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https://www.readbyqxmd.com/read/28922103/budd-chiari-syndrome
#1
Tomáš Grus, Lukáš Lambert, Gabriela Grusová, Rohan Banerjee, Andrea Burgetová
Budd-Chiari syndrome (BCS) is a rare disease with an incidence of 0.1 to 10 per million inhabitants a year caused by impaired venous outflow from the liver mostly at the level of hepatic veins and inferior vena cava. Etiological factors include hypercoagulable conditions, myeloprolipherative diseases, anatomical variability of the inferior vena cava, and environmental conditions. Survival rates in treated patients range from 42 to 100% depending on the etiology and the presence of risk factors including parameters of Child-Pugh score, sodium and creatinine plasma levels, and the choice of treatment...
2017: Prague Medical Report
https://www.readbyqxmd.com/read/28844514/comparison-of-the-cha2ds2-vasc-chads2-has-bled-orbit-and-atria-risk-scores-in-predicting-non-vitamin-k-antagonist-oral-anticoagulants-associated-bleeding-in-patients-with-atrial-fibrillation
#2
Xiaoxi Yao, Bernard J Gersh, Lindsey R Sangaralingham, David M Kent, Nilay D Shah, Neena S Abraham, Peter A Noseworthy
The increasing adoption of non-vitamin K antagonist oral anticoagulants (NOACs) for stroke prevention in atrial fibrillation (AF) necessitates a reassessment of bleeding risk scores. Because known risk factors for bleeding are largely the same as for stroke, we hypothesize that stroke risk scores could also be used to identify patients with high bleeding risks. We aimed to compare the performance of 2 stroke risk scores (Congestive Heart failure, hypertension, Age ≥75 [doubled], Diabetes, Stroke [doubled], Vascular disease, Age 65-74, and Sex [female] [CHA2DS2-VASc] and Cardiac failure, Hypertension, Age, Diabetes, Stroke [Doubled] [CHADS2]) and 3 bleeding risk scores (hypertension, abnormal renal/liver function [1 point each], stroke, bleeding history or predisposition, labile INR, elderly [...
July 31, 2017: American Journal of Cardiology
https://www.readbyqxmd.com/read/28837506/coagulopathy-in-children-with-liver-disease
#3
Patricia Sachi Kawada, Aisha Bruce, Patti Massicotte, Mary Bauman, Jason Yap
It was thought that a high International Normalized Ratio (INR) predicted bleeding in patients with chronic liver disease (CLD) and patients were 'autoanticoagulated'. Contrary to this belief, while patients with CLD experienced bleeding, they also developed thromboses. In the last decade, the prevailing literature challenged the idea that an elevated INR increased bleeding risk. The global assays of coagulation such as: thromboelastography (TEG)/rotational thromboelastometry (ROTEM) and thrombin generation assays (TGA) provide additional insight into coagulation processes...
August 23, 2017: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/28834866/liver-cirrhosis-caused-by-chronic-budd-chiari-syndrome
#4
Mengjie Lin, Feng Zhang, Yi Wang, Bin Zhang, Wei Zhang, Xiaoping Zou, Ming Zhang, Yuzheng Zhuge
Chronic Budd-Chiari syndrome (BCS) is a rare cause of liver cirrhosis (LC) and tends to be misdiagnosed in clinical practice. In order to characterize LC caused by chronic BCS, we conducted this retrospective observational study. Medical records of all patients who were initially diagnosed as chronic BCS with LC when discharged from our department from January, 2011 to October, 2016 were reviewed. Cirrhotic patients with known causes and cases lacked key data were excluded. Data of remaining patients was collected and analyzed...
August 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28804827/assessment-of-hereditary-thrombophilia-performance-of-antithrombin-at-testing
#5
Jana N Gausman, Richard A Marlar
Antithrombin (AT) is a naturally occurring plasma inhibitor of coagulation, which is a synthesized in the liver. AT inhibits coagulation serine proteases (the enzymatically activated forms of the clotting factors), mainly thrombin (factor IIa) and factor Xa, but also to a lesser extent factors IXa, XIa, and XIIa. Acting alone, AT inhibits coagulation factors, but does this very slowly; however, when coupled with heparin as a cofactor, the speed of inhibition is increased many fold. The AT/Heparin complex is the most powerful naturally occurring anticoagulant in blood...
