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antithrombin deficiency

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https://www.readbyqxmd.com/read/27902939/factor-xiii-deficiency-enhances-thrombin-generation-due-to-impaired-fibrin-polymerization-an-effect-corrected-by-factor-xiii-replacement
#1
Hanna H Pitkänen, Annukka Jouppila, Marja Lemponen, Minna Ilmakunnas, Jouni Ahonen, Riitta Lassila
INTRODUCTION: Factor XIII (FXIII) cross-links fibrin, completing blood coagulation. Congenital FXIII deficiency is managed with plasma-derived FXIII (pdFXIII) or recombinant FXIII (rFXIII) concentrates. AIM: As the mechanisms protecting patients with low FXIII levels (<5IU/dL) from spontaneous bleeds remain unknown we assessed the interplay between thrombin generation (TG), fibrin formation and clot kinetics before and after FXIII administration in three patients with FXIII deficiency...
November 16, 2016: Thrombosis Research
https://www.readbyqxmd.com/read/27891624/quarantine-versus-pathogen-reduced-plasma-coagulation-factor-content-and-rotational-thromboelastometry-coagulation
#2
Oliver M Theusinger, David Goslings, Jan-Dirk Studt, Brigitte Brand-Staufer, Burkhardt Seifert, Donat R Spahn, Beat M Frey
BACKGROUND: Different types of fresh-frozen plasma (FFP) exist, and the concentrations of plasma proteins vary between individuals and blood groups. Furthermore, processing may also influence the content. Quarantine-stored plasma (qFFP) and plasma that was pathogen-reduced using blood-safety (Intercept) technology (piFFP) were analyzed regarding procoagulant and anticoagulant hemostasis proteins, including endogenous thrombin (thrombin-generation) potential (ETP). MATERIALS AND METHODS: Thirty-five samples of each type of FFP were analyzed using only male Blood Group O donors...
November 27, 2016: Transfusion
https://www.readbyqxmd.com/read/27863268/subtypes-of-serpinc1-mutations-and-the-thrombotic-phenotype-of-inherited-antithrombin-deficient-individuals-in-chinese-han-population
#3
Dong Wang, Guanglin Cui, Senlin Hu, Dao Wen Wang
Inherited antithrombin (AT) deficiency is a rare autosomal disease that could increase the risk of venous thromboembolism (VTE) and usually caused by mutations of SERPINC1. Although a number of mutations of SERPINC1 have been reported in Chinese Han population, the impact of different subtypes of these mutations on the thrombotic phenotype is still unknown. Here, we performed a retrospective cohort study including 169 AT patients from 63 families to compare the clinical features between null mutation carriers and missense mutation carriers...
November 2016: Blood Cells, Molecules & Diseases
https://www.readbyqxmd.com/read/27858332/causative-genetic-mutations-for-antithrombin-deficiency-and-their-clinical-background-among-japanese-patients
#4
Akiko Sekiya, Fumina Taniguchi, Daisuke Yamaguchi, Sayaka Kamijima, Shonosuke Kaneko, Shiori Katsu, Miho Hanamura, Mao Takata, Haruka Nakano, Hidesaku Asakura, Shigeki Ohtake, Eriko Morishita
We summarize causative genetic mutations for antithrombin (AT) deficiency and their clinical background in Japanese patients. A total of 19 mutations, including seven novel mutations, were identified. We also summarize clinical symptoms of thrombosis, age at onset, family history, and contributing factors for thrombosis, and review the use of prophylactic anticoagulation in pregnant women with heterozygous type II heparin binding site defects (HBS) AT deficiency. The prevalence of thrombosis in probands with type I AT deficiency (88%) was double that observed in those with type II AT deficiency (50%)...
November 17, 2016: International Journal of Hematology
https://www.readbyqxmd.com/read/27853070/edoxaban-was-effective-for-the-treatment-of-deep-vein-thrombosis-and-pulmonary-thromboembolism-in-a-cancer-patient-with-antithrombin-iii-deficiency
#5
Hiroaki Kawano, Koji Maemura
A 57-year-old man with antithrombin III deficiency with deep vein thrombosis and pulmonary thromboembolism after surgery for colon cancer was managed with warfarin and inferior vena cava filtration. After starting chemotherapy for liver metastasis, the control of his prothrombin time-international normalized ratio in response to warfarin began to fluctuate. Warfarin was changed to edoxaban (an oral direct Factor Xa inhibitor), and he had no recurrence of venous thromboembolism or bleeding for approximately 1 year...
