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Coagulation in liver disease

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https://www.readbyqxmd.com/read/29766340/the-cholic-acid-extension-study-in-zellweger-spectrum-disorders-results-and-implications-for-therapy
#1
Femke C C Klouwer, Bart G P Koot, Kevin Berendse, Elles M Kemper, Sacha Ferdinandusse, Kiran V K Koelfat, Martin Lenicek, Frédéric M Vaz, Marc Engelen, Peter L M Jansen, Ronald J A Wanders, Hans R Waterham, Frank G Schaap, Bwee Tien Poll-The
INTRODUCTION: Currently, no therapies are available for Zellweger spectrum disorders (ZSDs), a group of genetic metabolic disorders characterised by a deficiency of functional peroxisomes. In a previous study, we showed that oral cholic acid (CA) treatment can suppress bile acid synthesis in ZSD patients and, thereby, decrease plasma levels of toxic C27 -bile acid intermediates, one of the biochemical abnormalities in these patients. However, no effect on clinically relevant outcome measures could be observed after 9 months of CA treatment...
May 15, 2018: Journal of Inherited Metabolic Disease
https://www.readbyqxmd.com/read/29761173/liver-enriched-transcription-factor-expression-relates-to-chronic-hepatic-failure-in-humans
#2
Jorge Guzman-Lepe, Eduardo Cervantes-Alvarez, Alexandra Collin de l'Hortet, Yang Wang, Wendy M Mars, Yoshinao Oda, Yuki Bekki, Masahiro Shimokawa, Huanlin Wang, Tomoharu Yoshizumi, Yoshihiko Maehara, Aaron Bell, Ira J Fox, Kazuki Takeishi, Alejandro Soto-Gutierrez
The mechanisms by which the liver fails in end-stage liver disease remain elusive. Disruption of the transcription factor network in hepatocytes has been suggested to mediate terminal liver failure in animals. However, this hypothesis remains unexplored in human subjects. To study the relevance of transcription factor expression in terminal stages of chronic liver failure in humans, we analyzed the expression of liver-enriched transcription factors (LETFs) hepatocyte nuclear factor (HNF)4α, HNF1α, forkhead box protein A2 (FOXA2), CCAAT/enhancer-binding protein (CEBP)α, and CEBPβ...
May 2018: Hepatology Communications
https://www.readbyqxmd.com/read/29758989/the-double-hazard-of-bleeding-and-thrombosis-in-hemostasis-from-a-clinical-point-of-view-a-global-assessment-by-rotational-thromboelastometry-rotem
#3
Olga Meltem Akay
Hemostasis is a complex dynamic process involving bleeding and thrombosis as two end-points. Conventional coagulation tests which are measured in plasma examine only isolated portions of the coagulation cascade, thereby giving no information on important interactions essential to the clinical evaluation of hemostatic function. Thromboelastography (TEG), originally described in 1948 has improved over the decades and become a valuable tool of coagulation testing because of the limitations of standard coagulation tests...
January 1, 2018: Clinical and Applied Thrombosis/hemostasis
https://www.readbyqxmd.com/read/29739078/review-on-vitamin-k-deficiency-and-its-biomarkers-focus-on-the-novel-application-of-pivka-ii-in-clinical-practice
#4
REVIEW
Rui Dong, Nianyue Wang, Yongfeng Yang, Li Ma, Qiang Du, Wei Zhang, Anh H Tran, Haiyoung Jung, Andrew Soh, Yijie Zheng, Shan Zheng
BACKGROUND: Vitamin K (VK) is a co-factor of the γ-glutamyl carboxylase that catalyzes the conversion of glutamate residues to γ-carboxyglutamate in VK-dependent proteins. The carboxylation reaction imparts the essential calcium-binding residues for the biological function of several proteins involved in the process of coagulation and bone metabolism. VK deficiency is frequently encountered in newborns and can lead to fatal hemorrhagic complications. This review describes and discusses the clinical application of VK deficiency testing...
April 1, 2018: Clinical Laboratory
https://www.readbyqxmd.com/read/29737459/safety-of-intra-articular-transplantation-of-lentivirally-transduced-mesenchymal-stromal-cells-for-haemophilic-arthropathy-in-a-non-human-primate
#5
Tsukasa Ohmori, Hiroaki Mizukami, Yuko Katakai, Sho Kawai, Hitoyasu Nakamura, Makoto Inoue, Tsugumine Shu, Hideharu Sugimoto, Yoichi Sakata
Joint bleeding and resultant arthropathy are major determinants of quality of life in haemophilia patients. We previously developed a mesenchymal stromal cell (MSC)-based treatment approach for haemophilic arthropathy in a mouse model of haemophilia A. Here, we evaluated the long-term safety of intra-articular injection of lentivirally transduced autologous MSCs in non-human primates. Autologous bone-marrow-derived MSCs transduced with a lentiviral vector expressing coagulation factor VIII (FVIII) were injected into the left knee joint of cynomolgus monkeys...
