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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/29147012/piperidylmethyloxychalcone-improves-immune-mediated-acute-liver-failure-via-inhibiting-tak1-activity
#2
Sun Hong Park, Jeong-Ah Kwak, Sang-Hun Jung, Byeongwoo Ahn, Won-Jea Cho, Cheong-Yong Yun, Chang Seon Na, Bang Yeon Hwang, Jin Tae Hong, Sang-Bae Han, Youngsoo Kim
Mice deficient in the toll-like receptor (TLR) or the myeloid differentiation factor 88 (MyD88) are resistant to acute liver failure (ALF) with sudden death of hepatocytes. Chalcone derivatives from medicinal plants protect from hepatic damages including ALF, but their mechanisms remain to be clarified. Here, we focused on molecular basis of piperidylmethyloxychalcone (PMOC) in the treatment of TLR/MyD88-associated ALF. C57BL/6J mice were sensitized with D-galactosamine (GalN) and challenged with Escherichia coli lipopolysaccharide (LPS, TLR4 agonist) or oligodeoxynucleotide containing unmethylated CpG motif (CpG ODN, TLR9 agonist) for induction of ALF...
November 17, 2017: Experimental & Molecular Medicine
https://www.readbyqxmd.com/read/29142540/clinical-audit-of-dengue-related-deaths-in-2011-mayo-hospital-lahore-pakistan
#3
Somia Iqtadar, Nabeel Akbar, Mehreen Mehmood, Sajid Abaidullah
Background and Objective: Dengue infection has evolved into an epidemic during last few years in Pakistan and has been associated with poor outcomes. Literature with respect to mortality risk factors in Dengue infection is not sufficient. This compelled us to conduct this study to find out major contributory factors to death in patients with dengue viral infection at one of Asia's ancient hospital setting with an aim to recognize complications at earliest and improve case management in future...
September 2017: Pakistan Journal of Medical Sciences Quarterly
https://www.readbyqxmd.com/read/29142481/rescue-case-of-low-birth-weight-infant-with-acute-hepatic-failure
#4
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/29142477/modified-model-for-end-stage-liver-disease-improves-short-term-prognosis-of-hepatitis-b-virus-related-acute-on-chronic-liver-failure
#5
Wei Chen, Jia You, Jing Chen, Qi Zheng, Jia-Ji Jiang, Yue-Yong Zhu
AIM: To investigate whether the short-term prognosis of hepatitis B virus (HBV)-related acute-on-chronic liver failure (ACLF) could be improved by using a modified model for end-stage liver disease (MELD) including serum lactate. METHODS: This clinical study was conducted at the First Affiliated Hospital, Fujian Medicine University, China. From 2009 to 2015, 236 patients diagnosed with HBV-related ACLF at our center were recruited for this 3-month follow-up study...
October 28, 2017: World Journal of Gastroenterology: WJG
https://www.readbyqxmd.com/read/29141664/prevalence-and-prognosis-of-acutely-ill-patients-with-organ-failure-at-arrival-to-hospital-protocol-for-a-systematic-review
#6
Peter Bank Pedersen, Asbjorn Hrobjartsson, Daniel Lykke Nielsen, Daniel Pilsgaard Henriksen, Mikkel Brabrand, Annmarie Touborg Lassen
BACKGROUND: Acutely ill patients are a heterogeneous group, and some of these suffer from organ failure. As the prognosis of organ failure improves with early treatment, it is important to identify these patients as early as possible. Most studies on organ failure have been performed in intensive care settings, or on selected groups of patients, where a high prevalence and mortality have been reported. Before patients arrive to the intensive care unit, or the general ward, most of them have passed through the emergency department (ED), where diagnosis and treatment has been initiated...