2016: Internal Medicine
https://www.readbyqxmd.com/read/27851127/1491-antithrombin-iii-deficiency-relates-to-mortality-in-sepsis
#6
Noriko Saito, Ayumi Kawasaki, Noboru Akizuki, Mizuho Namiki, Munekazu Takeda, Arino Yaguchi
No abstract text is available yet for this article.
December 2016: Critical Care Medicine
https://www.readbyqxmd.com/read/27848915/heparin-monitoring-clinical-outcome-and-practical-approach
#7
Noémie Despas, Anne-Sophie Larock, Hugues Jacqmin, Jonathan Douxfils, Bernard Chatelain, Marc Chatelain, François Mullier
Traditional anticoagulant agents such as unfractionated heparin (UFH), low molecular weight heparins (LMWHs), fondaparinux, danaparoid and bivalirudine are used in the prevention and treatment of thromboembolic diseases. However, these agents have limitations: their constraining parenteral route of administration and the need for regular coagulation monitoring for HNF. The LMWHs, with their more predictable anticoagulant response, don't require a systematic monitoring. The usefulness of LMWHs monitoring in several clinical situations such as pregnancy, obesity and renal insufficiency is a matter of debate...
December 1, 2016: Annales de Biologie Clinique
https://www.readbyqxmd.com/read/27799851/low-molecular-weight-heparin-can-benefit-women-with-recurrent-pregnancy-loss-and-sole-protein-s-deficiency-a-historical-control-cohort-study-from-taiwan
#8
Ming-Ching Shen, Wan-Ju Wu, Po-Jen Cheng, Gwo-Chin Ma, Wen-Chu Li, Jui-Der Liou, Cheng-Shyong Chang, Wen-Hsiang Lin, Ming Chen
BACKGROUND: Heritable thrombophilias are assumed important etiologies for recurrent pregnancy loss. Unlike in the Caucasian populations, protein S and protein C deficiencies, instead of Factor V Lieden and Prothrombin mutations, are relatively common in the Han Chinese population. In this study we aimed to investigate the therapeutic effect of low molecular weight heparin upon women with recurrent pregnancy loss and documented protein S deficiency. METHODS: During 2011-2016, 68 women with recurrent pregnancy loss (RPL) and protein S deficiency (both the free antigen and function of protein S were reduced) were initially enrolled...
2016: Thrombosis Journal
https://www.readbyqxmd.com/read/27795525/how-to-choose-and-understand-clinical-laboratory-tests-in-daily-clinical-management-of-hematologic-diseases
#9
Yoshinobu Seki
General statements have been published regarding the clinical laboratory tests for thrombosis and hemostasis to be used by hematologists who do not specialize in this field. Screening tests for bleeding are bleeding time, capillary fragility test, platelet count, prothrombin time, activated partial thromboplastin time (APTT), fibrinogen, fibrin/fibrinogen degradation products (FDP), and the cross mixing test conducted by measuring APTT. Screening tests for thrombosis and related factors include measurements of FDP, D-dimer, and thrombin-antithrombin complex...
2016: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
https://www.readbyqxmd.com/read/27795523/diagnosis-and-management-of-dic-complicated-by-hematological-malignancies
#10
Takayuki Ikezoe
The clinical features noted in individuals with disseminated intravascular coagulation (DIC) complicated by hematological malignancies include life threatening hemorrhage that is associated with thrombocytopenia and consumptive deficiency of coagulation factors. Exacerbation of DIC after the initiation of chemotherapy is also related to fatal hemorrhage. The Japanese Society of Thrombosis and Hemostasis recently proposed provisional DIC diagnostic criteria allowing evaluation of hypercoagulable markers such as soluble fibrin and thrombin-antithrombin complex to help physicians to diagnose DIC and initiate treatment in the early phase of coagulopathy...
