keyword
https://read.qxmd.com/read/38650319/relationship-between-plasma-tissue-factor-pathway-inhibitor-tfpi-levels-thrombin-generation-and-clinical-risk-of-bleeding-in-patients-with-severe-haemophilia-a-or-b
#1
JOURNAL ARTICLE
Brigitte Tardy-Poncet, Aurélie Montmartin, Hervé Chambost, Anne Lienhart, Birgit Frotscher, Pierre-Emmanuel Morange, Céline Falaise, Fanny Collange, Yesim Dargaud, Marie Toussaint-Hacquard, Laurent Ardillon, Bénédicte Wibaut, Emmanuelle Jeanpierre, Philippe Nguyen, Fabienne Volot, Bernard Tardy
INTRODUCTION: Bleeding severity in severe haemophilic patients, with low thrombin generation (TG) capacity, can vary widely between patients, possibly reflecting differences in tissue factor pathway inhibitor (TFPI) level. AIM: To compare free TFPI (fTFPI) levels in patients with severe haemophilia A (sHA) and severe haemophilia B (sHB) and to investigate in these patients as a whole the relationships between bleeding and TG potential, between TG potential and fTFPI level and between fTFPI level and bleeding tendency...
April 22, 2024: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://read.qxmd.com/read/38620079/fitusiran-reduces-bleeding-in-factor-x-deficient-mice
#2
JOURNAL ARTICLE
Sebastien Verhenne, Genevieve McCluskey, Hortense Maynadié, Frédéric Adam, Caterina Casari, Laurence Panicot-Dubois, Lydie Crescence, Christophe Dubois, Cecile V Denis, Peter J Lenting, Olivier D Christophe
Factor X (FX)-deficiency is a rare bleeding disorder manifesting a bleeding tendency caused by low FX activity levels. We aimed to explore the use of fitusiran (an investigational siRNA that silences antithrombin expression) to increase thrombin generation and the in vivo hemostatic potential under conditions of FX-deficiency. We therefore developed a novel model of inducible FX-deficiency, generating mice expressing <1% FX activity and antigen (f10low-mice). Compared to control f10WT-mice, f10low-mice had 6- and 4-fold prolonged clotting times in Prothrombin Time- and activated Partial Prothrombin Time-assays, respectively (p<0...
April 15, 2024: Blood
https://read.qxmd.com/read/38602530/symptomatic-corpus-luteum-hemorrhage-in-adolescent-females-with-itp
#3
JOURNAL ARTICLE
Alexander Yelak, Anat From, Oded Gilad, Dafna Brik Simon, Shiri Rubin, Miriam Cohen, Gil Amarilyo, Carina Levin, Doua Bakry, Shai Izraeli, Hannah Tamary, Joanne Yacobovich, Orna Steinberg-Shemer
Patients with immune thrombocytopenia (ITP) usually present with minor mucocutaneous bleeding. Corpus luteum hemorrhage (CLH) is generally asymptomatic but may, rarely, lead to severe intraperitoneal bleeding, mostly in patients with coagulation disorders. CLH causing intraperitoneal bleeding has only been described in few individuals with ITP. The objective of this retrospective observational study was to assess the clinical course and incidence of symptomatic CLH in adolescent females with newly diagnosed or chronic ITP...
April 11, 2024: European Journal of Pediatrics
https://read.qxmd.com/read/38600006/guidelines-for-the-management-of-coagulation-disorders-in-patients-with-cirrhosis
#4
I Aiza-Haddad, L E Cisneros-Garza, O Morales-Gutiérrez, R Malé-Velázquez, M T Rizo-Robles, R Alvarado-Reyes, L A Barrientos-Quintanilla, F Betancourt-Sánchez, E Cerda-Reyes, R Contreras-Omaña, M B Dehesa-Violante, N C Flores-García, D Gómez-Almaguer, M F Higuera-de la Tijera, M A Lira-Pedrin, J E Lira-Vera, H Manzano-Cortés, D E Meléndez-Mena, M R Muñoz-Ramírez, J L Pérez-Hernández, M V Ramos-Gómez, J F Sánchez-Ávila
Coagulation management in the patient with cirrhosis has undergone a significant transformation since the beginning of this century, with the concept of a rebalancing between procoagulant and anticoagulant factors. The paradigm that patients with cirrhosis have a greater bleeding tendency has changed, as a result of this rebalancing. In addition, it has brought to light the presence of complications related to thrombotic events in this group of patients. These guidelines detail aspects related to pathophysiologic mechanisms that intervene in the maintenance of hemostasis in the patient with cirrhosis, the relevance of portal hypertension, mechanical factors for the development of bleeding, modifications in the hepatic synthesis of coagulation factors, and the changes in the reticuloendothelial system in acute hepatic decompensation and acute-on-chronic liver failure...
