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https://www.readbyqxmd.com/read/28316091/2b-or-not-2b-a-prothrombotic-tendency-masquerading-as-a-bleeding-disorder
#1
Yvonne Brennan, Jennifer Curnow, Emmanuel J Favaloro
No abstract text is available yet for this article.
March 17, 2017: American Journal of Hematology
https://www.readbyqxmd.com/read/28306198/plasma-levels-of-plasminogen-activator-inhibitor-1-and-bleeding-phenotype-in-patients-with-von-willebrand-disease
#2
S Abdul, J Boender, J J M C Malfliet, J Eikenboom, K Fijn van Draat, E P Mauser-Bunschoten, K Meijer, J de Meris, B A P Laros-van Gorkom, J G van der Bom, F W G Leebeek, D C Rijken, S Uitte de Willige
INTRODUCTION: von Willebrand disease (VWD) is the most common inherited bleeding disorder. In VWD patients, large variations in bleeding tendency are observed, which cannot be completely explained by the variation in von Willebrand factor levels or activities. Thus, there must be additional factors, for instance, changes in fibrinolysis that have an effect on the variation in bleeding tendency in VWD patients. AIM: To investigate whether plasminogen activator inhibitor-1 (PAI-1) level influences the variation in bleeding tendency in VWD patients...
March 17, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28296243/measurement-of-platelet-aggregation-independent-of-patient-platelet-count-a-flow-cytometric-approach
#3
Pernille Just Vinholt, Henrik Frederiksen, Anne-Mette Hvas, Ulrik Sprogøe, Christian Nielsen
BACKGROUND: Methods for testing platelet aggregation in thrombocytopenia are missing. OBJECTIVE: To establish a flow cytometric test of in vitro platelet aggregation independent of the patient platelet count and examined the association of aggregation with bleeding history in thrombocytopenic patients. PATIENTS/METHODS: We established a flow cytometric assay of platelet aggregation and measured samples from healthy individuals pre-incubated with antiplatelet drugs and samples from two patients with inherited platelet disorders...
March 15, 2017: Journal of Thrombosis and Haemostasis: JTH
https://www.readbyqxmd.com/read/28271646/two-treatment-cases-of-severe-fever-and-thrombocytopenia-syndrome-with-oral-ribavirin-and-plasma-exchange
#4
In Park, Hye In Kim, Ki Tae Kwon
Severe fever with thrombocytopenia syndrome (SFTS) is an emerging tick-borne disease. The primary symptoms associated with SFTS are fever, thrombocytopenia, leukopenia, nausea, and vomiting. Disease progression shows high mortality rate accompanied with multiple organ failure, bleeding tendency, and altered mentality. However, only supportive care has been the basis for the treatment of SFTS. We are reporting two patients who showed central nervous system manifestation, but cured them with ribavirin together with plasma exchange in an early state...
January 19, 2017: Infection & Chemotherapy
https://www.readbyqxmd.com/read/28271416/bleeding-tendency-and-platelet-function-during-treatment-with-romiplostim-in-children-with-severe-immune-thrombocytopenic-purpura
#5
Elena V Suntsova, Irina M Demina, Anastasia A Ignatova, Nikolay M Ershov, Natalia M Trubina, Juliya Dobrynina, Irina V Serkova, Zhanna S Supik, Ekaterina V Orekhova, Lili A Hachatryan, Natalia N Kotskaya, Aleksey V Pshonkin, Aleksey A Maschan, Galina A Novichkova, Mikhail A Panteleev
It has been suggested that platelet function in chronic immune thrombocytopenic purpura (ITP) may be abnormal. Thrombopoietin mimetics used for treatment can affect it, but the data remain limited. We investigated platelet function of 20 children diagnosed with severe ITP (aged 1-16 years, 12 females and eight males). Platelet functional activity in whole blood was characterized by flow cytometry before and after stimulation with SFLLRN plus collagen-related peptide. Levels of CD42b, PAC1, and CD62P, but not CD61 or annexin V, were significantly increased (P < 0...
March 7, 2017: International Journal of Hematology
https://www.readbyqxmd.com/read/28267383/whole-exome-sequencing-identifies-a-mutation-in-thrombomodulin-as-the-genetic-cause-of-a-suspected-platelet-disorder-in-a-family-with-normal-platelet-function
#6
Annabel Maclachlan, Gerry Dolan, Charlotte Grimley, Steve P Watson, Neil V Morgan, On Behalf Of The Uk Gapp Study Group
Here, we describe a mother and son with a lifelong bleeding tendency and posttraumatic bleeding who were recruited to the UK Genotyping and Phenotyping of Platelets (GAPP) study with a suspected platelet function disorder. However, despite a clinically significant bleeding score, both had normal platelet counts and normal platelet function. The patients' blood was analyzed by light transmission aggregometry and genotyping by whole exome sequencing, as outlined by the GAPP study. Approximately 25 000 genetic variants were found for each patient as a result of sequencing and were filtered using a specialized bioinformatics pipeline...
