Clinical and Applied Thrombosis/Hemostasis最新文献

Hemophilia B (HB) is an inherited bleeding disorder caused by defects in the FⅨ gene, leading to severe coagulation dysfunction. This study designed eight pairs of primers covering eight exons of the FⅨ gene and used PCR and DNA sequencing to detect FⅨ gene mutations in 31 HB patients. Sequencing results were compared with normal sequences using Chromas software on Blast to identify mutation sites. Findings revealed the CpG dinucleotide region as a mutation hotspot and the 192nd nucleotide (FⅨ192) as a dinucleotide polymorphism site in the Chinese population. Pathogenic mutations included point mutations, deletions, insertions, and mutations affecting amino acids or splicing sites. For cases with only polymorphic sites, further exon sequencing is needed. This study adds new mutation data to the global HB database, supports research on racial differences in FⅨ gene mutations, and contributes to domestic HB statistics. The results aid in understanding the FⅨ gene's role in coagulation, elucidating HB pathogenesis, and providing a basis for future gene therapy.

Post-thrombotic syndrome (PTS) is one of the most common long-term complications of lower extremity deep vein thrombosis (DVT). In order to study the long-term adverse prognosis of patients with DVT, explore the influencing factors for the prognosis of DVT, and provide a reliable reference for future research in the field of venous thrombosis, we collected and summarized information about the incidence of PTS, the PTS score and grading, the associated symptoms and drug-related adverse reactions in 501 patients with DVT. In our study, 54.1% of patients with DVT (271 of 501) experienced indications and manifestations of PTS, the male to female ratio was approximately 1:1. During the long-term follow up, the most common symptoms of PTS were anterior tibial edema and pain. By statistical analysis, we found that the outcome of thrombosis was the influencing factor of PTS score (1-4 points, P<.05). The grading of PTS was primarily influenced by the history of varicose veins and DVT in the lower extremities. The duration of taking antithrombotic drugs affected the outcome of thrombosis (P<.05), especially among the female patients. In addition, varied factors, such as lower extremity DVT complicated with pulmonary embolism and the duration of antithrombotic drug use were found to increase the chances of experiencing drug-related adverse reactions (odds ratio [OR]=2.798, 95% confidence interval [CI]: 1.413-5.541 / OR=2.778, 95% CI: 1.231-6.269). The above 2 factors were significant only among female patients with DVT (OR=4.03, 95% CI: 1.608-10.103 / OR=3.918, 95% CI: 1.123-13.669).

Background: Since several studies have examined the use of direct oral anticoagulants (DOACs) in treating patients with splanchnic vein thrombosis (SVT), we conducted a meta-analyses to assess the safety and efficacy of DOACs compared to vitamin K antagonists (VKAs) in this population.

Methods: We conducted a comprehensive search using the PubMed, Embase, and Cochrane Library databases until June 2024. We used odds ratios (ORs) and 95% confidence intervals (CIs) as the effect measures to compare DOACs with VKAs.

Results: A total of 9 observational studies were included. The pooled analysis revealed that a trend towards higher complete recanalization rates with DOACs (71.4%) compared to VKAs (55.3%), though not statistically significant (OR 1.95; 95%CI 0.70 to 5.44). For SVT extension, a significant effect was observed favoring DOACs (OR 0.12; 95%CI 0.03 to 0.54). No significant differences were found in other efficacy outcomes or safety outcomes, except for major bleeding, which was significantly lower with DOACs (OR 0.27; 95%CI 0.13 to 0.56).

Conclusion: DOACs are superior to VKAs in SVT extension and major bleeding, suggesting that DOACs may be a favorable treatment option in the treatment of SVT.

The Knops blood group system is an independent blood group system recognized by International Society of Blood Transfusion (ISBT) in 1992, and latest time consisting of 13 antigens carried on a glycoprotein of 2489 amino acids and called the Complement C3b/C4b Receptor 1 (CR1). Erythrocyte KN antigen was first reported in 1970, and CR1 is a protein coding gene that is a member of the receptors of complement activation (RCA) family and is located in the "cluster RCA" region of chromosome 1. CR1 is an important participant in the erythrocyte immune machinery and plays an major role in inhibiting complement activation, and polymorphisms in its expression have been closely associated with a variety of diseases, including systemic lupus erythematosus (SLE), malaria, Plasmodium falciparum malaria, Alzheimer's disease (AD) and leprosy. Antibodies to the Knops system usually do not bind to complement and do not cause a hemolytic reaction. However, anti-Knops antibodies can be detected in the serum of some pregnant women. Generally, however, they only test positive by direct antiglobulin test (DAT) and most of them do not cause hemolytic disease of the newborn (HDN). This article is a review of the progress of the Knops blood group system.

