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1.
Journal of Biomedical Engineering ; (6): 1087-1096, 2021.
Article in Chinese | WPRIM | ID: wpr-921849

ABSTRACT

Fibrinogen (Fg) in human plasma plays an important role in hemostasis, vascular repair and tissue integrity. The surface chemistry of extracellular matrix or biological materials affects the orientation and distribution of Fg, and changes the exposure of integrin binding sites, thereby affecting its adhesion function to platelets. Here, the quantity, morphology and side chain exposure of Fg adsorbed on hydrophilic, hydrophobic and avidin surfaces were measured by atomic force microscopy (AFM) and flow cytometry (FCM), then the rolling behavior of platelets on Fg was observed through a parallel plate flow chamber system. Our results show that the hydrophobic surface leads to a large amount of cross-linking and aggregation of Fg, while the hydrophilic surface reduces the adsorption and accumulation of Fg while causing the exposure and spreading of the α chain on Fg and further mediating the adhesion of platelets. Fg immobilized by avidin / biotin on hydrophilic surface can maintain the monomer state, avoid over exposure and stretching of α chain, and bind to the platelets activated by the A1 domain of von Willebrand factor instead of inactivated platelets. This study would be helpful for improving the blood compatibility of implant biomaterials and reasonable experimental design of coagulation


Subject(s)
Adsorption , Blood Platelets , Fibrinogen , Humans , Platelet Adhesiveness , von Willebrand Factor
2.
Article in Chinese | WPRIM | ID: wpr-880165

ABSTRACT

OBJECTIVE@#To investigate the biological function of Cysteine rich (CysR) domain of a disintegrin and metalloprotease with thrombospondin type 1 repeats-13 (ADAMTS13) on cleavage of von Willebrand factor (vWF) and provide experimental evidence for exploring the pathogenesis of thrombotic thrombocytopenic purpura (TTP).@*METHODS@#The six amino acids (EDGTLS) in ADAMTS13 CysR domain were point mutated one by one, and the mutant ADAMTS13 proteins were expressed and purified. The cleavage products of vWF polymer by wild-type or mutant ADAMTS13 under denaturing condition or shear stress were separated by 1% SeaKem HGT agarose gel and detected by Western blot.@*RESULTS@#The mutant ADAMTS13 plasmids (M1: Glu515Ala; M2: Asp516Ala; M3: Gly517Ala; M4: Thr518Ala; M5: Leu519Ala; M6: Ser520Ala) were successfully constructed and the proteins of wild-type and mutant ADAMTS13 were purified. Wild-type ADAMTS13 almost completely cleaved the vWF polymer under denaturing condition, while the cleavage activity of M1 mutant was significantly reduced in the same condition (P<0.01). The cleavage activity of M1 mutant of ADAMTS13 was also significantly reduced compared with that of the wild-type under shear stress (P<0.01). The activity of M1 mutant to cleave the FRETS-vWF73 was dramatically reduced compared with that of wild-type ADAMTS13. However, the binding ability of M1 mutant to vWF was similar with that of wild-type ADAMTS13.@*CONCLUSION@#The CysR domain of ADAMTS13 plays an important role in the digestion of vWF under denaturing condition and shear stress. The Glu515 amino acid residue might be an important site for substrate recognition.


Subject(s)
ADAM Proteins , ADAMTS13 Protein/genetics , Humans , Purpura, Thrombotic Thrombocytopenic/genetics , von Willebrand Factor/genetics
5.
Braz. J. Pharm. Sci. (Online) ; 56: e18430, 2020. tab, graf
Article in English | LILACS | ID: biblio-1132056

ABSTRACT

To assess the effect of nesiritide on the endothelial function of iliac arteries following endothelia trauma. Right iliac artery trauma was created with a balloon catheter. Ten rabbits were treated with a 4-week subcutaneous injection of nesiritide at a fixed daily dose of 0.1mg/kg. Ten rabbits received daily normal saline injection. Plasma endothelin 1 (ET-1), nitric oxide (NO), and Von Willebrand Factor (vWF) were measured before and after the therapies. Tissue proliferating cell nuclear antigen (PCNA) was measured after the treatment. After the treatment, in the therapeutic group, the area under internal elastic membrane and the residual lumen area were higher than in the normal saline group (P <0.05). The plasma levels of ET-1 (91.6±6.8 vs 114.9±6.3 ng/L, P =0.001), vWF (134.6±10.8% vs 188.8±10.4%, P =0.001) and the ratio of PCNA positive expression (11.7±4.2% vs 36.2±11.4%, P =0.005) in the therapeutic group was lower than in the normal saline group, while the plasma levels of NO was higher (89.7±9.3 vs 43.5±5.3 µmol/L, P =0.001). Nesiritide inhibited remodeling of rabbit iliac artery following endothelial trauma. The inhibition of vascular remodeling may be related to the alleviated endothelial dysfunction and reduced expression of tissue proliferating cell nuclear antigen


