Phenotype and genotype analysis of patients with severe factor XI deficiency in Shaanxi Province, China
In this study, seven patients with severe FXI deficiency and several family members were analysed. The International Society on Thrombosis and Hemostasis-Bleeding Assessment Tool (ISTH-BAT) was applied to assess bleeding symptoms. FXI activity was determined using a one-stage method, and the FXI antigen was measured by enzyme-linked immunosorbent assay. Targeted capture next-generation sequencing and Sanger sequencing were applied to detect FXI gene mutations. The bleeding phenotype varied, although none of the participants had a history of spontaneous bleeding. One maternal received replacement therapy during the perinata...
Source: Blood Coagulation and Fibrinolysis - November 19, 2021 Category: Hematology Tags: ORIGINAL ARTICLES Source Type: research
How to use low-molecular-weight heparin to treat neonatal thrombosis in clinical practice
Among children, neonates have the highest incidence of thrombosis due to risk factors such as catheter instrumentation, an evolving coagulation system and congenital heart disease. Low-molecular-weight heparins (LMWHs) are the most commonly used anticoagulants in neonates. Published guidelines delineate dosing and monitoring protocols for LMWH therapy in newborns. However, challenging clinical situations frequently present that warrant healthcare providers to think critically beyond the range of guidelines, and judiciously resolve specific problems. This review describes the use of LMWH in the neonatal population, includin...
Source: Blood Coagulation and Fibrinolysis - November 19, 2021 Category: Hematology Tags: REVIEW ARTICLE Source Type: research
Optimizing factor VIII dosing in obese individuals with haemophilia A
No abstract available (Source: Blood Coagulation and Fibrinolysis)
Source: Blood Coagulation and Fibrinolysis - October 1, 2021 Category: Hematology Tags: LETTERS TO THE EDITOR Source Type: research
Optimal dosing of bemiparin for deep vein thrombosis treatment in children: a retrospective study
No abstract available (Source: Blood Coagulation and Fibrinolysis)
Source: Blood Coagulation and Fibrinolysis - October 1, 2021 Category: Hematology Tags: LETTERS TO THE EDITOR Source Type: research
P-selectin expression assay in a repeatedly serotonin-release assay-negative patient with heparin-induced thrombocytopenia
Heparin-induced thrombocytopenia (HIT) is an immune complication of heparin therapy caused by antibodies to complexes of platelet factor 4 (PF4) and heparin. Pathogenic antibodies to PF4/heparin bind and activate platelets to propagate a hypercoagulable state culminating in life-threatening thrombosis. The serotonin-release assay (SRA) is considered the gold-standard test to diagnose HIT. However, the sensitivity of the SRA was questioned with reported cases of clinical diagnosis of HIT and negative SRA. Herein, we present the utility of platelet factor 4-dependent P-selectin expression assay (PEA) in diagnosing HIT in a p...
Source: Blood Coagulation and Fibrinolysis - October 1, 2021 Category: Hematology Tags: CASE REPORTS Source Type: research
Eltrombopag treatment for severe immune thrombocytopenia during pregnancy: a case report
Primary immune thrombocytopenia (ITP) is an autoimmune disorder characterized by isolated thrombocytopenia (platelet count (Source: Blood Coagulation and Fibrinolysis)
Source: Blood Coagulation and Fibrinolysis - October 1, 2021 Category: Hematology Tags: CASE REPORTS Source Type: research
Hereditary factor V deficiency from heterozygous mutations with a novel variant p.Pro798Leufs∗13 in the F5 gene
To explore the causative mutation for autosomal recessive inheritance factor V (FV) deficiency in a Chinese family. Relative coagulation indexes and the FV antigen were tested by the one-stage clotting method and ELISA, respectively. At the same time, the calibrated automated thrombogram (CAT) was used to analyze the mutant protein function. All 25 exons, flanking sequences, 5′ and 3′ untranslated regions of the F5 were amplified by PCR and sequenced directly, while each suspected variant was verified by reverse sequencing. The possible impact of the mutant was analyzed by the corresponding bioinformatics software. The...
Source: Blood Coagulation and Fibrinolysis - October 1, 2021 Category: Hematology Tags: MUTATION REPORT Source Type: research
Mannose-binding lectin-associated serine protease-1 cleaves plasminogen and plasma fibronectin: prefers plasminogen over known fibrinogen substrate
This study reports that plasminogen and plasma fibronectin are two hitherto unknown substrates of MASP-1. Conversion of plasminogen to plasmin like molecule by MASP-1 was confirmed by cleavage of plasmin specific substrate and digestion of fibrin clot. The role of MASP-1 in clot dissolution was confirmed by turbidity assay. Our study shows that MASP-1 selects plasminogen over fibrinogen to be a preferable substrate. MASP-1 promotes the fibrinolytic activity by the generation of plasmin like molecule from plasminogen and further destabilizes the clot by digestion of plasma fibronectin. (Source: Blood Coagulation and Fibrinolysis)
Source: Blood Coagulation and Fibrinolysis - October 1, 2021 Category: Hematology Tags: ORIGINAL ARTICLES Source Type: research
Evaluation of the activated partial thromboplastin time and its influential factors in ischemic heart disease patients under heparin treatment
This study aimed to detect the defects of the current methods used to monitor unfractionated heparin (UFH) anticoagulant effect and find possible assistive parameters for activated partial thromboplastin time (aPTT) test to improve treatment performance. The required information was gathered from patients’ case records, treatment charts and laboratory reports. Kendall's tau correlation coefficient was calculated for analysing the relationship between variables. The partial least squares (PLS) and the stepwise multiple regression were operated, and the area under the receiver operating characteristic curve (AUC) and the r...