November 15, 2017: Systematic Reviews
https://www.readbyqxmd.com/read/29135887/ischemia-reperfusion-injury-in-aged-livers-the-energy-metabolism-inflammatory-response-and-autophagy
#7
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/29132195/current-evidence-for-the-use-of-n-acetylcysteine-following-liver-resection
#8
REVIEW
Richard Kemp, Jonathan Mole, Dhanny Gomez
BACKGROUND: N-acetylcysteine (NAC) has many uses in medicine; notable in the management of paracetamol toxicity, acute liver failure and liver surgery. The aim of this review was to critically appraise the published literature for the routine use of NAC in liver resection surgery. METHODS: An electronic search was performed of EBSCOhost (Medline and CINAHL database), PubMed and the Cochrane Library for the period 1990-2016. MeSH headings: 'acetyl-cysteine', 'liver resection' and 'hepatectomy' were used to identify all relevant articles published in English...
November 13, 2017: ANZ Journal of Surgery
https://www.readbyqxmd.com/read/29127519/neutrophil-biology-within-hepatic-environment
#9
REVIEW
Débora Moreira Alvarenga, Matheus Silvério Mattos, Alan Moreira Araújo, Maísa Mota Antunes, Gustavo Batista Menezes
Neutrophils are the most abundant leukocyte in the human circulation. These short-lived cells are constantly produced from hematopoietic stem cells (HSC) within the bone marrow from which they daily reach the blood and perform major roles in innate immunity. Neutrophils are the first cells to reach inflamed tissues and are armed with a plethora of enzymes that help both with their trafficking within tissues and the killing of pathogens. Damaged or infected organs are rapidly invaded by neutrophils. Their erroneous activation within parenchyma or the vasculature is involved in the pathogenesis of several inflammatory diseases including arthritis, colitis, sepsis, acute lung injury and liver failure...
November 10, 2017: Cell and Tissue Research
https://www.readbyqxmd.com/read/29118875/a-fatal-case-of-intra-abdominal-hemorrhage-following-diagnostic-blind-percutaneous-liver-biopsy-in-a-patient-with-peliosis-hepatis
#10
Saptarshi Biswas, Shekhar Gogna, Prem Patel
Peliosis hepatis (PH) is a rare vascular condition of the liver characterized by the presence of cystic blood filled cavities distributed randomly throughout the liver parenchyma. We describe a case of a 42-year-old previously healthy male patient, airlifted to us in a state of hemorrhagic shock after undergoing percutaneous diagnostic liver biopsy for lesions seen on CT scan. Repeat CT scan with IV contrast on presentation in our hospital showed intraperitoneal bleeding. Hepatic angiography failed to identify any specific bleeding source...
October 2017: Gastroenterology Research
https://www.readbyqxmd.com/read/29118330/molecular-spectrum-of-secretome-regulates-the-relative-hepatogenic-potential-of-mesenchymal-stem-cells-from-bone-marrow-and-dental-tissue
#11
Ajay Kumar, Vinod Kumar, Vidya Rattan, Vivekananda Jha, Arnab Pal, Shalmoli Bhattacharyya
Liver regeneration is a spontaneous process that occurs after liver injury, but acute liver failure is a complex and fatal disease which is difficult to treat. Cell-based therapies are promising alternative therapeutic approach for liver failure and different cell sources have been tested in this regard. We investigated the comparative hepatogenic potential of human bone marrow stem cells (BMSC) with stem cells derived from human dental pulp (DPSC), apical papilla (SCAP) and follicle (DFSC) during this study...
November 8, 2017: Scientific Reports
https://www.readbyqxmd.com/read/29115798/acute-alcoholic-hepatitis-a-literature-review-and-proposal-of-treatment
#12
Gianni Testino, Silvia Leone
Severe acute alcoholic hepatitis (AAH) can lead to a clinical picture with a sixmonth mortality rate in more than 70% of cases. This clinical picture is characterized by: jaundice with a duration of less than three months, jaundice at the first failure event, serum bilirubin greater than 5 mg / dL, ratio AST / ALT> 2: 1, AST less than 500 IU / L, ALT <300 IU / L, neutrophil leukocytosis and a GGT increase. In addition, encephalopathy, fever, asthenia and coagulopathy may be present. Its onset may also be characterized by portalhypertensionrelated complications, particularly bleeding and hepatorenal syndrome...