2016: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
https://www.readbyqxmd.com/read/27786338/coagulation-factor-xii-induces-pro-inflammatory-cytokine-responses-in-macrophages-and-promotes-atherosclerosis-in-mice
#11
Sandra Vorlova, Miriam Koch, Helga D Manthey, Clement Cochain, Martin Busch, Sweena M Chaudhari, David Stegner, Manuel Yepes, Kristina Lorenz, Marc W Nolte, Bernhard Nieswandt, Alma Zernecke
Atherosclerosis is considered a chronic inflammatory disease of the vessel wall. Coagulation pathways and immune responses contribute to disease development. The role of coagulation factor XII (FXII) in vascular inflammation, however, remains controversial. We here investigated the function of FXII in atherosclerosis using apolipoprotein E and FXII-deficient (F12(-/-)Apoe(-/-)) mice. Compared to F12(+/+)Apoe(-/-) controls, atherosclerotic lesion formation was reduced in F12(-/-)Apoe(-/-) mice. This was associated with a decrease in serum interleukin (IL)-1β and IL-12 levels and reduced expression of pro-inflammatory cytokines in the aorta in atherosclerotic F12(-/-)Apoe(-/-) mice, as well as diminished Th1-cell differentiation in the aorta, blood, and lymphoid organs...
October 27, 2016: Thrombosis and Haemostasis
https://www.readbyqxmd.com/read/27783482/an-asymmetric-antithrombin-dimer-is-a-key-intermediate-for-polymerization-revealed-by-hydrogen-deuterium-exchange-mass-spectrometry
#12
Morten Beck Trelle, Shona H Pedersen, Eva Christina Østerlund, Jeppe Buur Madsen, Søren Risom Kristensen, Thomas J D Jørgensen
Antithrombin deficiency is associated with increased risk of venous thrombosis. In certain families this condition is caused by pathogenic polymerization of mutated antithrombin in the blood. To facilitate future development of pharmaceuticals against antithrombin polymerization an improved understanding of the polymerogenic intermediates is crucial. However, X-ray crystallography of these intermediates is severely hampered by the difficulty in obtaining well-diffracting crystals of transient and heterogeneous noncovalent protein assemblies...
October 26, 2016: Analytical Chemistry
https://www.readbyqxmd.com/read/27766527/high-prevalence-of-congenital-thrombophilia-in-patients-with-pregnancy-related-or-idiopathic-venous-thromboembolism-pulmonary-embolism
#13
Makoto Ikejiri, Hideo Wada, Norikazu Yamada, Maki Nakamura, Naoki Fujimoto, Kaname Nakatani, Akimasa Matsuda, Yosihito Ogihara, Takeshi Matsumoto, Yuki Kamimoto, Tomoaki Ikeda, Naoyuki Katayama, Masaaki Ito
Congenital thrombophilia which is characterized by deficiencies in proteins such as antithrombin (AT), protein C (PC) and protein S (PS), is a major cause of venous thromboembolism (VTE). A total of 130 patients with VTE were evaluated for congenital thrombophilia based on the activity of AT, PC, or PS. Fifteen VTE patients with congenital AT deficiency (11.5 %), 16 with congenital PC deficiency (12.3 %) and eight with congenital PS deficiency (6.2 %) were diagnosed using DNA analysis. The frequency of congenital AT deficiency was significantly higher in subjects with pregnancy-related and idiopathic VTE than in those with VTE due to other causes, and congenital PC and PS deficiency were frequently associated with idiopathic VTE...
October 20, 2016: International Journal of Hematology
https://www.readbyqxmd.com/read/27766051/thrombophilia-in-east-asian-countries-are-there-any-genetic-differences-in-these-countries
#14
Toshiyuki Miyata, Keiko Maruyama, Fumiaki Banno, Reiko Neki
In recent years, genetic analyses of congenital deficiencies of three anticoagulant proteins, antithrombin, protein C (PC) and protein S (PS), in East Asian patients with venous thromboembolism (VTE) have greatly increased. The PS-K196E mutation is often identified in the Japanese population with an allelic frequency of 0.86 %, and a total of approximately 10,000 Japanese are estimated to be homozygotes. The heterozygotes show PS anticoagulant activities ranging from 40 to 110 %, and 16 % lower mean anticoagulant activity than that in wild-type individuals...
2016: Thrombosis Journal
https://www.readbyqxmd.com/read/27745619/hypercoagulable-states-what-the-oral-surgeon-needs-to-know
#15
Robert Bona
Thrombophilia or hypercoagulable conditions can be thought of as either inherited or acquired. The inherited disorders include deficiencies of antithrombin, protein C, or protein S or the common disorders of factor V Leiden and prothrombin G20210A gene mutation. All these disorders are inherited as autosomal dominant and predispose individuals primarily to venous thrombosis. Acquired thrombophilic conditions are seen in individuals with cancer, phospholipid antibodies, and a whole host of other conditions that alter endothelial function, change blood levels of coagulant or anticoagulant proteins, activate platelets, or have other effects on coagulation proteins, platelet function, or the endothelium...