2017: Methods in Molecular Biology
https://www.readbyqxmd.com/read/28795966/the-coagulation-profile-of-end-stage-liver-disease-and-considerations-for-intraoperative-management
#6
Katherine T Forkin, Douglas A Colquhoun, Edward C Nemergut, Julie L Huffmyer
The coagulopathy of end-stage liver disease results from a complex derangement in both anticoagulant and procoagulant processes. With even minor insults, cirrhotic patients experience either inappropriate bleeding or clotting, or even both simultaneously. The various phases of liver transplantation along with fluid and blood product administration may contribute to additional disturbances in coagulation. Thus, anesthetic management of patients undergoing liver transplantation to improve hemostasis and avoid inappropriate thrombosis in the perioperative environment can be challenging...
August 8, 2017: Anesthesia and Analgesia
https://www.readbyqxmd.com/read/28772332/-expert-knowledge-and-supporting-advice-for-the-clinical-use-of-nintedanib-in-patients-with-idiopathic-pulmonary-fibrosis
#7
J Behr, A Günther, M Kreuter, D Koschel, A Prasse, M Pfeifer, U Costabel
In October 2016, a group of German IPF experts were invited by Boehringer Ingelheim to meet in Frankfurt with the aim, (a) to discuss relevant aspects of the management and treatment of idiopathic pulmonary fibrosis (IPF) using nintedanib; and, (b) to provide supportive advice for daily clinical practice with nintedanib. The resulting information compiled in this document is confined to practical issues regarding the use of nintedanib in patients with IPF. Where different therapeutic options were available, the choice of IPF medication was not discussed and the experts alluded to current guidelines for the diagnosis and treatment of IPF...
September 2017: Pneumologie
https://www.readbyqxmd.com/read/28740966/portal-vein-thrombosis-as-a-rare-cause-of-abdominal-pain-when-to-consider
#8
Cengiz Tavusbay, Erdinç Kamer, Turan Acar, İbrahim Kokulu, Haldun Kar, Özlem Gür
Extrahepatic portal vein thrombosis (PVT) is a rare condition that is characterized by the presence of thrombus within any segment of the portal vein, including the right and left intrahepatic branches. It may also extend to the splenic or superior mesenteric veins. Portal vein thrombosis may be related to cirrhosis or liver malignancy as well as to local inflammatory conditions in the abdomen and genetic or acquired thrombophilic diseases. Currently, PVT is being increasingly diagnosed due to advances in modern imaging techniques...
2017: Turk J Surg
https://www.readbyqxmd.com/read/28734547/management-of-hemostatic-disorders-in-patients-with-advanced-liver-disease-admitted-to-an-intensive-care-unit
#9
REVIEW
Ton Lisman, William Bernal
Patients with liver diseases frequently acquire complex changes in their hemostatic system. Traditionally, bleeding complications in patients with liver disease were ascribed to these hemostatic changes, and liver diseases were considered as an acquired bleeding disorder. Nowadays, it is increasingly acknowledged that patients with liver diseases are in "hemostatic rebalance" due to a commensurate decline in pro- and anticoagulant drivers. Indeed, both thrombosis and bleeding may complicate liver disease. Such complications may be particularly worrisome in critically ill patients with liver disease...
June 23, 2017: Transfusion Medicine Reviews
https://www.readbyqxmd.com/read/28711073/lupus-nephritis-and-pregnancy-concerns-and-management
#10
Liz Lightstone, Michelle A Hladunewich
Pregnancy associated with lupus, especially lupus nephritis, is often fraught with concern for both the mother and fetus. Thus, it is paramount that care begins preconception so that proper planning in terms of optimizing the medical regimen, discontinuation of fetotoxic agents, and treatment of active disease can occur. It is well known that active nephritis at the time of conception is associated with poor outcomes. Even with quiescent disease, recent data indicate that being lupus anticoagulant-positive, nonwhite or Hispanic, and using antihypertensive medications were all predictors of worse pregnancy outcomes...
July 2017: Seminars in Nephrology
https://www.readbyqxmd.com/read/28710306/ruxolitinib-treatment-in-an-infant-with-jak2-polycythaemia-vera-associated-budd-chiari-syndrome
#11
Mehmet Enes Coskun, Sue Height, Anil Dhawan, Nedim Hadzic
Budd-Chiari syndrome (BCS) is caused by hepatic venous outflow obstruction commonly seen with myeloproliferative neoplasms (MPNs). Polycythaemia vera (PV) is a very rare MPN in childhood. This is the youngest reported patient diagnosed with PV and BCS secondary to JAK V617F mutation.A 26-month-old girl was admitted with a 5-month history of abdominal distension, hepatosplenomegaly and ascites. Imaging studies revealed occlusion of the right hepatic vein and marked attenuation of the middle and left hepatic veins...