May 8, 2018: International Journal of Hematology
https://www.readbyqxmd.com/read/29723040/role-of-the-blood-coagulation-cascade-in-hepatic-fibrosis
#6
Asmita Pant, Anna K Kopec, James P Luyendyk
Liver is the primary source of numerous proteins critical for normal function of the blood coagulation cascade. Because of this, diseases of the liver, particularly when affiliated with severe complications like cirrhosis, are associated with abnormalities of blood clotting. Although conventional interpretation has inferred cirrhosis as a disorder of uniform bleeding risk, it is now increasingly appreciated as a disease wherein the coagulation cascade is precariously rebalanced. Moreover, prothrombotic risk factors are also associated with a more rapid progression of fibrosis in humans, suggesting that coagulation proteases participate in disease pathogenesis...
May 3, 2018: American Journal of Physiology. Gastrointestinal and Liver Physiology
https://www.readbyqxmd.com/read/29707035/treatment-of-danhong-injection-combined-with-naoxintong-capsule-in-acute-coronary-syndrome-patients-undergoing-pci-operation-study-for-a-randomized-controlled-and-double-blind-trial
#7
Shuai Zhao, Yong Tang, Hairong Cai, Weifeng Liu, Lieyuan Zhang, Dongjie Chen, Bojun Chen
Objective: This double-blind and randomized placebo-controlled trial evaluated the safety and efficacy of Danhong injection combined with Naoxintong capsule in patients with acute coronary syndrome (ACS) after percutaneous coronary intervention (PCI). Methods: ACS patients scheduled to undergo PCI ( n = 130) were equally and randomly apportioned to either a treatment or control group. After PCI, the treatment group received Danhong injection combined with Naoxintong capsule for 12 weeks, while the control group was given placebo...
2018: Evidence-based Complementary and Alternative Medicine: ECAM
https://www.readbyqxmd.com/read/29699571/safety-and-efficacy-of-dehydrated-ethanol-soaking-of-the-operative-field-in-the-treatment-of-spontaneous-hepatocellular-carcinoma-rupture
#8
Jian Sun, Yue Zhu, Yao-Rong Peng, Wen-Bin Li, He-Yun Zhang, Zhen-Yu Zhou, Lin Wang, An-de Ma, Jie Wang
BACKGROUND: The aim of our study was to evaluate the clinical safety and value of ethanol surgical field infiltration (ESFI), combined with distilled water peritoneal lavage (DWPL), after hepatectomy in patients with hepatocellular carcinoma (HCC) rupture. METHODS: Rat liver tissue samples were soaked in dehydrated ethanol for different soaking times, and 18 rats were assigned to three groups that underwent different soaking methods of the hepatectomy cut surface...
April 26, 2018: World Journal of Surgical Oncology
https://www.readbyqxmd.com/read/29685474/the-evaluation-and-management-of-rocky-mountain-spotted-fever-in-the-emergency-department-a-review-of-the-literature
#9
Michael Gottlieb, Brit Long, Alex Koyfman
BACKGROUND: Rocky Mountain spotted fever (RMSF) is potentially deadly and can present subtly with signs and symptoms overlapping with other clinical conditions. Delayed diagnosis can be fatal. OBJECTIVE: This review provides an evidence-based summary of the current data for the evaluation and management of RMSF in the emergency department. DISCUSSION: RMSF occurs through transmission of Rickettsia rickettsii by an infected tick. Exposure in the United States occurs most commonly from April to September, and high-risk locations include wooded, shrubby, or grassy areas...
April 20, 2018: Journal of Emergency Medicine
https://www.readbyqxmd.com/read/29656466/evaluation-of-high-fat-high-fructose-diet-treatment-in-factor-viii-coagulation-factor-deficient-mouse-model
#10
Alaknanda Mishra, Shailendra Arindkar, Preeti Sahay, Jerald Mahesh Kumar, Pramod K Upadhyay, Subeer S Majumdar, Perumal Nagarajan
Non-alcoholic fatty liver disease (NAFLD)-like conditions enhance the production and action of clotting factors in humans. However, studies examining the effect of NAFLD due to high-fat high-fructose (HFHF) diet in factor VIII-deficient (haemophilia A) animals or patients have not been reported previously. In this study, we investigated the individual role of factor VIII in the progression of diet-induced NAFLD in the factor 8-/- (F8-/- ) mouse model system and its consequences on the haemophilic status of the mice...
