italian-registry-of-congenital-bleeding-disorders
#1
Adele Giampaolo, Francesca Abbonizio, Romano Arcieri, Hamisa Jane Hassan
In Italy, the surveillance of people with bleeding disorders is based on the National Registry of Congenital Coagulopathies (NRCC) managed by the Italian National Institute of Health (Istituto Superiore di Sanità). The NRCC collects epidemiological and therapeutic data from the 54 Hemophilia Treatment Centers, members of the Italian Association of Hemophilia Centres (AICE). The number of people identified with bleeding disorders has increased over the years, with the number rising from approx. 7000 in 2000 to over 11,000 in 2015...
March 19, 2017: Journal of Clinical Medicine
https://www.readbyqxmd.com/read/28335101/prevalence-and-incidence-of-depression-during-interferon-based-antiviral-therapy-in-chronic-hepatitis-c-patients-in-the-republic-of-korea
#2
Joo Yeong Baeg, In Hee Kim, Seung Young Seo, Young Seok Kim, Eun Uk Jung, Junhyeon Cho, Jung Wha Chung, Eun Sun Jang, Jin Wook Kim, Sook-Hyang Jeong
Background/Aims: The association between depression and chronic hepatitis C virus (HCV) infection or pegylated interferon α and ribavirin therapy (PR therapy) has not been extensively studied in Korea. We aimed to clarify the prevalence of depression and its incidence during PR therapy in chronic hepatitis C (CHC) patients. Methods: In this prospective, multicenter study, 114 CHC patients were screened for depression using two self-reported scales, the Beck Depression Inventory-I (BDI-I) and the Hospital Anxiety and Depression scale (HADS)...
March 24, 2017: Gut and Liver
https://www.readbyqxmd.com/read/28334808/downregulation-of-pathways-implicated-in-liver-inflammation-and-tumorigenesis-of-glycogen-storage-disease-type-ia-mice-receiving-gene-therapy
#3
Goo-Young Kim, Joon Hyun Kwon, Jun-Ho Cho, Lisa Zhang, Brian C Mansfield, Janice Y Chou
Glycogen storage disease type Ia (GSD-Ia) is characterized by impaired glucose homeostasis and long-term risks of hepatocellular adenoma (HCA) and carcinoma (HCC). We have shown that the non-tumor-bearing (NT), recombinant adeno-associated virus (rAAV) vector-treated GSD-Ia mice (AAV-NT mice) expressing a wide range (0.9-63%) of normal hepatic glucose-6-phosphatase-α activity maintain glucose homeostasis and display physiologic features mimicking animals living under calorie restriction (CR). We now show that in AAV-NT mice, the signaling pathways of the CR mediators, AMP-activated protein kinase (AMPK) and sirtuin-1 are activated...
March 13, 2017: Human Molecular Genetics
https://www.readbyqxmd.com/read/28334495/elbasvir-grazoprevir-does-not-worsen-renal-function-in-patients-with-hepatitis-c-virus-infection-and-pre-existing-renal-disease
#4
K Rajender Reddy, David Roth, Annette Bruchfeld, Peggy Hwang, Barbara Haber, Michael N Robertson, Eliav Barr, Wayne Greaves
AIM: Treatment options have been limited for patients with hepatitis C virus (HCV) infection and chronic kidney disease stage 4/5 (CKD 4/5). The aim of this analysis was to evaluate the impact of elbasvir/grazoprevir on estimated glomerular filtration rate (eGFR) in patients with CKD stage 3 enrolled in phase II/III clinical trials. METHODS: We conducted a retrospective integrated analysis of patients with CKD 3 enrolled in the EBR/GZR phase II/III clinical trials...