Subject(s)
Animals , Male , Rabbits , Iliac Aneurysm/classification , Endothelin-1/adverse effects , Natriuretic Peptide, Brain/analysis , Endothelial Cells/drug effects , Wounds and Injuries/classification , von Willebrand Factor/analysis , Catheters/classification , Iliac Artery , Nitric Oxide/analysis
6.
Rev. cuba. obstet. ginecol ; 45(4): e405, oct.-dic. 2019.
Article in Spanish | LILACS, CUMED | ID: biblio-1126714

ABSTRACT

RESUMEN La hemorragia uterina anormal es un término empleado para las alteraciones en la regularidad, duración y/o volumen de sangrado menstrual y es considerada una causa común de consulta médica y en ocasiones supone un reto diagnóstico para el médico tratante. Dentro del abordaje de la etiología de dicha patología, las coagulopatías afectan alrededor del 13 por ciento de las mujeres, y la más común es la enfermedad de von Willebrand. El objetivo de este trabajo fue realizar una revisión de la literatura científica actual sobre el papel que cumple la enfermedad de von Willebrand en la hemorragia uterina anormal. Esta es una patología hereditaria derivada de una deficiencia del factor von Willebrand encargado de la adhesión plaquetaria. La prevalencia de esta enfermedad puede ser baja, sin embargo, cuando se estudia la población de mujeres con menorragia, la frecuencia puede ir de 5 a 20 por ciento. Se han descrito diferentes problemas ginecológicos asociados a la enfermedad de von Willebrand, tales como menorragia, dismenorrea y una importante deficiencia de hierro asociada a esta, además de una mayor incidencia de quistes ováricos, endometriosis, hiperplasia endometrial y pólipos endometriales. La literatura actual sugiere que se realice tamizaje a aquellas mujeres con cuadro clínico sugestivo. Con respecto al tratamiento la literatura reporta el uso de ácido tranexámico y anticonceptivos orales, pero el que mayor utilidad ha demostrado es la desmopresina(AU)


ABSTRACT Abnormal uterine bleeding is a term used for alterations in the regularity, duration and / or volume of menstrual bleeding and it is considered a common cause of medical consultation; sometimes it is a diagnostic challenge for the treating physician. Within the aetiology approach of said pathology, coagulopathies affect around 13 percent of women, and the most common is von Willebrand disease. The objective is to review the current scientific literature on the influence of von Willebrand disease in abnormal uterine bleeding. This is an inherited pathology derived from a deficiency of the von Willebrand factor responsible for platelet adhesion. The prevalence of this disease may be low, however, when studying the population of women with menorrhagia, the frequency can range from 5 to 20 percent. Different gynecological problems associated with von Willebrand disease have been described, such as menorrhagia, dysmenorrhea and a significant iron deficiency associated with it, in addition to a higher incidence of ovarian cysts, endometriosis, endometrial hyperplasia and endometrial polyps. The current literature suggests that those women with suggestive clinical symptoms should be screened. Regarding treatment, the literature reports the use of tranexamic acid and oral contraceptives, nonetheless desmopressin has proven to be most useful(AU)


Subject(s)
Humans , Female , Uterine Hemorrhage/diagnosis , von Willebrand Diseases/pathology , Blood Coagulation Disorders/epidemiology , von Willebrand Factor , Ovarian Cysts/epidemiology , Review Literature as Topic
7.
Univ. salud ; 21(3): 277-287, Sep.-Dic. 2019. tab
Article in Spanish | LILACS, COLNAL | ID: biblio-1043549

ABSTRACT

Resumen Introducción: El tipo de grupo sanguíneo entre otros factores, influye en los niveles plasmáticos del Factor de von Willebrand (FvW), su actividad biológica podría incidir en el desarrollo de eventos trombóticos y hemorrágicos. Objetivo: Describir las características y los mecanismos de reacciones postrasduccionales del grupo sanguíneo que permiten la variación en la concentración plasmática del FvW. Materiales y métodos: Revisión teórico descriptiva de tipo documental. Las bases de datos consultadas fueron Medline, Lilacs, ScienceDirect, Scopus, SciELO, Proquest, Ovid y Pubmed. Como criterio de selección se incluyeron artículos en idioma inglés y español a partir del año 2010 y algunos anteriores como referente histórico. Resultados: Se describieron los principales mecanismos e investigaciones que evidencian la influencia del tipo de grupo sanguíneo ABO en los niveles plasmáticos del FvW, así como la estructura y función de dicha proteína. Conclusiones: Las concentraciones plasmáticas del FvW pueden depender del tipo de grupo sanguíneo, la edad, sexo, embarazo, ciclo menstrual, variación de proteínas y factores bioquímicos e inmunológicos. Se podría tener en cuenta el tipo de grupo sanguíneo de los pacientes como un posible factor predictor a futuro de complicaciones clínicas tanto trombóticas como hemorrágicas.