July 14, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28709650/anticoagulation-control-in-warfarin-treated-patients-undergoing-cardioversion-of-atrial-fibrillation-from-the-edoxaban-versus-enoxaparin-warfarin-in-patients-undergoing-cardioversion-of-atrial-fibrillation-trial
#12
RANDOMIZED CONTROLLED TRIAL
Gregory Y H Lip, Naab Al-Saady, James Jin, Ming Sun, Michael Melino, Shannon M Winters, Dmitry Zamoryakhin, Andreas Goette
In the Edoxaban Versus Enoxaparin-Warfarin in Patients Undergoing Cardioversion of Atrial Fibrillation (ENSURE-AF) study (NCT 02072434), edoxaban was compared with enoxaparin-warfarin in 2,199 patients undergoing electrical cardioversion of nonvalvular atrial fibrillation (AF). In this multicenter prospective randomized open blinded end-point trial, we analyzed patients randomized to enoxaparin-warfarin. We determined time to achieve therapeutic range (TtTR); time in therapeutic range (TiTR); their clinical determinants; relation to sex, age, medical history, treatment, tobacco use, race risk (SAMe-TT2R2) score; and impact on primary end points (composite of stroke, systemic embolic event[SEE], myocardial infarction [MI], and cardiovascular death [CVD] and composite of major + clinically relevant nonmajor bleeding)...
September 1, 2017: American Journal of Cardiology
https://www.readbyqxmd.com/read/28698855/the-imbalance-of-procoagulant-and-anticoagulant-factors-in-patients-with-chronic-liver-diseases-in-north-india
#13
Priyanka Saxena, Chhagan Bihari, Roshni Mirza, Ajeet Singh Bhadoria, Shiv K Sarin
No abstract text is available yet for this article.
June 2017: Blood Research
https://www.readbyqxmd.com/read/28685257/budd-chiari-syndrome-hepatic-venous-outflow-tract-obstruction
#14
REVIEW
Dominique-Charles Valla
BACKGROUND: Budd-Chiari syndrome (BCS) is a rare disease characterized by hepatic venous outflow tract obstruction (HVOTO). METHODS: Recent literature has been analyzed for this narrative review. RESULTS: Primary BCS/HVOTO is a result of thrombosis. The same patient often has multiple risk factors for venous thrombosis and most have at least one. Presentation and etiology may differ between Western and certain Eastern countries. Myeloproliferative neoplasms are present in 40% of patients and are usually associated with the V617F-JAK2 mutation in myeloid cells, in particular peripheral blood granulocytes...
July 6, 2017: Hepatology International
https://www.readbyqxmd.com/read/28665498/enoxaparin-does-not-ameliorate-liver-fibrosis-or-portal-hypertension-in-rats-with-advanced-cirrhosis
#15
José I Fortea, Alexander Zipprich, Carolina Fernandez-Mena, Marta Puerto, Cristina R Bosoi, Jorge Almagro, Marcus Hollenbach, Juan Bañares, Belén Rodríguez-Sánchez, Emilia Cercenado, Marc-André Clément, Christopher F Rose, Rafael Bañares, Javier Vaquero, Cristina Ripoll
BACKGROUND & AIMS: Recent studies suggest that heparins reduce liver fibrosis and the risk of decompensation of liver disease. Here, we evaluated the effects of enoxaparin in several experimental models of advanced cirrhosis. METHODS: Cirrhosis was induced in male Sprague-Dawley (SD) rats by: i. Oral gavage with carbon tetrachloride (CCl4ORAL ), ii. Bile duct ligation (BDL), and iii. CCl4 inhalation (CCl4INH ). Rats received saline or enoxaparin s.c. (40 IU/Kg/d or 180 IU/Kg/d) following various protocols...
June 30, 2017: Liver International: Official Journal of the International Association for the Study of the Liver
https://www.readbyqxmd.com/read/28630388/spontaneous-liver-rupture-associated-with-anticoagulant-therapy-a-case-report
#16
Marco Clementi, Sara Colozzi, Stefano Guadagni, Beatrice Pessia, Federico Sista, Mario Schietroma, Andrea Della Penna, Gianfranco Amicucci
Spontaneous hepatic rupture without underlying liver diseases is uncommon entity. We report a rare case of spontaneous rupture of liver hematoma in patient treated with warfarin end enoxaparin sodium because of pulmonary embolism. Two day after admission the patient complained generalized abdominal pain and hemodynamic instability. The abdominal US and TC scan revealed free fluid and lesion at right liver lobe. The patient, despite intravenous fluid support and blood transfusion, was hemodinamically instable and urgent laparotomy was needed...