March 7, 2017: Platelets
https://www.readbyqxmd.com/read/28251495/new-clotting-disorders-that-cast-new-light-on-blood-coagulation-and-may-play-a-role-in-clinical-practice
#7
REVIEW
A Girolami, E Cosi, S Ferrari, A M Lombardi, B Girolami
Recently several variants of clotting factors have shown a peculiar behavior so that they appear as new defects. The factors involved are FII, FV and FIX. Prothrombin deficiency is usually associated with bleeding. Recently a few prothrombin abnormalities involving Arg396 mutations, have been demonstrated to show antithrombin resistance with the consequent appearance of a thrombophilic state and venous thromboses in young age. The same is true for an abnormal FIX (FIX Padua). The thrombotic manifestations in the latter condition are also venous...
March 1, 2017: Journal of Thrombosis and Thrombolysis
https://www.readbyqxmd.com/read/28240343/term-twin-birth-impact-of-mode-of-delivery-on-outcome
#8
Elina Ylilehto, Outi Palomäki, Heini Huhtala, Jukka Uotila
The main aims of this study were to compare maternal and neonatal outcomes in term twin birth according to the planned mode of delivery and to study the effects of chorionicity and inter-twin delivery time on neonatal outcome MATERIAL AND METHODS: A single-center cohort study of 495 women with twin deliveries at ≥ 37+0 weeks of gestation. Term twin deliveries were divided into a trial of labor group (TOL, 69.3%) and a planned cesarean section (CS) group (30.7%). The primary outcomes were maternal and neonatal morbidity RESULTS: 80...
February 27, 2017: Acta Obstetricia et Gynecologica Scandinavica
https://www.readbyqxmd.com/read/28239082/minimal-resection-of-jejuna-dieulafoy-s-lesion-using-an-intraoperative-fluoroscopic-localization-of-the-metallic-coils-used-in-angiography
#9
Kwang Il Seo, Won Moon, Cheon Woo Lee, Seun Ja Park, Moo In Park, Seung Eun Kim, Jae Hyun Kim, Ki Young Yoon, Hee Kyung Chang
Dieulafoy's lesions of the Jejunum are extremely rare. Therefore, localization of lesions is very difficult due to their small size and tendency of occasional bleeding. However, it is important to mention the location of the Dieulafoy's lesions to prevent excessive intestinal resections or, even worse, resection of the normal intestine. We report a case of preoperative localization of a Dieulafoy's lesion embolized by a metallic coil that allows a surgeon to accurately identify the bleeding, permitting a minimally invasive surgical treatment...
February 25, 2017: Korean Journal of Gastroenterology, Taehan Sohwagi Hakhoe Chi
https://www.readbyqxmd.com/read/28229167/mutation-in-a-highly-conserved-glycine-residue-in-strand-5b-of-plasminogen-activator-inhibitor-1-causes-polymerisation
#10
Takayuki Iwaki, Kotomi Nagahashi, Katsuhiro Takano, Katsue Suzuki-Inoue, Naohiro Kanayama, Kazuo Umemura, Tetsumei Urano
Serpinopathy is characterised as abnormal accumulation of serine protease inhibitors (SERPINs) in cells and results in clinical symptoms owing to lack of SERPIN function or excessive accumulation of abnormal SERPIN. We recently identified a patient with functional deficiency of plasminogen activator inhibitor-1 (PAI-1), a member of the SERPIN superfamily. The patient exhibited life-threatening bleeding tendencies, which have also been observed in patients with a complete deficiency in PAI-1. Sequence analysis revealed a homozygous single-nucleotide substitution from guanine to cytosine at exon 9, which changed amino acid residue 397 from glycine to arginine (c...