Objective: This study aimed to investigate the association between the triglyceride-glucose (TyG) index and cryptogenic stroke (CS) in patients diagnosed with patent foramen ovale (PFO) using transesophageal echocardiography (TEE).

Methods: A retrospective, single-center study was conducted at a tertiary education and research hospital from January 2015 to December 2023. The study population included 1017 consecutive patients with sinus rhythm diagnosed with PFO, of whom 210 had CS. The TyG Index was calculated using triglyceride and fasting glucose levels.

Results: Patients with CS were older (47.53 ± 12.34 years) compared to control group (44.40 ± 17.82 years, p = .005). The proportion of males was higher in the CS group (56.2%) compared to the control group (48.3%, p = .042). Laboratory findings revealed higher TyG Index (8.87 ± 0.51 vs 8.63 ± 0.55, p < .001) in patients with CS. TyG Index was an independent predictor of CS in patients with PFO (OR: 2.832, 95%CI: 1.979-4.053 p < .001).

Conclusion: Elevated TyG Index levels was associated with CS in patients with PFO. The TyG index may serve as a useful biomarker for assessing CS risk in this population.

Introduction: Patients with ischemic stroke (IS) and atrial fibrillation (AF) face a higher risk of recurrent vascular events. This study evaluates the impact of atherosclerotic vascular disease burden across different vascular territories on the risk of vascular events in patients with recent ischemic stroke and AF within 90 days. Patients and Methods: We included patients with IS and AF from the International RAF network in a prospective 90-day follow-up. Atherosclerotic vascular disease was identified by at least one of the following: Symptomatic ischemic heart disease, symptomatic peripheral artery disease, internal carotid stenosis ≥50%, or the presence of plaques in the aorta. The primary outcome was a composite of stroke, transient ischemic attack, systemic embolism, cerebral bleeding, and major extracranial bleeding within 90 days postacute stroke. Patients were categorized into 5 groups based on the number of affected atherosclerotic vascular territories, with those with no atherosclerotic vascular disease as the reference. Kaplan-Meier curves were generated and compared using the log-rank test to determine the predictive value of the number of diseased territories for the risk of events. Data analysis was performed with SPSS/PC Win Package 25.0. Results: Of the 2148 patients (mean age 77.59; 53.86% female), 744 (34.60%) had atherosclerosis. Multivariable analysis revealed that involvement of 3 (hazard ratio [HR] 2.80, 95% confidence interval [CI]: 1.20-6.53) or 4 (HR 6.81, 95% CI: 1.02-36.24) vascular territories was significantly associated with the risk of combined events. Conclusions: In patients with recent ischemic stroke and AF, atherosclerosis across multiple territories correlates with a higher risk of future vascular events.

European real-world data indicate that front-line treatment with caplacizumab is associated with improved clinical outcomes compared with delayed caplacizumab treatment. The objective of the study was to describe the characteristics, treatment patterns, and outcomes in hospitalized patients with an immune-mediated thrombotic thrombocytopenic purpura (iTTP) episode treated with front-line versus delayed caplacizumab in the US. This retrospective cohort analysis of a US hospital database included adult patients (≥18 years) with an acute iTTP episode (a diagnosis of thrombotic microangiopathy and ≥1 therapeutic plasma exchange [TPE] procedure) from January 21, 2019, to February 28, 2021. Unadjusted baseline characteristics, treatment patterns, healthcare resource utilization, and costs were compared between patients who received front-line versus delayed (<2 vs ≥2 days after TPE initiation) caplacizumab treatment. Out of 39 patients, 16 (41.0%) received front-line and 23 (59.0%) received delayed treatment with caplacizumab. Baseline characteristics and symptoms were similar between the two groups. Patients who received front-line caplacizumab treatment had significantly fewer TPE administrations (median: 5.0 vs 12.0); and a significantly shorter hospital stay (median: 9.0 days vs 16.0 days) than patients receiving delayed caplacizumab therapy. Both of these were significantly lower in comparison of means (t-test P < .01). Median inpatient costs (inclusive of caplacizumab costs) were 54% higher in the delayed treated patients than in the front-line treated patients (median: $112 711 vs $73 318). TPE-specific cost was lower in the front-line treated cohort (median: $6 989 vs $10 917). In conclusion, front-line treatment with caplacizumab had shorter hospitalizations, lower healthcare resource utilization, and lower costs than delayed caplacizumab treatment after TPE therapy.