April 9, 2024: Revista de Gastroenterología de México
https://read.qxmd.com/read/38595261/-vertebral-fractures-combined-with-prolonged-activated-partial-prothrombin-time-a-case-report
#5
JOURNAL ARTICLE
Xinzhu Bai, Jinhui He, Songsong Lu, Chun Li, Yilin Wang, Jian Xiong
With the development of modern medical standards, autoimmune diseases and their associated successive osteoporosis have received increasing attention in recent years. Patients with autoimmune diseases, due to the characteristics of the disease and the prolonged use of glucocorticoid hormone therapy, may affect the bone formation and bone absorption of the patient, followed by severe successive osteoporosis, thereby increasing the risk of osteoporotic vertebral fractures. Vertebral compression fractures of the spine are common fracture types in patients with osteoporotic fractures...
April 18, 2024: Beijing da Xue Xue Bao. Yi Xue Ban, Journal of Peking University. Health Sciences
https://read.qxmd.com/read/38594875/actn1-related-thrombocytopenia-homozygosity-for-an-actn1-variant-results-in-a-more-severe-phenotype
#6
JOURNAL ARTICLE
Melania Eva Zanchetta, Serena Barozzi, Federica Isidori, Caterina Marconi, Loredana Farinasso, Roberta Bottega, Anna Savoia, Alessandro Pecci, Michela Faleschini
ACTN1-related thrombocytopenia is a rare disorder caused by heterozygous variants in the ACTN1 gene characterized by macrothrombocytopenia and mild bleeding tendency. We describe for the first time two patients affected with ACTN1-RT caused by a homozygous variant in ACTN1 (c.982G>A) with mild heart valve defects unexplained by any other genetic variants investigated by WES. Within the reported family, the homozygous sisters have moderate thrombocytopenia and marked platelet macrocytosis with giant platelets, revealing a more severe haematological phenotype compared to their heterozygous relatives and highlighting a significant effect of allelic burden on platelet size...
April 9, 2024: British Journal of Haematology
https://read.qxmd.com/read/38564005/bernard-soulier-syndrome-caused-by-two-novel-heterozygous-gp1ba-gene-mutations-a-case-report-and-literature-review
#7
REVIEW
Senlin Zhang, Jing Ling, Kai Cui, Shihong Zhan, Jiajia Zheng, Wenyi Wang, Junjie Fan, Shaoyan Hu
BACKGROUND: Bernard-Soulier syndrome (BSS) is a rare inherited macrothrombocytopenia, usually autosomal recessive, which is characterized by prolonged bleeding, thrombocytopenia, and abnormally large platelets. METHODS: For more than 6 years, we misdiagnosed a patient with BSS without an obvious bleeding tendency as having idiopathic thrombocytopenia purpura (ITP), prior to obtaining a genetic analysis. On admission, routine hematology showed a platelet count of 30 × 109 /L and mean platelet volume (MPV) of 14...
December 2024: Hematology (Amsterdam, Netherlands)
https://read.qxmd.com/read/38562627/fracture-management-in-chronic-kidney-disease-challenges-and-considerations-for-orthopedic-surgeons
#8
REVIEW
Wan Kee Hong, Sejoong Kim, Hyun Sik Gong
Orthopedic surgeons treating fractures need to consider comorbidities, including chronic kidney disease (CKD), which affects millions worldwide. CKD patients are at elevated risk of fractures due to osteoporosis, especially in advanced stages. In addition, fractures in CKD patients pose challenges due to impaired bone healing and increased post-fracture complications including surgical site infection and nonunion. In this article, we will discuss factors that must be considered when treating fractures in CKD patients...