April 15, 2018: International Journal of Experimental Pathology
https://www.readbyqxmd.com/read/29644153/acute-fatty-liver-of-pregnancy-a-thorough-examination-of-a-harmful-obstetrical-syndrome-and-its-counterparts
#11
Joshua Ronen, Shahzeb Shaheen, David Steinberg, Kevin R Justus
Diagnosed in one of every 20,000 deliveries, acute fatty liver of pregnancy (AFLP) was considered to be a deadly disease for many years. However, advancements in the clinical and surgical management of pregnant mothers have lead to a drastic decrease in maternal morbidity and mortality. The corresponding case recounts a 23-year-old gravida 2 para 1 (G2P1) at 38 weeks gestational age (GA) with no relevant past medical or family medical history that presented to the emergency department (ED) with a five-day history of nausea, protracted vomiting, hypertension, and new-onset headache...
February 6, 2018: Curēus
https://www.readbyqxmd.com/read/29626027/lesson-of-the-month-1-massive-spontaneous-haematomas-in-an-elderly-man
#12
Francesco Fiorini, Louise Braddick, Hasnain Hashim, Robert M Ayto, Alana Jacobs, Yetunde Baoku, Shahir S Hamdulay
A 73-year-old man presented with bilateral leg pain and swelling, and no history of trauma or bleeding disorders. Clinical examination, biochemistry and magnetic resonance imaging of the thighs were suggestive of muscle haematomas. These progressed significantly during the admission, requiring blood transfusion. Normal vascular anatomy on computed tomography, renal and liver function, and absence of infection made a bleeding diathesis more likely. This may be caused by coagulation defects, platelet disorders and vascular fragility...
March 2018: Clinical Medicine: Journal of the Royal College of Physicians of London
https://www.readbyqxmd.com/read/29605063/classification-and-epidemiologic-aspects-of-acute-liver-failure
#13
REVIEW
Daniel Pievsky, Neil Rustgi, Nikolaos T Pyrsopoulos
Acute liver failure is a rare condition with high short-term morbidity and mortality. The most widely accepted definition is an abnormality in coagulation with any degree of encephalopathy in a patient without cirrhosis and an illness duration of less than 26 weeks. Multiple classifications systems are currently in use to help categorize the condition. This article reviews the most commonly used systems. The epidemiologic aspects of the disease are also reviewed, including incidence, prevalence, demographics, geographic distribution, and racial and cultural factors and are discussed for the various subtypes of acute liver failure...
May 2018: Clinics in Liver Disease
https://www.readbyqxmd.com/read/29584463/do-we-still-need-cryoprecipitate-cryoprecipitate-and-fibrinogen-concentrate-as-treatments-for-major-hemorrhage-how-do-they-compare
#14
Alex Novak, Simon J Stanworth, Nicola Curry
Major hemorrhage is a source of significant mortality and morbidity worldwide. Identification and characterization of coagulation impairment associated with major hemorrhage has suggested a key role for fibrinogen deficiency, however the optimum mode of replacement of fibrinogen remains unclear, and standardized major hemorrhage protocols may overlook context-dependent variations in individual patients' clotting derangement. Areas covered: This paper examines the current practice and evidence regarding the role of different modes of fibrinogen replacement in major hemorrhage in 3 distinct clinical settings: trauma, obstetric hemorrhage, and gastrointestinal hemorrhage with associated liver disease...
May 2018: Expert Review of Hematology
https://www.readbyqxmd.com/read/29580525/assessment-of-effects-of-phenolic-fractions-from-leaves-and-petals-of-dandelion-in-selected-components-of-hemostasis
#15
Bernadetta Lis, Dariusz Jędrejek, Anna Stochmal, Beata Olas
Aerial parts and roots of Taraxacum officinale (dandelion) have been found to be rich sources of polyphenols, including cinnamic acid derivatives, flavonoids and triterpenoids, which exert different biological activities, such as anti-inflammatory, anticancer and antimicrobial. Additionally, the whole plant is recognized as safe and well tolerated by humans, with no reported adverse effects. Nowadays, dandelion is a commonly available dietary supplement and a component of pharmaceutical preparations used for the treatment of bladder, liver, and spleen...
