effect of hydroxyethyl starch on COVID-19 patients-associated hypofibrinolytic state.

Background: Despite systematic thromboprophylaxis, 30% of the COVID-19 patients in intensive care units develop thrombosis. This occurrence is associated with a hypofibrinolytic state measured by thromboelastometry when adding tissue plasminogen activator (tPA) to citrated whole blood for a further run for EXTEM (ROTEM).

Objectives: Because hydroxyethyl starches (HESs) affect fibrin polymerization, we have assessed its potential effect on tPA-induced fibrinolysis.

Absence of Missense Variant Detection in Inherited Dysfibrinogenemia May Result from a Poor Raw Data Analysis Algorithm or Mosaicism.

Variant identification underlying inherited dysfibrinogenemia quite exceptionally fails. We report on two dysfibrinogenemia cases whose underlying DNA variant could not be identified by Sanger analysis. These failures result from two distinct mechanisms.

Mutational Epidemiology of Congenital Fibrinogen Disorders.

Background:  Numerous mutations in , or lead to congenital fibrinogen disorders (CFDs), but their epidemiology is not well characterized. The aim of this study was to evaluate the molecular epidemiology of CFD and to develop a genotyping strategy.

Methods:  Genetic data from 266 unrelated CFD patients genotyped at our laboratory and from a CFD open access database ( = 1,142) were evaluated.

Bisalbuminaemia due to novel mutation at a critical residue involved in recycling; Albumin Lyon (510His→Arg).

Objectives: To define the underlying cause of bisalbuminaemia in an individual presenting with spontaneous venous thrombosis.

Method: Plasma was examined by electrospray time-of-flight mass spectrometry (TOF MS) to assess albumin mutations and to quantify variant expression level. Tryptic peptide mapping and DNA sequencing were used to precisely define the mutation.

Early fibrinogen degradation coagulopathy: a predictive factor of parenchymal hematomas in cerebral rt-PA thrombolysis.

Background And Purpose: The purpose of this study was to systematically determine the correlations between the post-thrombolytic changes of hemostasis parameters and the occurrence of early intracerebral hemorrhage (ICH).

Methods: In 72 consecutive patients with cerebral infarcts treated with rt-PA, plasma levels of fibrinogen, plasminogen, alpha2-antiplasmin, factor XIII, fibrin(ogen) degradation products (FDPs) and d-Dimers were measured at baseline, 2 and 24h after thrombolysis. Correlations were studied between the hemostasis events and early (less than 24h) hemorrhagic infarcts (HIs) or parenchymatous hematomas (PH).

Post-thrombolysis haemostasis changes after rt-PA treatment in acute cerebral infarct. Correlations with cardioembolic aetiology and outcome.

Background: Little is known, in man, in the post-thrombolytic molecular dynamics of haemostasis, particularly the effect of rt-PA on antifibrinolytic components such as alpha2 anti-plasmin and Factor XIII.

Aims And Hypothesis: The purpose of this study was to systematically determine changes in coagulation and fibrinolytic parameters after thrombolysis with rt-PA during 24h. We also aimed to correlate these parameters with different acute ischemic stroke subtypes and global outcome.

Fibrinogen and von Willebrand factor and susceptibility vessel sign on T2*-weighted gradient echo imaging.

Background: We investigated the relationship between von Willebrand factor (vWF), fibrin monomers (FM), fibrinogen baseline levels and the presence of susceptibility vessel sign (SVS) on T2*-weighted gradient echo imaging in acute ischemic stroke.

Methods: SVS was assessed at admission using T2*-weighted GRE. Plasmatic levels of vWF, FM and fibrinogen were evaluated before the initiation of intravenous thrombolysis.

Plasminogen controls inflammation and pathogenesis of influenza virus infections via fibrinolysis.

Detrimental inflammation of the lungs is a hallmark of severe influenza virus infections. Endothelial cells are the source of cytokine amplification, although mechanisms underlying this process are unknown. Here, using combined pharmacological and gene-deletion approaches, we show that plasminogen controls lung inflammation and pathogenesis of infections with influenza A/PR/8/34, highly pathogenic H5N1 and 2009 pandemic H1N1 viruses.

The natural occurrence of human fibrinogen variants disrupting inter-chain disulfide bonds (A{alpha}Cys36Gly, A{alpha}Cys36Arg and A{alpha}Cys45Tyr) confirms the role of N-terminal A{alpha} disulfide bonds in protein assembly and secretion.

Analyses of site-directed fibrinogen mutants expressed in several recombinant models have previously shown that both inter- and intra-chain disulfide bonds are critical for fibrinogen assembly and secretion. Four naturally occurring mutations on AαCys36 and AαCys45 residues are reported here to be associated with decreased fibrinogen levels. This confirms the main role of the AαCys36-BβCys65 and AαCys45-γCys23 disulfide bonds in reaching a normal fibrinogen plasma level.

An unusual clinical presentation of factor XIII deficiency and issues relating to the monitoring of factor XIII replacement therapy.

Congenital factor XIII deficiency is a very rare bleeding disorder. Patients with severe FXIII deficiency usually exhibit severe bleeding diatheses. Factor XIII is also involved in maintaining pregnancy, and women with factor XIII deficiency have a high risk of spontaneous abortions.

A splicing donor site point mutation in intron 6 of the plasmin inhibitor (alpha2 antiplasmin) gene with heterozygous deficiency and a bleeding tendency.

A new case of familial plasmin inhibitor (alpha2 antiplasmin) deficiency is reported. The bleeding symptoms are moderate, happening after surgery or trauma or consisting of abnormal uterine bleeding induced by hormone replacement therapy. It is easily corrected with tranexamic acid.

Criteria for the Specific Measurement of Plasmin Inhibitor Activity Using an Enzymatic Procedure.

There is a lack of well-established criteria for the specific measurement of fibrinolytic parameters. On behalf of the SSC, the subcommittee on Fibrinolysis started a process to develop criteria for the specific measurement of fibrinolytic variables. This report describes the criteria for the specific measurement of plasmin inhibitor activity.