April 2024: Clinics in Orthopedic Surgery
https://read.qxmd.com/read/38559558/risk-factors-analysis-and-the-establishment-of-nomogram-prediction-model-for-picc-related-venous-thrombosis-in-patients-with-lymphoma-a-double-center-cohort-based-case-control-study
#9
JOURNAL ARTICLE
Xue-Xing Wang, Yuan He, Jie Chu, Jin-Song Xu
OBJECTIVE: The objective of this study is to examine the risk factors associated with the occurrence of PICC-Related Venous Thrombosis (PICC-RVTE) in individuals diagnosed with lymphoma, as well as to develop a predictive risk nomogram model. METHODS: A total of 215 patients with lymphoma treated at Yunnan Provincial Tumor Hospital from January 2017 to December 2020 were retrospectively evaluated as the training cohort; 90 patients with lymphoma treated at the Department of Oncology of the First People's Hospital of Anning, Affiliated to Kunming University of Science and Technology during the January 2021 to September 2023 were evaluated as the validation cohort...
2024: Frontiers in Oncology
https://read.qxmd.com/read/38552250/versatile-and-remotely-controllable-light-induced-coagulation-of-particles-under-flow-in-a-2d-channel
#10
JOURNAL ARTICLE
Daniela Vasquez-Muñoz, Fabian Rohne, Anjali Sharma, Nino Lomadze, Svetlana Santer, Marek Bekir
On-demand switch on/off blood clogging is of paramount importance for the survival of mammals, for example as a quick response to seal damage wounds to minimize their bleeding rate. This mechanism is a complex chain process from initiated red blood cell aggregation at the target location (open wound) that quickly seals on a macroscopic scale the damaged flash. Inspired by nature an on-demand switchable particle clogging mechanism is developed with high spatial resolution down to micrometer size using light as an external non-invasive stimulation...
March 29, 2024: Small
https://read.qxmd.com/read/38552183/discovery-of-2-3-dihydro-1-4-dioxino-2-3-g-benzofuran-derivatives-as-protease-activated-receptor-4-par4-antagonists-with-potent-antiplatelet-aggregation-activity-and-low-bleeding-tendency
#11
JOURNAL ARTICLE
Panpan Chen, Cai Chen, Yizheng Zheng, Fangjun Chen, Zhaojun Liu, Shenhong Ren, Hangyu Song, Tongdan Liu, Zhipeng Lu, Hongbin Sun, Yi Kong, Haoliang Yuan
Patients with arterial embolic disease have benefited greatly from antiplatelet therapy. However, hemorrhage risk of antiplatelet agents cannot be ignored. Herein, we describe the discovery of 2,3-dihydro[1,4]dioxino[2,3- g ]benzofuran compounds as novel PAR4 antagonists. Notably, the isomers 36 and 37 with the chemotype of phenoxyl methylene substituted on the 2,3-dihydro-1,4-dioxine ring exhibited potent in vitro antiplatelet activity (IC50 = 26.13 nM for 36 and 14.26 nM for 37 ) and significantly improved metabolic stability in human liver microsomes ( T 1/2 = 97...
March 29, 2024: Journal of Medicinal Chemistry
https://read.qxmd.com/read/38548639/clinical-evaluation-of-resorbable-polylactic-acid-pla-intracanal-posts-for-primary-incisor-restoration-randomized-controlled-clinical-trial
#12
JOURNAL ARTICLE
Hua Xu, Xuefang Chen, Junrong Wang, Qian Zou, Fengna Qi, Xiaolan Ma
This randomized, controlled clinical trial compares the clinical performance of glass-fibre and resorbable polylactic acid (PLA) intracanal posts used to restore carious primary incisors in young patients. The study sample includes 180 primary upper central incisors of 90 children aged 3 to 4 years. All patients were randomly divided into two equal groups of 45 children who received PLA and glass-fibre (GFP) intracanal posts. The clinical assessment of incisor restorations was carried out immediately upon completion and at months 3, 6 and 12 according to the following criteria: anatomical form, marginal adaptation, surface roughness, marginal pigmentation, colour match, secondary caries and contact point...