January 20, 2017: Annali Italiani di Chirurgia
https://www.readbyqxmd.com/read/28614247/meld-score-as-a-predictor-of-mortality-length-of-hospital-stay-and-disease-burden-a-single-center-retrospective-study-in-39-323-inpatients
#17
Jan A Roth, Carl Chrobak, Sabine Schädelin, Balthasar L Hug
The laboratory-based model for end-stage liver disease (MELD) score reflects the function of the kidney, liver, and extrinsic coagulation pathway and might be used as a general prognostic tool for the assessment of patients. We therefore aimed to investigate a potential association of the MELD score with mortality, length of hospital stay (LOS), and disease burden in a general patient population.We performed a retrospective observational study at a tertiary referral center. From January 2012 through December 2013, all consecutive inpatients aged 18 years were eligible for the study; patients with missing MELD parameters on hospital admission and/or treatments influencing the international normalized ratio, that is, novel oral anticoagulants and vitamin K antagonists, were excluded...
June 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28610817/vascular-diseases-of-the-liver-clinical-guidelines-from-the-catalan-society-of-digestology-and-the-spanish-association-for-the-study-of-the-liver
#18
Marta Martín-Llahí, Agustín Albillos, Rafael Bañares, Annalisa Berzigotti, M Ángeles García-Criado, Joan Genescà, Virginia Hernández-Gea, Elba Llop-Herrera, Helena Masnou-Ridaura, José Mateo, Carmen A Navascués, Ángela Puente, Marta Romero-Gutiérrez, Macarena Simón-Talero, Luis Téllez, Fanny Turon, Cándido Villanueva, Roberto Zarrabeitia, Juan Carlos García-Pagán
Despite their relatively low prevalence, vascular diseases of the liver represent a significant health problem in the field of liver disease. A common characteristic shared by many such diseases is their propensity to cause portal hypertension together with increased morbidity and mortality. These diseases are often diagnosed in young patients and their delayed diagnosis and/or inappropriate treatment can greatly reduce life expectancy. This article reviews the current body of evidence concerning Budd-Chiari syndrome, non-cirrhotic portal vein thrombosis, idiopathic portal hypertension, sinusoidal obstruction syndrome, hepatic vascular malformations in hereditary haemorrhagic telangiectasia, cirrhotic portal vein thrombosis and other rarer vascular diseases including arterioportal fistulas...
June 11, 2017: Gastroenterología y Hepatología
https://www.readbyqxmd.com/read/28604569/use-of-four-factor-prothrombin-complex-concentrate-in-the-reversal-of-warfarin-induced-and-nonvitamin-k-antagonist-related-coagulopathy
#19
Hannah Young, Jeremy L Holzmacher, Richard Amdur, Stephen Gondek, Babak Sarani, Mary E Schroeder
: To evaluate the efficacy of international normalized ratio (INR) reversal using four-factor prothrombin complex concentrate (4F-PCC) in nonmedication-induced coagulopathy. We performed a single-site, retrospective cohort study of patients receiving off-label use of 4F-PCC. Cohorts included liver dysfunction if they had acute liver decompensation or cirrhosis without other causative factors of liver failure such as sepsis, coagulopathy of acute sepsis (CAS) if they had documentation of sepsis and no underlying liver disorder, known factor deficiencies, or medication-induced coagulopathy, or warfarin if they were taking warfarin...
June 9, 2017: Blood Coagulation & Fibrinolysis: An International Journal in Haemostasis and Thrombosis
https://www.readbyqxmd.com/read/28604464/liver-transplantation-in-a-patient-with-antiphospholipid-syndrome-a-case-report
#20
Rachel C Steckelberg, Zarah D Antongiorgi, Randolph H Steadman
Antiphospholipid syndrome (APS) is an acquired thrombophilic disorder characterized by autoantibodies to cell membrane phospholipids. While altered coagulation can complicate end-stage liver disease, there are few reports describing the perioperative management for liver transplantation in recipients with a preexisting hypercoagulable disorder, such as APS. We present a patient with a history of APS, Budd-Chiari syndrome with cirrhosis, hepatopulmonary syndrome, and heparin-induced thrombocytopenia who underwent liver transplantation complicated by hepatic artery thrombosis...
September 1, 2017: A & A Case Reports
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