March 23, 2017: Hepatology Research: the Official Journal of the Japan Society of Hepatology
https://www.readbyqxmd.com/read/28333957/immune-response-of-rats-vaccinated-orally-with-various-plant-expressed-recombinant-cysteine-proteinase-constructs-when-challenged-with-fasciola-hepatica-metacercariae
#5
Malgorzata Kesik-Brodacka, Agnieszka Lipiec, Monika Kozak Ljunggren, Luiza Jedlina, Katarzyna Miedzinska, Magdalena Mikolajczak, Andrzej Plucienniczak, Andrzej B Legocki, Halina Wedrychowicz
BACKGROUND: Cysteine proteinases of Fasciola hepatica are important candidates for vaccine antigens because of their role in fluke biology and host-parasite relationships. In our previous experiments, we found that a recombinant cysteine proteinase cloned from adult F. hepatica (CPFhW) can protect rats against liver fluke infections when it is administered intramuscularly or intranasally in the form of cDNA. We also observed considerable protection upon challenge following mucosal vaccination with inclusion bodies containing recombinant CPFhW produced in Escherichia coli...
March 23, 2017: PLoS Neglected Tropical Diseases
https://www.readbyqxmd.com/read/28333769/the-incidence-of-low-seroimmunity-to-hepatitis-b-virus-in-children-with-inflammatory-bowel-disease
#6
Abhishek Watts, William E Bennett, Jean P Molleston, Sandeep K Gupta, Joseph M Croffie, Shamaila Waseem, Brian A McFerron, Steven J Steiner, Sanjay Kumar, Charles P Vanderpool, Emily C Hon, Molly A Bozic, Girish C Subbarao, Marian D Pfefferkorn
OBJECTIVES: Patients with inflammatory bowel disease (IBD) often receive immunosuppressive therapy which may make them vulnerable to infections such as hepatitis B. We hypothesized that hepatitis B virus (HBV) titers are low in the vaccinated pediatric IBD population. The aims of our study were to identify the incidence of lower titers of hepatitis B surface antibody (HBsAb) and determine which patient factors may be associated with lower HBsAb titers. METHODS: Patients with diagnosis of IBD, ages 5-18 years, were prospectively enrolled...
March 22, 2017: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/28333153/a-novel-inhibitor-idpp-interferes-with-entry-and-egress-of-hcv-by-targeting-glycoprotein-e1-in-a-genotype-specific-manner
#7
Myungeun Lee, Jaewon Yang, Eunji Jo, Ji-Young Lee, Hee-Young Kim, Ralf Bartenschlager, Eui-Cheol Shin, Yong-Soo Bae, Marc P Windisch
Despite recent advances in curing chronic hepatitis C (CHC), the high economic burden to therapy, viral drug resistance, difficult to treat hepatitis C virus (HCV) genotypes and patient groups are still of concern. To address this unmet medical needs, we devised strategies to identify novel viral interventions through target-free high-throughput screening of small molecules utilizing a phenotypic-based HCV infection assay. Thereby, a very potent (EC50 46 ± 26 pM) iminodipyridinopyrimidine (IDPP) drug candidate was selected, and confirmed in primary human hepatocytes (EC50 0...
March 23, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28332729/transplantation-of-kidneys-from-hepatitis-c-positive-donors-into-hepatitis-c-virus-infected-recipients-followed-by-early-initiation-of-direct-acting-antiviral-therapy-a-single-center-retrospective-study
#8
Kalyan Ram Bhamidimarri, Marco Ladino, Fernando Pedraza, Giselle Guerra, Adela Mattiazzi, Linda Chen, Gaetano Ciancio, Warren Kupin, Paul Martin, George Burke, David Roth
The availability of direct acting antiviral agents (DAA) has transformed the treatment of hepatitis C virus (HCV) infection. The current study is a case series that reports the outcomes from a cohort of twenty-five HCV-infected ESRD patients who received a kidney from an anti-HCV-positive deceased organ donor followed by treatment with DAAs in the early post-transplant period. Time to transplantation and the efficacy of DAA therapy as measured by sustained viral response at 12 weeks were assessed. The median waiting time from original date of activation on the UNOS waiting list until transplantation was 427 days, however, the median time from entering the patient into UNet(sm) for a HCV positive offer until transplantation was only 58 days...