November 2016: Oral and Maxillofacial Surgery Clinics of North America
https://www.readbyqxmd.com/read/27734187/the-use-of-direct-oral-anticoagulants-in-inherited-thrombophilia
#16
Jessica W Skelley, C Whitney White, Angela R Thomason
To review the use of the direct oral anticoagulant (DOAC) agents in inherited thrombophilia based on the literature. MEDLINE, International Pharmaceutical Abstracts, and Google Scholar searches (1970-May 2016) were conducted for case reports, case series, retrospective cohorts, or clinical trials using the key words: protein C deficiency, protein S deficiency, antithrombin deficiency, activated protein C resistance, Factor V Leiden, hypercoagulable, NOACs, dabigatran, apixaban, rivaroxaban, betrixaban, edoxaban, Xa inhibitor, direct thrombin inhibitor...
October 12, 2016: Journal of Thrombosis and Thrombolysis
https://www.readbyqxmd.com/read/27722126/venous-thromboembolism-in-pediatric-patients-a-single-institution-experience-in-korea
#17
Hyoung Soo Choi, Chang Won Choi, Heon Min Kim, Hye Won Park
BACKGROUND: While venous thromboembolism (VTE) is uncommon, its incidence is increasing in children. We aimed to evaluate the incidence, risk factors, treatment, and outcome of pediatric VTE cases at a single tertiary hospital in Korea. METHODS: We retrospectively analyzed the records of consecutive pediatric VTE patients admitted to the Seoul National University Bundang Hospital between April 2003 and March 2016. RESULTS: Among 70,462 hospitalizations, 25 pediatric VTE cases were identified (3...
September 2016: Blood Research
https://www.readbyqxmd.com/read/27712638/calciphylaxis-a-disease-of-pannicular-thrombosis
#18
Rokea A El-Azhary, Michelle T Patzelt, Robert D McBane, Amy L Weaver, Robert C Albright, Alina D Bridges, Paul L Claus, Mark D P Davis, John J Dillon, Ziad M El-Zoghby, LaTonya J Hickson, Rajiv Kumar, Kathleen A M McCarthy-Fruin, Marian T McEvoy, Mark R Pittelkow, David A Wetter, Amy W Williams, James T McCarthy
OBJECTIVE: To identify coagulation risk factors in patients with calciphylaxis and the relationship between anticoagulation use and overall survival. PATIENTS AND METHODS: Study subjects were 101 patients with calciphylaxis seen at Mayo Clinic from 1999 to September 2014. Data including thrombophilia profiles were extracted from the medical records of each patient. Survival status was determined using patient registration data and the Social Security Death Index...
October 2016: Mayo Clinic Proceedings
https://www.readbyqxmd.com/read/27708219/a-small-deletion-in-serpinc1-causes-type-i-antithrombin-deficiency-by-promoting-endoplasmic-reticulum-stress
#19
Jingjing Su, Liang Shu, Zhou Zhang, Lei Cai, Xin Zhang, Yu Zhai, Jianren Liu
Antithrombin (AT) deficiency is an autosomal dominant disorder, and identification of mutation AT variants would improve our understanding of the anticoagulant function of this serine protease inhibitor (SERPIN) and the molecular pathways underlying this disorder. In the present study, we performed whole-exome sequencing of a Chinese family with deep vein thrombosis, and identified a new small deletion that eliminates four amino acids (INEL) from exon 4 of SERPINC1 gene. This causes type I AT deficiency by enhancing the intracellular retention of this protein...
September 30, 2016: Oncotarget
https://www.readbyqxmd.com/read/27701728/warfarin-induced-skin-necrosis-in-patients-with-low-protein-c-levels
#20
Marino Marčić, Ljiljana Marčić, Marina Titlić
Warfarin-induced skin necrosis (WISN) is a rare complication of anticoagulant therapy associated with a high incidence of  morbidity and mortality requiring immediate drug cessation. At particular risk are those with various thrombophilic abnormalities, especially when warfarinisation is undertaken rapidly with large loading doses of warfarin. Cutaneous findings include petechiae that progress to ecchymosis and hemorrhagic bullae. With the increasing number of patients anticoagulated as out-patients for thromboprophylaxis, we are concerned that the incidence of skin necrosis may increase...
August 2016: Acta Medica Iranica
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