February 23, 2017: Thrombosis and Haemostasis
https://www.readbyqxmd.com/read/28223734/early-thrombomodulin-%C3%AE-administration-outcome-for-acute-disseminated-intravascular-coagulopathy-in-gastrointestinal-surgery
#11
Hirotaka Konishi, Kazuma Okamoto, Katsutoshi Shoda, Tomohiro Arita, Toshiyuki Kosuga, Ryo Morimura, Shuhei Komatsu, Yasutoshi Murayama, Atsushi Shiozaki, Yoshiaki Kuriu, Hisashi Ikoma, Masayoshi Nakanishi, Daisuke Ichikawa, Hitoshi Fujiwara, Eigo Otsuji
AIM: To investigate the efficacy of thrombomodulin (TM)-α for treatment of disseminated intravascular coagulopathy (DIC) in the field of gastrointestinal surgery. METHODS: Thirty-six peri-operative DIC patients in the field of gastrointestinal surgery who were treated with TM-α were retrospectively investigated. The relationships between patient demographics and the efficacy of TM-α were examined. Analysis of survival at 28 d was also performed on some parameters by means of the Kaplan-Meier method...
February 7, 2017: World Journal of Gastroenterology: WJG
https://www.readbyqxmd.com/read/28217222/non-vitamin-k-antagonist-oral-anticoagulants-versus-warfarin-for-cardioversion-of-atrial-fibrillation-in-clinical-practice-a-single-center-experience
#12
Naoki Shibata, Itsuro Morishima, Kenji Okumura, Yasuhiro Morita, Kensuke Takagi, Ruka Yoshida, Hiroaki Nagai, Toshiro Tomomatsu, Yoshihiro Ikai, Kazushi Terada, Kazuhito Tsuzuki, Hideyuki Tsuboi, Takahito Sone, Toyoaki Murohara
BACKGROUND: Anticoagulation therapy with the vitamin K antagonist (VKA) warfarin has been demonstrated to reduce thromboembolic risk after electrical cardioversion (ECV). However, data concerning ECV with non-VKA oral anticoagulants (NOACs) is limited. The objective of this study was to determine the efficacy and safety of NOACs in patients undergoing ECV in a real-world clinical practice at a single center in Japan. METHODS: We retrospectively analyzed the data of 406 consecutive patients who underwent ECV for atrial fibrillation (AF) or flutter under anticoagulation with one of the three NOACs (n=149) or with a VKA (n=257)...
February 2017: Journal of Arrhythmia
https://www.readbyqxmd.com/read/28214640/the-art-of-mimicry-anterior-clinoid-dural-based-cavernous-hemangioma-mistaken-for-a-meningioma
#13
Tarek R Mansour, Yacine Medhkour, Pouya Entezami, Robert Mrak, Jason Schroeder, Azedine Medhkour
BACKGROUND: Cavernous hemangiomas account for 5%-13% of central nervous system vascular lesions. They are usually found intra-axially but rarely involve extra-axial structures, most commonly the middle cranial fossa. A cavernous hemangioma manifesting as a clinoid meningioma is extremely rare. CASE DESCRIPTION: A 36-year-old man presented with an asymptomatic intracranial mass found incidentally after a motor vehicle accident. Preoperative magnetic resonance imaging revealed a well-defined dural-based lesion arising from the right anterior clinoid process that was nearly homogeneously enhancing...
February 15, 2017: World Neurosurgery
https://www.readbyqxmd.com/read/28210401/zygomatic-intraosseous-hemangioma-case-report-and-literature-review
#14
REVIEW
David B Powers, Elda Fisher, Detlev Erdmann
Intraosseous hemangiomas are uncommon intrabony lesions, representing approximately 0.5 to 1% of all intraosseous tumors. Their description varies from "benign vasoformative neoplasms" to true hamartomatous proliferations of endothelial cells forming a vascular network with intermixed fibrous connective tissue stroma. These commonly present as a firm, painless swelling. Intraosseous hemangiomas present more commonly in females than in males and most likely occur in the fourth decade of life. The most common etiology of intraosseous hemangioma is believed to be prior trauma to the area...
March 2017: Craniomaxillofacial Trauma & Reconstruction
https://www.readbyqxmd.com/read/28205396/can-the-diagnostic-reliability-of-the-thrombin-generation-test-as-a-global-haemostasis-assay-be-improved-the-impact-of-calcium-chloride-concentration
#15
L A Parunov, S S Surov, Y Liang, T K Lee, M V Ovanesov
BACKGROUND: Thrombin generation test (TGT) is a global haemostasis assay with a potential to predict bleeding tendencies and treatment effects in patients with haemophilia. Despite 15 years of clinical research, the diagnostic value of TGT remains controversial, possibly due to suboptimal sensitivity to coagulation deficiencies, robustness and reproducibility. OBJECTIVE: The goal of this study was to explore the effect of calcium chloride (CaCl2 ) concentration on the TGT's response to intrinsic coagulation factors (F) VIII, IX and XIa...