March 2024: Journal of Clinical Pediatric Dentistry
https://read.qxmd.com/read/38546342/a-low-bleeding-risk-thrombolytic-agent-citpa5
#13
JOURNAL ARTICLE
Shanli Chen, Sudan Fang, Yang Zhou, Zhiwei Huang, Shujuan Yu, Dan Chen, Zhiyou Wang, Yanyan Xu, Peiwen Liu, Yongkun Li, Wei Lin, Longguang Jiang, Cai Yuan, Mingdong Huang
AIMS: Alteplase is a cornerstone thrombolytic agent in clinical practice, but presents a potential bleeding risk. Stroke patients need pre-screening to exclude hemorrhagic stroke before using Alteplase. In this study, we develop a new thrombolytic agent citPA5, characterized by an enhanced safety profile and minimal bleeding tendency. METHODS AND RESULTS: A clot lysis agent, named citPA5, is developed based on rtPA with point mutations to completely suppress its proteolytic activity in the absence of fibrin...
March 28, 2024: Cardiovascular Research
https://read.qxmd.com/read/38525959/the-effect-of-abcb1-and-ces1-polymorphisms-on-plasma-levels-of-dabigatran-and-risk-of-hemorrhagic-complications-in-ischemic-stroke-patients
#14
JOURNAL ARTICLE
Olšerová Anna, Janský Petr, Magerová Hana, Šrámková Tereza, Kešnerová Petra, Kmetonyová Silvia, Šulc Vlastimil, Halmová Hana, Šrámek Martin, Šarbochová Ivana, Paulasová-Schwabová Jaroslava, Benešová Kateřina, Macek Jan, Maťoška Václav, Tomek Aleš
BACKGROUND: Dabigatran directly inhibits thrombin and is used in primary and secondary stroke prevention in individuals with nonvalvular atrial fibrillation. The prodrug dabigatran etexilate is absorbed by enteral P-glycoprotein (ABCB1) and then activated by hepatic and intestinal carboxylesterases (CES1) to produce active metabolites. Variations in dabigatran metabolism because of genetics may affect concentration levels and clinical outcomes. STUDY QUESTION: We conducted a study to assess how polymorphisms in the CES1 (rs2244613) and ABCB1 (rs4148738) genes affect the through plasma level (cmin) of dabigatran and its correlation to clinical outcomes...
March 20, 2024: American Journal of Therapeutics
https://read.qxmd.com/read/38523974/a-case-of-scurvy-associated-with-intracerebral-hemorrhage-in-a-patient-with-alcohol-use-disorder
#15
Yohei Masuda, Kuniyasu Saigusa, Yasuhiro Norisue
Vitamin C deficiency, also known as scurvy, causes abnormalities in connective tissues and varied symptoms. We describe a patient with putaminal hemorrhage, a very rare presentation of scurvy. A 39-year-old man presented with weakness in the left arm and left leg. Right putaminal hemorrhage was initially diagnosed, and he underwent evacuation of the intracerebral hemorrhage. Scurvy was suspected when repeated physical examinations revealed a bleeding tendency and multiple untreated dental caries, missing teeth, and gingivitis...
February 2024: Curēus
https://read.qxmd.com/read/38505580/management-of-severe-factor-xi-deficiency-in-pregnancy-a-case-report
#16
Fatima El Hassouni, Asmae Bentaleb, Sofia Lahbabi, Rajae Tachinante, Mohamed Elkhorassani, Mounia Yousfi
Factor XI (FXI) deficiency is one of the rarest coagulation disorders with a frequency of 1:1,000,000. A 30-year-old woman, diagnosed with FXI deficiency at the age of 4, was admitted to our maternity service at 25 weeks of gestation. The patient had no history of hemorrhage or abnormal bleeding, and the clinical examination was unremarkable. Antenatal care was also normal. The parturient was admitted in early labor at 38 weeks of gestation. Despite the absence of clinical hemorrhagic syndrome, a transfusion of fresh frozen plasma combined with tranexamic acid was initiated once the active stage of labor was started...