May 2018: Food Research International
https://www.readbyqxmd.com/read/29565502/correlation-between-coronary-artery-lesion-quantitative-score-and-osahs-and-relative-risk-factors
#16
T Zhang, C Zhang, R-X Chen, B Shao, G-A Liu
OBJECTIVE: The purpose of this study was to investigate the relationship of obstructive sleep apnea hypopnea syndrome (OSAHS) with coronary artery lesion quantitative score Syntax Score (SX score) and risk factors for coronary heart disease (CHD). PATIENTS AND METHODS: A total of 115 patients with OSAHS admitted to the Department of Cardiology in our hospital from January 2011 to June 2015 were selected. Philips Respironics Alice 5 Polysomnography was used for sleep monitoring...
March 2018: European Review for Medical and Pharmacological Sciences
https://www.readbyqxmd.com/read/29529680/-gallstones-and-liver-obstruction-in-medical-texts-of-antiquity
#17
Frank Ursin, Florian Steger
INTRODUCTION:  Gallstones are rarely mentioned in the medical texts of antiquity. The physician, Alexander of Tralles mentions-for the first time-stones in the gallbladder as a possible cause for obstructive jaundice. This designation is found in his textbook on medicine under the heading "obstruction of the liver". Based on that observation, we describe the ancient history of hepatic obstruction and investigate the connection with the rare reference of gallstones in the medical texts of antiquity...
March 2018: Zeitschrift Für Gastroenterologie
https://www.readbyqxmd.com/read/29528784/wilson-disease-and-lupus-nephritis-is-it-coincidence-or-a-true-association
#18
Saishree Pradhan, Sriram Krishnamurthy, Barath Jagadisan, Nachiappa G Rajesh, Subashini Kaliaperumal, Suryaprakash Ramasamy, Nandeeswari Subramanian
A 12-year-old girl born to third-degree consanguineous parents presented with recurrent episodes of haematuria for 8 months in association with peri-orbital and lower limb oedema for 20 days. There was no jaundice, hepatomegaly or neurological abnormality at presentation. An older brother had died following jaundice at 10 years of age. Urinalysis showed multiple dysmorphic erythrocytes without proteinuria and there was leucopenia, thrombocytopenia and hypo-albuminaemia (23 g/L). C3 component of complementaemia was low and anti-nuclear antibodies and anti-double-stranded DNA antibodies were strongly positive by immunofluorescence...
March 12, 2018: Paediatrics and International Child Health
https://www.readbyqxmd.com/read/29513969/evaluation-of-anticoagulant-effect-of-sulfated-polysaccharide-sps-from-codium-dwarkense-borgesen-in-%C3%AE%C2%BA-carrageenan-induced-hypercoagulable-state-in-wistar-albino-rats
#19
Hiral N Golakiya, Viren N Naik, Hiren N Hirapara, Kalpanaben H Mody, Alpesh P Goswami, Chandrabhanu Tripathi
Our aim of the present study was to evaluate the anticoagulant effect of sulfated polysaccharides (SPS) from Codium dwarkense Bergesen in ic-carrageenan induced hypercoagulable state in Wistar albino rats. 48 Wistar albino rats of either sex were randomly divided into 6 groups - disease control, active control, treatment lowmand high dose and preventive low and high dose. K-Carrageenan (3 mg/kg) single dose intravenously was given in rat tail vein at pre-decided time to produce hypercoagulable state. Baseline, 24, 48 and 72 hours duration blood samples were collected for PT, INR, aPTT, platelet count and fibrinogen level, FDP and D-Dimer measurements...
May 2017: Acta Poloniae Pharmaceutica
https://www.readbyqxmd.com/read/29509634/comparison-of-transjugular-liver-biopsy-and-percutaneous-liver-biopsy-with-tract-embolization-in-pediatric-patients
#20
Sheryl Tulin-Silver, Chrystal Obi, Nishita Kothary, Matthew Lungren
PURPOSE: The aim of the study was to compare safety and efficacy of transjugular liver biopsy (TJLB) and percutaneous liver biopsy (PLB) with tract embolization in pediatric patients with liver disease. MATERIALS AND METHODS: TJLB and PLB between December 2009 and October 2015 were retrospectively reviewed. Primary endpoints were adequate sampling and complication rate. Patient age, weight, coagulation factors, ascites, blood transfusions, adequacy of biopsy sample, number of biopsy samples, and complications were compared...
March 5, 2018: Journal of Pediatric Gastroenterology and Nutrition
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