February 15, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28199463/effect-of-methyl-aminolevulinate-photodynamic-therapy-with-and-without-ablative-fractional-laser-treatment-in-patients-with-microinvasive-squamous-cell-carcinoma-a-randomized-clinical-trial
#16
Seung-Hwan Choi, Ki-Ho Kim, Ki-Hoon Song
Importance: Surgical excision is the standard treatment for cutaneous squamous cell carcinoma (SCC). However, microinvasive SCC (Clark level II) is limited to the papillary dermis, and it should be differentiated from invasive SCC. Ablative fractional laser-primed photodynamic therapy (AFL-PDT) may have enhanced efficacy. Objective: To compare 1 session of AFL-PDT with 2 sessions of conventional methyl aminolevulinate-PDT (MAL-PDT) for the treatment of microinvasive SCC...
March 1, 2017: JAMA Dermatology
https://www.readbyqxmd.com/read/28195875/endoscopic-variceal-ligation-combined-with-argon-plasma-coagulation-versus-ligation-alone-for-the-secondary-prophylaxis-of-variceal-bleeding-a-systematic-review-and-meta-analysis
#17
Xiaoyan Li, Tingting Jiang, Jian Gao
Endoscopic variceal ligation (EVL) is often recommended as an effective method for the treatment of esophageal varices, despite the important tendency of variceal recurrence. Recent studies indicate that combining EVL with argon plasma coagulation (APC) may be a more effective therapy than ligation alone. To investigate these findings, we carried out a systematic review and meta-analysis to compare the safety and efficacy of EVL combined with APC versus ligation alone for the secondary prophylaxis of esophageal variceal hemorrhage...
February 13, 2017: European Journal of Gastroenterology & Hepatology
https://www.readbyqxmd.com/read/28182647/performance-analysis-of-a-machine-learning-flagging-system-used-to-identify-a-group-of-individuals-at-a-high-risk-for-colorectal-cancer
#18
Yaron Kinar, Pinchas Akiva, Eran Choman, Revital Kariv, Varda Shalev, Bernard Levin, Steven A Narod, Ran Goshen
Individuals with colorectal cancer (CRC) have a tendency to intestinal bleeding which may result in mild to severe iron deficiency anemia, but for many colon cancer patients hematological abnormalities are subtle. The fecal occult blood test (FOBT) is used as a pre-screening test whereby those with a positive FOBT are referred to colonscopy. We sought to determine if information contained in the complete blood count (CBC) report coud be processed automatically and used to predict the presence of occult colorectal cancer (CRC) in the setting of a large health services plan...
2017: PloS One
https://www.readbyqxmd.com/read/28176962/renal-angiomyolipoma-in-pregnancy-surgical-management-with-fetal-preservation-approach-in-a-developing-setting
#19
Fred O Ugwumba, Emeka F Nnakenyi, Okechukwu C Okafor, Augustine C Onuh, Paschalina C Ezechukwu, Sunday Urube
Renal angiomyolipomas (RAML) are uncommon benign renal tumours that are associated with a tendency to rupture resulting in sometimes-torrential retroperitoneal hemorrhage as the Wunderlich syndrome or as severe potentially exsanguinating hematuria. When hemorrhage from RAML occurs in pregnancy it presents a unique challenge requiring timely and appropriately adapted intervention with the goal of preventing fatality, preserving renal function as well as preventing fetal loss if possible. We report the management of severe bleeding from RAML in pregnancy and highlight the need to adopt a management strategy that suits the practice environment and offers the patient standard and enduring care...
October 24, 2016: Clinics and Practice
https://www.readbyqxmd.com/read/28176610/bleeding-manifestations-in-heterozygotes-with-congenital-fvii-deficiency-a-comparison-with-unaffected-family-members-during-a-long-observation-period
#20
Antonio Girolami, Elisabetta Cosi, Silvia Ferrari, Bruno Girolami, Anna Maria Lombardi
OBJECTIVES: To determine whether heterozygotes with FVII deficiency have a bleeding tendency or not. PATIENTS AND METHODS: Eighty-four patients (OK) heterozygous for FVII deficiency, at the onset of the study, were paired with unaffected family members and followed for a long period of time (mean 22.6 years) for the occurrence of bleeding. Diagnosis of heterozygosis had to be based on family studies, clotting, immunological assays and genetic analysis. RESULTS: The mean FVII activity level was 0...
February 8, 2017: Hematology (Amsterdam, Netherlands)
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