March 23, 2017: Transplant International: Official Journal of the European Society for Organ Transplantation
https://www.readbyqxmd.com/read/28332476/new-onset-hepatitis-c-virus-associated-glomerulonephritis-following-sustained-virologic-response-with-direct-acting-antiviral-therapy%C3%A2
#9
Muriel Ghosn, Matthew B Palmer, Catherine E Najem, Danny Haddad, Peter A Merkel, Jonathan J Hogan
Glomerulonephritis (GN) is an important extra-hepatic manifestation of infection with hepatitis C virus (HCV). HCV-associated GN occurs due to HCV-induced lymphoproliferation, leading to the generation of pathogenic immune complexes, including complexes containing cryoglobulins. The management of HCV-associated extra-hepatic disease is focused on viral eradication, with direct-acting antiviral agents leading to high rates of sustained virologic remission. There have been a few reports of relapsing cryoglobulinemic vasculitis after sustained virologic remission was achieved with interferon-based therapies...
March 23, 2017: Clinical Nephrology
https://www.readbyqxmd.com/read/28332314/the-mitochondrial-hinge-protein-uqcrh-is-a-novel-prognostic-factor-for-hepatocellular-carcinoma
#10
Eun-Ran Park, Sang-Bum Kim, Jee-San Lee, Yang-Hyun Kim, Dong-Hyoung Lee, Eung-Ho Cho, Sun-Hoo Park, Chul Ju Han, Bu-Yeo Kim, Dong Wook Choi, Young Do Yoo, Ami Yu, Jae Won Lee, Ja June Jang, Young Nyun Park, Kyung-Suk Suh, Kee-Ho Lee
Alterations in mitochondrial respiration contribute to the development and progression of cancer via abnormal biogenesis, including generation of reactive oxygen species. Ubiquinol-cytochrome c reductase hinge protein (UQCRH) consists of the cytochrome bc1 complex serving respiration in mitochondria. In the present study, we analyzed UQCRH abnormalities in hepatocellular carcinoma (HCC) and its association with clinical outcomes of patients. UQCRH expression in HCC was determined via semiquantitative and quantitative real-time reverse transcriptase polymerase chain reaction of 96 surgically resected HCC tissues positive for hepatitis B virus surface antigen...
March 23, 2017: Cancer Medicine
https://www.readbyqxmd.com/read/28332306/impact-of-iatrogenic-iron-overload-on-the-course-of-hepatitis-c-in-dialysis-population-a-plea-for-caution
#11
Guy Rostoker, Nosratola D Vaziri
About 2.5% of the world population, corresponding to about 177 million individuals, are infected by hepatitis C virus (HCV), a small, single-stranded RNA virus. The prevalence of HCV infection among dialysis patients in Japan, Europe, and North America during the 2012 to 2015 period was found to be 8.7% in the DOPPS study. Nosocomial HCV spread in hemodialysis facilities still occurs. Increased hepatic tissue iron has been shown to play a deleterious role in the course of hepatitis C, favor development of fibrosis and cirrhosis and possibly increase the risk of liver cancer in the general population...
March 23, 2017: Hemodialysis International
https://www.readbyqxmd.com/read/28332273/reducing-the-incidence-of-hepatic-artery-thrombosis-in-pediatric-liver-transplantation-effect-of-microvascular-techniques-and-a-customized-anticoagulation-protocol
#12
William A Ziaziaris, Alexandre Darani, Andrew J A Holland, Angus Alexander, Jonathan Karpelowsky, Pasquale Barbaro, Michael Stormon, Edward O'Loughlin, Albert Shun, Gordon Thomas
We aimed to assess the incidence of HAT over three eras following implementation of microvascular techniques and a customized anticoagulation protocol in a predominantly cadaveric split liver transplant program. We retrospectively reviewed pediatric liver transplants performed between April 1986 and 2016 and analyzed the incidence HAT over three eras. In E1, 1986-2008, each patient received a standard dose of 5 U/kg/h of heparin and coagulation profiles normalized passively. In E2, 2008-2012, microvascular techniques were introduced...
