Fibrinogen dysfunction and fibrinolysis state in patients with hepatitis B-related cirrhosis.

Objective: To assess the fibrinogen function in patients with hepatitis B-related cirrhosis and explore the relationship between dysfibrinogenemia and bleeding and thrombotic events.

Methods: Medical records and laboratory data of the patients with hepatitis B-related cirrhosis were collected. Patients were categorized into three groups based on the Child-Pugh score.

Heterozygous Prothrombin Mutation-Associated Thrombophilia.

Background:  Venous thromboembolism (VTE) is predisposed by thrombotic mutations in patients with hereditary thrombophilia. Although prothrombin deficiencies caused by homozygous or compound heterozygous mutations are associated with bleeding diathesis, rare cases have shown a correlation between heterozygous prothrombin mutations and thrombosis.

Materials And Methods:  We surveyed genetic variants involved in thrombosis and hemostasis in 347 patients with unprovoked VTE or having a positive family history of thrombosis.

Structural and functional characterization of novel F7 mutations identified in Chinese factor VII-deficient patients.

Hereditary factor VII (FVII) deficiency is a rare recessive bleeding disorder with an estimated prevalence of 1/500 000. We had investigated 50 unrelated Chinese patients with FVII deficiency and identified, in total, 25 mutations, including 18 missense mutations and 5 splicing mutations, on the F7 gene. The nucleotide transition c.

Inactivated SARS-CoV-2 vaccine does not influence the profile of prothrombotic antibody nor increase the risk of thrombosis in a prospective Chinese cohort.

The presence of antiphospholipid antibodies was shown to be associated with thrombosis in coronavirus disease 2019 (COVID-19) patients. Recently, according to reports from several studies, the vaccine-induced immune thrombotic thrombocytopenia is mediated by anti-platelet factor 4 (PF4)-polyanion complex in adenovirus-vectored COVID-19 vaccine recipients. It is impendent to explore whether inactivated COVID-19 vaccine widely used in China influences prothrombotic autoantibody production and induces thrombosis.

Low factor V level ameliorates bleeding diathesis in patients with combined deficiency of factor V and factor VIII.

Combined factor V (FV) and FVIII deficiency (F5F8D) is a rare autosomal-recessive bleeding disorder caused by mutations in lectin mannose binding-1 (LMAN1) and multiple coagulation factor deficiency-2 (MCFD2). Six causative homozygous mutations (5 in LMAN1 and 1 in MCFD2) were identified in 6 patients with F5F8D. A thrombin-generation assay, triggered with tissue factor (1 pM) in F5F8D plasma, paradoxically exhibited enhanced thrombin generation compared with normal plasma.

Inhibitor eradication and bleeding management of acquired hemophilia A: a single center experience in China.

Acquired hemophilia A (AHA) is a rare disease resulting from autoantibodies against coagulation factor VIII that leads to spontaneous bleeding. This study reports the clinical characteristics and treatment outcomes of a relatively sizable cohort of patients with AHA. We retrospectively analyzed the characteristics and outcomes of 42 patients with AHA diagnosed in our center from January 2014 through December 2018.

[Identification of genetic defects in a Chinese pedigree with factor XIII deficiency: case report and literature review].

Objective: To perform phenotypic diagnosis, genetic diagnosis and prenatal diagnosis of inherited coagulation factor XIII (FXIII)deficiency in a Chinese family also provide a review of inherited coagulation F XIII deficiency.

Methods: The activity levels of F XIII (F XIII:C) of proband and family members were measured by clot solubility test and REA-chrom F XIII kit. The antigen levels of F XIII(FXIII:Ag) were measured by enzyme-linked immunosorbent assay.

Regional variations in the prevalence and misdiagnosis of air flow obstruction in China: baseline results from a prospective cohort of the China Kadoorie Biobank (CKB).

Background: Despite the great burden of chronic respiratory diseases in China, few large multicentre, spirometry-based studies have examined its prevalence, rate of underdiagnosis regionally or the relevance of socioeconomic and lifestyle factors.

Methods: We analysed data from 512 891 adults in the China Kadoorie Biobank, recruited from 10 diverse regions of China during 2004-2008. Air flow obstruction (AFO) was defined by the lower limit of normal criteria based on spirometry-measured lung function.