This study aimed to investigate the influence of prothrombotic risk factors on long-term outcomes of patients with perinatal arterial ischemic stroke. The study was conducted through an analysis of monitoring results that were regularly maintained for approximately 20 years at a tertiary stroke-monitoring center. The study assessed prothrombotic risk factors, radiological area of involvement, clinical presentation, treatments, clinical outcomes, and long-term outcomes of the 48 patients included in the study, with a mean monitoring time of 77.6 ± 45.7 months (range: 6-204). Our results showed that the presence of prothrombotic risk factors did not affect long-term outcomes. However, patients with middle cerebral artery infarction had the highest risk of developing cerebral palsy, whereas those with presumed stroke had the highest risk of developing epilepsy. This study suggests that prothrombotic risk factors should not be evaluated during the acute stage unless there is a strong suspicion of the patient's history, and prevention or early diagnosis of presumed stroke patients will positively impact their long-term prognosis.

Purpose: Ticagrelor is an antiplatelet drug, and its use increases the risk of bleeding. Coronary artery disease is significantly influenced by the widespread occurrence of diabetes mellitus. In order to decrease the incidence of clinical adverse events, a novel bleeding and thrombosis score is developed in this research.

Methods: We conducted a retrospective analysis of patient data from two medical centers who were diagnosed with diabetes mellitus and treated with ticagrelor. We gathered information on every patient from the electronic database of the hospital and follow-up. The collected data were statistically analyzed to obtain risk factors for bleeding and ischemic events.

Results: A total of 851 patients with diabetes mellitus who have been administered ticagrelor are included in our investigation. A total of 76 patients have bleeding events and 80 patients have ischemic events. The analysis of multiple variables indicates that characteristics like the age of >65, having a previous occurrence of bleeding, experiencing anemia, using aspirin, and taking atorvastatin are linked to a higher likelihood of bleeding. Additionally, the age of >65, smoking, having a history of blood clots, and having a BMI ≥ 30 are found to increase the risk of ischemia.

Conclusion: The A₄B score established in this study was better than the HAS-BLED score，and the same is true for the ABST score to the CHA₂DS-VASc score. This new risk assessment model can potentially detect patients who are at high risk for bleeding and ischemic events. For high-risk patients, the dose of ticagrelor can be adjusted appropriately or the medication can be adjusted.(2023-09-11, ChiCTR2300075627).

For patients with hemophilia A and high-titer inhibitors treated with bypassing agents there are no reliable methods to assess treatment effect. We investigated the utility of global hemostatic methods in assessing treatment with bypassing agents (rFVIIa or activated prothrombin complex [aPCC]). All patients with hemophilia A and inhibitors followed at the Coagulation Unit or the Pediatric Coagulation Unit at Karolinska University Hospital aged 6 years and above were eligible for this noninterventional study. Baseline plasma samples were spiked with bypassing agents in increasing concentrations (aPCC 50 U/kg, 100 U/kg, 150 U/kg, and rFVIIa 90 μg/kg and 270 μg/kg) in vitro. For patients treated with factor concentrates or bypassing agents follow-up samples were collected (in vivo tests). The samples were analyzed using overall hemostatic potential (OHP), and calibrated automated thrombogram, Calibrated Automated Thrombogram (CAT). Nine patients with hemophilia A with inhibitors were included. Spiking with rFVIIa normalized the coagulation potential in 6/8 samples, in 3 only with high dose. Only one sample did not improve adequately after spiking with aPCC. The improvement in hemostasis was reliably shown by both CAT and OHP. The baseline potential was, however, more often measurable by OHP compared to CAT. Factor concentrate had been administered to 5 patients normalizing the hemostatic potential in vivo in 2 (without spiking). The hemostatic improvement induced by spiking with rFVIIa or aPCC is shown by OHP and CAT, but the results have to be evaluated in larger cohorts.