March 22, 2017: Pediatric Transplantation
https://www.readbyqxmd.com/read/28332272/clinical-evaluation-of-sofosbuvir-ledipasvir-in-chronic-hepatitis-c-genotype-1-with-and-without-prior-daclatasvir-asnaprevir-therapy
#13
Etsuko Iio, Noritomo Shimada, Koichi Takaguchi, Tomonori Senoh, Yuichiro Eguchi, Masanori Atsukawa, Akihito Tsubota, Hiroshi Abe, Keizo Kato, Atsunori Kusakabe, Tomokatsu Miyaki, Kentaro Matsuura, Kayoko Matsunami, Noboru Shinkai, Kei Fujiwara, Shunsuke Nojiri, Yasuhito Tanaka
AIM: This study explored treatment outcomes of sofosbuvir (SOF)/ledipasvir (LDV) therapy for chronic hepatitis C patients with and without prior daclatasvir (DCV)/asunaprevir (ASV) therapy. METHODS: Overall, 530 Japanese patients who were infected with hepatitis C virus genotype 1 received SOF/LDV therapy for 12 weeks, and resistance-associated variants (RAVs) in the HCV NS5A and NS5B regions were assessed at baseline and virological relapse by direct sequencing...
March 22, 2017: Hepatology Research: the Official Journal of the Japan Society of Hepatology
https://www.readbyqxmd.com/read/28332201/identification-of-novel-variants-in-hla-class-ii-region-related-to-hla-dpb1-expression-and-disease-progression-in-patients-with-chronic-hepatitis-c
#14
Katsushi Hiramatsu, Hidetaka Matsuda, Tomoyuki Nemoto, Takuto Nosaka, Yasushi Saito, Tatsushi Naito, Kazuto Takahashi, Kazuya Ofuji, Masahiro Ohtani, Hiroyuki Suto, Toshihiro Yasuda, Yukio Hida, Hideki Kimura, Yoshihiro Soya, Yasunari Nakamoto
BACKGROUND: Recent genome-wide studies have demonstrated that HLA class II gene may play an important role in viral hepatitis. We studied genetic polymorphism and RNA expression of HLA class II genes in HCV-related liver diseases. METHODS: The study was performed in groups consisting of 24 patients with HCV-related liver disease (12 of persistent normal ALT: PNALT group and 12 of advanced liver disease: ALD group) and 26 patients without HCV infection (control group)...
March 22, 2017: Journal of Medical Virology
https://www.readbyqxmd.com/read/28331967/decreased-basal-hepatic-glucose-uptake-in-impaired-fasting-glucose
#15
Mariam Alatrach, Christina Agyin, John Adams, Ralph A DeFronzo, Muhammad A Abdul-Ghani
AIMS/HYPOTHESIS: This research aimed to define the pathophysiological defects responsible for the elevated fasting plasma glucose (FPG) concentration and excessive rise in post-load plasma glucose observed in individuals with impaired fasting glucose (IFG). METHODS: We used tracer techniques to quantify basal splanchnic (primarily hepatic) glucose uptake and glucose fluxes following glucose ingestion in individuals with normal glucose tolerance (NGT; n = 10) and IFG (n = 10)...
March 22, 2017: Diabetologia
https://www.readbyqxmd.com/read/28331841/resistance-testing-for-the-treatment-of-chronic-hepatitis-c-with-direct-acting-antivirals-when-and-for-how-long
#16
Ana Belén Pérez, Natalia Chueca, Federico García
The need to test for resistance associated substitutions (RAS) has been intensively debated in the past two years. In the absence of pangenotypic combinations, it seems reasonable that, if available, RAS testing in the NS5A gene at baseline for genotypes 1a and 3 may help to avoid overtreatment in terms of ribavirin usage and/or prolonged treatment duration. When patients fail treatment, RAS testing may also be useful to guide the selection of the new regimen, especially for those that need urgent retreatment and that have failed a combination including an NS5A inhibitor...