November 7, 2017: Minerva Medica
https://www.readbyqxmd.com/read/29113427/-clinical-application-of-mesenchymal-stem-cells-in-treatment-of-acute-on-chronic-liver-failure-and-related-research-advances
#13
L Y Feng, D Z Zhang
Acute-on-chronic liver failure is a syndrome characterized by acute exacerbation of chronic hepatitis, organ failure, and high mortality. Clinical treatment of acute-on-chronic liver failure included comprehensive medical treatment, artificial liver support system, and liver transplantation, but such methods have their own shortcomings and patients tend to have a poor prognosis. Mesenchymal stem cells (MSCs), as a new type of cell therapy, have wide sources and are easy to extract and culture. Many studies have shown that MSC treatment not only helps to achieve a high survival rate, but also has good tolerability and safety; therefore, the clinical value of MSCs has become a hot research topic...
September 20, 2017: Zhonghua Gan Zang Bing za Zhi, Zhonghua Ganzangbing Zazhi, Chinese Journal of Hepatology
https://www.readbyqxmd.com/read/29113002/-acute-liver-failure-as-a-rare-case-of-a-frequent-disease
#14
Angela Marie Thiel, Jürgen Rissland, Frank Lammert, Markus Casper
Here we report the case of a 63-year-old female patient who was admitted with acute liver failure. The etiology turned out to be an acute fulminant hepatitis caused by hepatitis C virus (HCV) infection of unknown origin. Fulminant hepatitis due to acute hepatitis C is very rare and hardly reported but a potential cause of acute liver failure due to massive hepatic necrosis, which may lead to encephalitis and multi-organ-failure. During follow-up spontaneous virological clearance was observed. The case illustrates that acute HCV infection, which is confirmed by the detection of HCV-RNA, has to be considered in the differential diagnosis of acute liver failure...
November 7, 2017: Zeitschrift Für Gastroenterologie
https://www.readbyqxmd.com/read/29112564/a-surgical-clostridium-associated-risk-of-death-score-predicts-mortality-after-colectomy-for-clostridium-difficile
#15
Audrey S Kulaylat, Zain Kassam, Christopher S Hollenbeak, David B Stewart
BACKGROUND: A Clostridium difficile-associated risk of death score was recently developed and validated by using a national cohort of both nonsurgical and surgical patients admitted with C difficile infection. However, risk scores specifically derived from surgical cohorts and designed for patients with C difficile infection are currently unavailable. OBJECTIVE: The aim of this study was to develop a risk of death score for patients with C difficile infection who are being considered for total abdominal colectomy because of the failure of medical therapy...
December 2017: Diseases of the Colon and Rectum
https://www.readbyqxmd.com/read/29108199/-value-of-neutrophil-lymphocyte-ratio-in-predicting-hepatitis-b-related-liver-failure
#16
Y Fan, X Li, X F Zhou, D Z Zhang, X F Shi
Objective: To investigate the value of neutrophil-lymphocyte ratio (NLR) in predicting hepatitis B-related liver failure. Methods: A total of 349 subjects were enrolled, among whom 60 were healthy persons who underwent physical examination (group A), 111 had severe chronic hepatitis B (group B), 92 had decompensated hepatitis B cirrhosis (group C), and 86 had acute-on-chronic liver failure (ACLF) (group D). Routine blood test results, liver function parameters, and coagulation parameters were collected, and NLR was calculated...