2024: SAGE Open Medical Case Reports
https://read.qxmd.com/read/38494557/clinical-observation-of-hypofibrinogenemia-induced-by-the-treatment-of-tocilizumab-in-rheumatic-diseases-and-exploration-of-risk-factor-for-hypofibrinogenemia
#17
JOURNAL ARTICLE
Qi An, Rulan Ma, Dawei Yuan, Jing Huang, Jing Luo, Yanhua Wang, Ying Pan, Pei Wang, Xiaohong Lv, Dan Pu, Lan He
OBJECTIVE: The objective of this study was to analyze the changes in plasma fibrinogen (FIB) levels during tocilizumab (TCZ) treatment in patients with rheumatic diseases, to clarify the incidence of hypofibrinogenemia and its possible risk factors, and to establish a nomogram model for predicting the probability of hypofibrinogenemia in rheumatoid arthritis (RA) patients treated with TCZ. METHODS: Clinical data of patients treated with TCZ at the Department of Rheumatology and Immunology, the First Affiliated Hospital of Xi'an Jiaotong University from January 2014 to October 2021 were retrospectively analyzed to observe the incidence of hypofibrinogenemia in several rheumatic diseases at different time points...
March 18, 2024: Clinical Rheumatology
https://read.qxmd.com/read/38481322/acute-type-a-aortic-intramural-hematoma-complicated-with-preoperative-hemopericardium-early-and-late-surgical-outcome-analyses
#18
JOURNAL ARTICLE
Chun-Yu Lin, Ming-Chang Kao, Hsin-Fu Lee, Meng-Yu Wu, Chi-Nan Tseng
BACKGROUND: Acute type A aortic intramural hematoma (ATAIMH) is a variant of acute type A aortic dissection (ATAAD), exhibiting an increased risk of hemopericardium and cardiac tamponade. It can be life-threatening without emergency treatment. However, comprehensive studies of the clinical features and surgical outcomes of preoperative hemopericardium in patients with ATAIMH remain scarce. This retrospective study aims to investigate the clinical features and early and late outcomes of patients who underwent aortic repair surgery for ATAIMH complicated with preoperative hemopericardium...
March 13, 2024: Journal of Cardiothoracic Surgery
https://read.qxmd.com/read/38477834/frequency-clinical-and-laboratory-findings-of-platelet-secretion-disorders-in-patients-referred-to-the-specialized-coagulation-laboratory-of-the-iranian-blood-transfusion-organization
#19
JOURNAL ARTICLE
Massoumeh Shahbazi, Minoo Ahmadinejad, Vahid Pirhajati Mahabadi, Amir Teimourpour, Khadijeh Golzadeh
OBJECTIVES: Platelet secretion disorders (PSDs) are a subgroup of platelet function disorders (PFDs) caused by defects in the content or release of platelet granules. These patients have a variable degree of mucocutaneous bleeding tendency. The diagnostic facilities of PSDs are imitated in Iran, even in specialized coagulation laboratories. The present study aims to estimate the frequency of PSDs among patients referred to the Iranian Blood Transfusion Organization (IBTO). METHODS: The research population includes all patients referred to the specialized coagulation laboratory of IBTO and requested platelet function and von Willebrand testing by their physicians...
March 12, 2024: Blood Coagulation & Fibrinolysis: An International Journal in Haemostasis and Thrombosis
https://read.qxmd.com/read/38459580/protocol-of-a-phase-ii-study-to-evaluate-the-efficacy-and-safety-of-deep-inspiration-breath-hold-daily-online-adaptive-radiotherapy-for-centrally-located-lung-tumours-pudding-study
#20
JOURNAL ARTICLE
Noriko Kishi, Masahiro Yoneyama, Hiroyuki Inoo, Minoru Inoue, Hiraku Iramina, Akiyoshi Nakakura, Tomohiro Ono, Hideaki Hirashima, Takanori Adachi, Norimasa Matsushita, Makoto Sasaki, Takahiro Fujimoto, Mitsuhiro Nakamura, Yukinori Matsuo, Takashi Mizowaki
BACKGROUND: Centrally located lung tumours present a challenge because of their tendency to exhibit symptoms such as airway obstruction, atelectasis, and bleeding. Surgical resection of these tumours often requires sacrificing the lungs, making definitive radiotherapy the preferred alternative to avoid pneumonectomy. However, the proximity of these tumours to mediastinal organs at risk increases the potential for severe adverse events. To mitigate this risk, we propose a dual-method approach: deep inspiration breath-hold (DIBH) radiotherapy combined with adaptive radiotherapy...
March 8, 2024: Radiation Oncology
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