March 2017: Germs
https://www.readbyqxmd.com/read/28331419/falsely-elevated-serum-vitamin-b12-levels-were-associated-with-the-severity-and-prognosis-of-chronic-viral-liver-disease
#17
Takaaki Sugihara, Masahiko Koda, Toshiaki Okamoto, Kenichi Miyoshi, Tomomitsu Matono, Kenji Oyama, Keiko Hosho, Jun-Ichi Okano, Hajime Isomoto, Yoshikazu Murawaki
BACKGROUND: Vitamin B12 is stored primarily in the liver, and highly elevated serum vitamin B12 levels occur in acute hepatitis and severe alcoholic liver disease. We evaluated the relationship between vitamin B12 levels and liver disease severity and long term prognosis in patients with chronic viral hepatitis and cirrhosis. METHODS: We enrolled 90 patients (57 men, 33 women) with chronic viral hepatitis and cirrhosis who admitted to our hospital as a prospective cohort study...
March 2017: Yonago Acta Medica
https://www.readbyqxmd.com/read/28331190/natural-killer-cell-activation-contributes-to-hepatitis-b-viral-control-in-a-mouse-model
#18
Shiwen Tong, Guangze Liu, Minghong Li, Xiumei Li, Qian Liu, Hong Peng, Shiying Li, Hong Ren, Wenwei Yin
The roles of CD4 + T cells and CD8 + T cells in hepatitis B virus (HBV) infection have been well documented. However, the role of innate immunity in HBV infection remains obscure. Here we examined the effect of activation of innate immunity by polyinosinic: polycytidylic acid (PolyI:C) on HBV infection. A chronic HBV replication mouse model was established by hydrodynamical injection of pAAV/HBV1.2 plasmid into C57BL/6 mice. We found that HBV did not seem to induce an active NK-cell response in the mouse model...
March 22, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28330995/hepatic-fcrn-regulates-albumin-homeostasis-and-susceptibility-to-liver-injury
#19
Michal Pyzik, Timo Rath, Timothy T Kuo, Sanda Win, Kristi Baker, Jonathan J Hubbard, Rosa Grenha, Amit Gandhi, Thomas D Krämer, Adam R Mezo, Zachary S Taylor, Kevin McDonnell, Vicki Nienaber, Jan Terje Andersen, Atsushi Mizoguchi, Laurence Blumberg, Shalaka Purohit, Susan D Jones, Greg Christianson, Wayne I Lencer, Inger Sandlie, Neil Kaplowitz, Derry C Roopenian, Richard S Blumberg
The neonatal crystallizable fragment receptor (FcRn) is responsible for maintaining the long half-life and high levels of the two most abundant circulating proteins, albumin and IgG. In the latter case, the protective mechanism derives from FcRn binding to IgG in the weakly acidic environment contained within endosomes of hematopoietic and parenchymal cells, whereupon IgG is diverted from degradation in lysosomes and is recycled. The cellular location and mechanism by which FcRn protects albumin are partially understood...
March 22, 2017: Proceedings of the National Academy of Sciences of the United States of America
https://www.readbyqxmd.com/read/28330813/atp-binding-cassette-transporter-a1-deficiency-in-human-induced-pluripotent-stem-cell-derived-hepatocytes-abrogates-hdl-biogenesis-and-enhances-triglyceride-secretion
#20
Xin Bi, Evanthia E Pashos, Marina Cuchel, Nicholas N Lyssenko, Mayda Hernandez, Antonino Picataggi, James McParland, Wenli Yang, Ying Liu, Ruilan Yan, Christopher Yu, Stephanie L DerOhannessian, Michael C Phillips, Edward E Morrisey, Stephen A Duncan, Daniel J Rader
Despite the recognized role of the ATP-binding Cassette Transporter A1 (ABCA1) in high-density lipoprotein (HDL) metabolism, our understanding of ABCA1 deficiency in human hepatocytes is limited. To define the functional effects of human hepatocyte ABCA1 deficiency, we generated induced pluripotent stem cell (iPSC)-derived hepatocyte-like cells (HLCs) from Tangier disease (TD) and matched control subjects. Control HLCs exhibited robust cholesterol efflux to apolipoprotein A-I (apoA-I) and formed nascent HDL particles...
March 14, 2017: EBioMedicine
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