October 20, 2017: Zhonghua Gan Zang Bing za Zhi, Zhonghua Ganzangbing Zazhi, Chinese Journal of Hepatology
https://www.readbyqxmd.com/read/29108122/pyroptosis-by-caspase11-4-gasdermin-d-pathway-in-alcoholic-hepatitis
#17
Elena Khanova, Raymond Wu, Wen Wang, Rui Yan, Yibu Chen, Samuel W French, Cristina Llorente, Stephanie Q Pan, Qihong Yang, Yuchang Li, Raul Lazaro, Charles Ansong, Richard D Smith, Ramon Bataller, Timothy Morgan, Bernd Schnabl, Hidekazu Tsukamoto
Alcoholic hepatitis (AH) continues to be a disease with high mortality and no efficacious medical treatment. Although severe AH is presented as acute on chronic liver failure, what underlies this transition from chronic alcoholic steatohepatitis (ASH) to AH, is largely unknown. To address this question, unbiased RNA-seq and proteomic analyses were performed on livers of the recently developed AH mouse model which exhibits the shift to AH from chronic ASH upon weekly alcohol binge, and these results are compared with gene expression profiling data from AH patients...
November 6, 2017: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
https://www.readbyqxmd.com/read/29107739/comparative-restoration-of-acute-liver-failure-by-menstrual-blood-stem-cells-compared-with-bone-marrow-stem-cells-in-mice-model
#18
Mina Fathi-Kazerooni, Gholamreza Tavoosidana, Masoud Taghizadeh-Jahed, Sayeh Khanjani, Hananeh Golshahi, Caroline E Gargett, Haleh Edalatkhah, Somaieh Kazemnejad
BACKGROUND AIMS: The application of menstrual blood stem cells (MenSCs) in regenerative medicine is gaining increasing attention. The aim of this study was to investigate the therapeutic potential of MenSCs compared with bone marrow-derived stem cells (BMSCs) in an animal model of CCl4-induced acute hepatic failure. METHODS: Injured Balb/C mice were divided into multiple groups and received MenSCs, BMSCs or hepatocyte progenitor-like (HPL) cells derived from these cells...
October 26, 2017: Cytotherapy
https://www.readbyqxmd.com/read/29105936/circulating-cxcl10-in-cirrhotic-portal-hypertension-might-reflect-systemic-inflammation-and-predict-aclf-and-mortality
#19
Jennifer M Lehmann, Karina Claus, Christian Jansen, Alessandra Pohlmann, Robert Schierwagen, Carsten Meyer, Daniel Thomas, Steffen Manekeller, Joan Claria, Christian P Strassburg, Christian Trautwein, Hermann E Wasmuth, Marie-Luise Berres, Jonel Trebicka
BACKGROUND & AIMS: CXCR% ligands play an important role in hepatic injury, inflammation and fibrosis. While CXCL9 and CXCL11 are associated with survival in patients receiving transjugular intrahepatic portosystemic shunt (TIPS), the role of CXCL10 in severe portal hypertension remains unknown. METHODS: A total of 89 cirrhotic patients were analysed. CXCL10 protein levels were measured in portal and hepatic blood at TIPS insertion and 2 weeks later in 24 patients...
November 6, 2017: Liver International: Official Journal of the International Association for the Study of the Liver
https://www.readbyqxmd.com/read/29104502/mesenchymal-stem-cell-dependent-modulation-of-liver-diseases
#20
REVIEW
Marina Gazdic, Aleksandar Arsenijevic, Bojana Simovic Markovic, Ana Volarevic, Ivanka Dimova, Valentin Djonov, Nebojsa Arsenijevic, Miodrag Stojkovic, Vladislav Volarevic
Acute liver failure and cirrhosis display sequential and overlapping severe pathogenic processes that include inflammation, hepatocyte necrosis, and fibrosis, carrying a high mortality rate. Mesenchymal stem cells (MSCs) are a heterogeneous subset of stromal stem cells with immunonodulatory characteristics. MSCs are considered to act through multiple mechanisms to coordinate a dynamic, integrated response to liver inflammation and fibrosis, which prevents the progressive distortion of hepatic architecture. Accordingly, MSCs as well as their products have been investigated as a novel therapeutic approach for the treatment of inflammatory and fibrotic liver diseases...
2017: International Journal of Biological Sciences
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