Effectiveness of a television advertisement campaign on giving cigarettes in a chinese population.

Background: Anti-tobacco television advertisement campaigns may convey messages on smoking-related health consequences and create norms against giving cigarettes.

Methods: Altogether, 156 and 112 slots of a television advertisement "Giving cigarettes is giving harm" were aired on Suzhou and Yizheng, respectively, over one month in 2010. Participants were recruited from 15 locations in Suzhou and 8 locations in Yizheng using a street intercept method.

Prevalence and correlates of airflow obstruction in ∼317,000 never-smokers in China.

In China, the burden of chronic obstructive disease (COPD) is high in never-smokers but little is known about its causes in this group. We analysed data on 287 000 female and 30 000 male never-smokers aged 30-79 years from 10 regions in China, who participated in the China Kadoorie Biobank baseline survey (2004-2008). Prevalence of airflow obstruction (AFO) (pre-bronchodilator forced expiratory volume in 1 s (FEV1)/forced vital capacity (FVC) <0.

Molecular basis and thrombotic manifestations of antithrombin deficiency in 15 unrelated Chinese patients.

Introduction: Antithrombin (AT) deficiency is associated with an increasing risk of thrombosis.

Materials And Methods: 15 unrelated patients with AT deficiency defined by thrombophilic assays were recruited and detailed clinical information about patients, focusing on the personal and family history of thromboembolism (TE), were recorded. Mutation analysis was performed by direct sequencing of an AT gene (SERPINC1) in the patients and their family members.

[Functional study of abnormal fibrinogen caused by Arg275His mutation in fibrinogen γ chain].

Objective: To investigate the function of abnormal fibrinogen in two inherited dysfibrinogenemia pedigrees.

Methods: Routine coagulation tests were conducted in the probands and related family members. The antigen and activity levels of fibrinogen were detected by immunoturbidimetry assay and clauss assay, respectively.

[The phenotypic and genotypic diagnosis of three Chinese patients with von Willebrand disease].

Objective: To analyze the phenotype and genotype of three patients with von Willebrand disease (vWD), and to explore its molecular pathogenesis.

Methods: Bleeding time (BT), APTT, ristocetin induced platelet aggregation (RIPA), von Willebrand factor (vWF):ristocetin cofactor (Rco) (vWF:Rco), vWF antigen (vWF:Ag), vWF activity (vWF:A) test, vWF collagen binding assay (vWF:CB) and multimer analysis were detected for phenotype diagnosis. The dynamic process of blood coagulation was evaluated by using the thrombelastography.

[Phenotype and genotype analysis of two Chinese pedigrees with type 3 von Willebrand diseases].

Objective: To analyze the phenotype and genotype of two Chinese pedigrees with von Willebrand diseases, and to investigate the molecular pathogenesis.

Methods: Bleeding time (BT), activated partial thromboplastin time (APTT), ristocetin-induced platelet aggregation (RIPA), von Willebrand factor-ristocetin cofactor (vWF:Rco), von Willebrand factor antigen (vWF:Ag), von Willebrand factor activity (vWF:A), von Willebrand factor collagen binding assay (vWF:CB) and multimer analysis were used for phenotype diagnosis. DNA was extracted.

[Genotype and function analyses of four inherited dysfibrinogenemia pedigree caused by Arg16 amino acid substitution in fibrinogen Aα chain].

Objective: To analyze the phenotype, genotype and function in four Chinese pedigrees with inherited dysfibrinogenemia.

Methods: Routing tests including activated partial thromboplastin time (APTT), prothrombin time (PT), thrombin time (TT), reptilase time (RT), the activities of antithrombin (AT), protein C (PC) and protein S (PS) were detected in four pedigrees. The activity and antigen of plasma fibrinogen were analyzed by Clauss and immunoturbidimetry methods, respectively.

[Molecular mechanisms of recurrent venous thrombosis in two pedigrees with type I antithrombin deficiency].

Objective: To investigate the clinical phenotype, genotype and molecular mechanism of recurrent venous thrombosis in two Chinese pedigrees with type I antithrombin (AT) deficiency.