Source: Blood Coagulation and Fibrinolysis - October 1, 2021 Category: Hematology Tags: ORIGINAL ARTICLES Source Type: research
Whole blood viscosity in patients with aortic stenosis
Whole blood viscosity (WBV) may promote endothelial shear stress, inflammation, and can accelerate the atherosclerotic process. We aimed to evaluate the relationship between WBV and aortic stenosis.
The study included 209 participants of whom 49 patients had severe aortic stenosis, 98 patients had mild-to-moderate aortic stenosis and 62 patients served as control.
WBV values were significantly higher for high shear rate (HSR) (P = 0.001) and for low shear rate (LSR) (P = 0.002) in severe aortic stenosis group. HSR and LSR were correlated with mean systolic transaortic gradient (P (Source: Blood Coagulation and Fibrinolysis)
Source: Blood Coagulation and Fibrinolysis - October 1, 2021 Category: Hematology Tags: ORIGINAL ARTICLES Source Type: research
Levels of procoagulant microparticles expressing phosphatidylserine contribute to bleeding phenotype in patients with inherited thrombocytopenia
Inherited thrombocytopenia is a heterogeneous group of hereditary disorders with varying bleeding tendencies, not simply related to platelet count. Platelets transform into different subpopulations upon stimulation, including procoagulant platelets and platelet microparticles (PMPs), which are considered critical for haemostasis.
We aimed to investigate whether abnormalities in PMP and procoagulant platelet function were associated with the bleeding phenotype of inherited thrombocytopenia patients.
We enrolled 53 inherited thrombocytopenia patients. High-throughput sequencing of 36 inherited thrombocytopenia related ...
Source: Blood Coagulation and Fibrinolysis - October 1, 2021 Category: Hematology Tags: ORIGINAL ARTICLES Source Type: research
Very low-dose recombinant Factor VIIa administration for cardiac surgical bleeding reduces red blood cell transfusions and renal risk: a matched cohort study
Outcomes following administration of very-low-dose recombinant activated factor VIIa (vld-rFVIIa) for cardiac surgical bleeding remain debatable. We sought to determine the association of vld-rFVIIa and adverse surgical outcomes. Retrospective, cohort matching of patients undergoing cardiac surgery who received vld-rFVIIa (median 13.02 μg/kg) for perioperative bleeding were matched to cardiac surgical patients who had bleeding and received standard of care for bleeding without Factor VIIa administration. Of the 362 matched patients (182 in each group), patients who received rFVIIa required significantly less red blood c...
Source: Blood Coagulation and Fibrinolysis - October 1, 2021 Category: Hematology Tags: ORIGINAL ARTICLES Source Type: research
The utility and complications of plasma administration in cirrhotic patients undergoing minimally invasive procedures
Patients with cirrhosis have coagulopathy often necessitating correction with blood products, such as plasma products (fresh frozen plasma and plasma frozen within 24 h) prior to certain invasive procedures. However, plasma administration has the potential for substantial negative adverse effects such as volume overload, transfusion-related lung injury and allergic/anaphylactic reactions. In addition, its effectiveness in preventing bleeding is similarly unclear. The purpose of this study was to determine the safety and efficacy of plasma administration in cirrhotic patients undergoing minimally invasive procedures, spec...
Source: Blood Coagulation and Fibrinolysis - October 1, 2021 Category: Hematology Tags: ORIGINAL ARTICLES Source Type: research
A mild deficiency of ADAMTS13 is associated with severity in COVID-19: comparison of the coagulation profile in critically and noncritically ill patients
This study aimed to analyse the coagulation parameters of critically and noncritically ill patients with COVID-19 bilateral pneumonia, determine if coagulation factors consumption occurs and explore other potential mechanisms of COVID-19 coagulopathy. Critically and noncritically ill patients with a diagnosis of COVID-19 bilateral pneumonia were recruited. For each patient, we performed basic coagulation tests, quantification of coagulation factors and physiological inhibitor proteins, an evaluation of the fibrinolytic system and determination of von Willebrand Factor (vWF) and ADAMTS13. Laboratory data were compared with ...
Source: Blood Coagulation and Fibrinolysis - October 1, 2021 Category: Hematology Tags: ORIGINAL ARTICLES Source Type: research
Relationship between oxidant-antioxidant status and hypercoagulobility indices in children with iron deficiency anaemia
This study investigated the probable relationship between oxidative stress and hypercoagulability in children with IDA.
This study included 57 children diagnosed with IDA (IDA group) between October 2016 and October 2017 in addition to 48 healthy children (control group). The maximum clot firmness (MCF) index, and clot formation time (CFT) index, which are indicators of hypercoagulability in rotational thromboelastometry assays [intrinsic TEM (INTEM) and extrinsic TEM (EXTEM)] derived from our previous study, were recorded. Total oxidant status (TOS), total antioxidant capacity (TAC) and oxidative stress index (OSI) wer...
Source: Blood Coagulation and Fibrinolysis - October 1, 2021 Category: Hematology Tags: ORIGINAL ARTICLES Source Type: research