Methods: The routine coagulation screening tests were detected, thrombin generation tests was performed to evaluate the hypercoagulation. Anticardiolipin antibody (ACA) and lupus anticoagulant (LA) were detected with enzyme-linked immunosorbent assay (ELISA) and diluted viper venom time assay (DVVT), respectively.

[Two novel mutations in one pedigree with hereditary Factor VII deficiency].

Objective: To explore the mutations of coagulation factor VII (FVII) gene in one pedigree with hereditary FVII deficiency, and to investigate the molecular mechanisms of FVII deficiency.

Methods: FVII gene mutations were analysed in the pedigree by direct DNA sequencing. The mutated DNA fragments were cloned into pMD19-T simple TA vector, and sequenced to confirm their distribution on chromosome.

[Analysis of phenotype and genotype in three Chinese pedigrees with inherited dysfibrinogenemia].

Objective: To analyze the phenotype and genotype in three Chinese pedigrees with inherited dysfibrinogenemia.

Methods: Laboratory tests including activated partial thromboplastin time (APTT), prothrombin time (PT), thrombin time (TT), reptilase time (RT), and the activities of antithrombin (AT:C), protein C (PC:C) and protein S(PS:C) were detected in three pedigrees. The activity and antigen of plasma fibrinogen (Fg) were analyzed by Clauss and immunoturbidimetry methods, respectively.

[Phenotype and genotype analysis of three Chinese pedigrees with von Willebrand disease].

Objective: To analyze phenotype and genotype of three Chinese pedigrees with von Willebrand disease (vWD), and explore the molecular mechanism.

Methods: Bleeding time (BT), activated partial thromboplastin time (APTT), ristocetin-induced platelet aggregation (RIPA), von Willebrand factor (vWF): ristocetin cofactor (RCof) (vWF:RCof), vWF antigen (vWF:Ag), vWF activity (vWF:A) test, vWF collagen binding assay (vWF:CB), vWF and Factor VIII (FVIII) binding assay (vWF:FVIII:B) and multimer analysis were used for phenotype diagnosis. Genomic DNA was extracted from the peripheral blood (PB).

[Analysis of phenotype and genotype in four Chinese pedigrees with inherited coagulation factor V deficiency.].

Objective: To identify the phenotype and genotype in four Chinese pedigrees with inherited coagulation factor V (FV) deficiency.

Methods: The tests of activated partial thromboplastin time (APTT), prothrombin time (PT), FV activity (FV:C) and FV antigen (FV:Ag) were used for phenotype diagnosis. All the exons and exon-intron boundaries of F5 gene were amplified by PCR and analyzed by direct sequencing.

[Two new mutations of AT gene in type I inherited antithrombin deficiency.].

Objective: To identify the clinical phenotype and gene mutation in two kindreds with type I inherited antithrombin (AT) deficiency.

Methods: The coagulation and anticoagulation testing and thrombophilia screening were used for phenotypic diagnosis and immunonephelometry and chromogenic assay for plasma level of AT antigen (AT:Ag) and AT activity (AT:A), respectively. All of the seven exons and intron-exon boundaries and untranslation regions of AT gene were amplified by PCR, and the PCR products analysis was by direct sequencing.

[The application of thrombin generation tests to warfarin anticoagulation monitoring].

Objectives: To explore the thrombin generation capacity in patients on warfarin therapy with different prothrombin time international normalized ratio (PT-INR), the capacity in relation to bleeding, and the application of thrombin generation tests to warfarin therapy monitoring.

Methods: Seventy eight blood samples were taken from patients on warfarin therapy for more than 3 months owing to valve replacement or atrial fibrillation. The patients' case history and PT-INR were collected and thrombin generation tests were performed in all samples.

[Studies on inherited coagulation factor VII deficiency and tissue factor abnormality in a pedigree].

Objective: To investigate the mechanism of clinical haemorrhage in an inherited coagulation factor VII (FVII) deficiency and tissue factor abnormality pedigree.

Methods: All exons, exon-intron boundaries and the 3', 5' untranslated sequences of FVII and tissue factor (TF) genes were amplified by PCR and sequenced directly. Any mutation identified by direct sequencing was confirmed by reverse sequencing.