Abstract Introduction: The type of blood group among other factors influences the plasma levels of von Willebrand Factor (FvW) and its biological activity could influence the development of thrombotic and hemorrhagic events. Objective: To describe the characteristics and mechanisms of post-translational reactions of the blood group that generate variation in the plasma concentration of FvW. Materials and methods: A descriptive theoretical review of documentary type. The databases consulted were Medline, Lilacs, ScienceDirect, Scopus, SciELO, Proquest, Ovid and Pubmed. As a selection criterion, articles in English and Spanish were included beginning in 2010 and some previous ones as historical reference. Results: The main mechanisms and investigations that show the influence of the ABO blood group type on the plasma levels of FvW, as well as the structure and function of this protein were described. Conclusions: FvW plasma concentrations may depend on the type of blood group, age, sex, pregnancy, menstrual cycle, protein variation and biochemical and immunological factors. The type of blood group of patients could be considered as a possible future predictor of both thrombotic and hemorrhagic clinical complications.


Subject(s)
Humans , Blood Group Antigens , von Willebrand Factor , Thrombophilia , ADAMTS13 Protein
8.
Journal of Experimental Hematology ; (6): 1596-1601, 2019.
Article in Chinese | WPRIM | ID: wpr-775679

ABSTRACT

OBJECTIVE@#To obtain the recombinant protein of spacer domain in von Willebrand factor cleaving protease (ADAMTS13), and further study its biological function in ADAMTS13.@*METHODS@#The prokaryotic expression vector was constructed by using the template of plasmid with full-length ADAMTS13, and then transfected into E coli., following the induction of IPTG with the low temperature (30 ℃). The recombinant protein was purified with Ni-NTA agarose column by gradient imidazole. The purity and immune activity of purified products were identified with SDS-PAGE and Western blot respectively. By Adding the recombinant protein to the plasma of immune-mediated thrombotic thrombocytopenic purpura (iTTP) patients, the activity of ADAMTS13 was tested.@*RESULTS@#The prokaryotic expression vector was successfully constructed and the protein of spacer domain with the high purity was obtained. Western blot showed that the recombinant fragment could both react with monoclonal antibody against 6×His and polyclonal sheep IgG against ADAMTS13 (Gln34-Trp688). The protein formed a main lane at the position of 15 kDa with SDS-PAGE. It was demonstrated that the recombinant protein could efficiently elevate the ADAMTS13 activity in plasma of iTTP patients to reach normal level by functional experiment.@*CONCLUSION@#The recombinant protein has high purity and immune activity, which provides the experimental basis for further research on mechanism of iTTP involved in spacer domain.


Subject(s)
ADAM Proteins , ADAMTS13 Protein , Animals , Escherichia coli , Humans , Purpura, Thrombotic Thrombocytopenic , Recombinant Proteins , Sheep , von Willebrand Factor
9.
Article in Chinese | WPRIM | ID: wpr-774334

ABSTRACT

OBJECTIVE@#To establish a novel flow cytometric immunobead array (FCIA) for detecting plasma von Willebrand factor activity (vWF:GPIbR) and apply it in ischemic stroke (IS).@*METHODS@#Microspheres coated with anti-human platelet glycoprotein Ibα (GPIbα) monoclonal antibody SZ151 IgG, were incubated with recombinant fragment of GPIbα, then added ristocetin and plasma, finally incubated with FITC-conjugated sheep-anti-human vWF IgG polyclonal antibody, and detected by flow cytometry. vWF antigen (vWF:Ag), vWF:GPIbR, and vWF collagen binding assay (vWF:CB) were also included for evaluating vWF levels in IS patients.@*RESULTS@#The intra-assay coefficient variations (CVs) and inter-assay CVs of FCIA were 7.7% and 13.5%, respectively. The slope of the linear regression was 0.9739 (r=0.855, P<0.001), and the Bland-Altman bias was 9.95%, indicating a good correlation between FCIA and ELISA. The FCIA had better sensitivity, specificity and accuracy as compared with those by ELISA (P<0.05). The levels of vWF:Ag, vWF:GPIbR and vWF:CB in IS patients were significantly higher in comparison with those in healthy controls (H=7.8, 6.4, 6.2, respectively, P<0.01), the level of vWF:GPIbR in IS patients positively correlated with levels of vWF:Ag, high-sensitivity C-reactive protein, Autar score and hospitalization time.@*CONCLUSION@#The FCIA for detecting plasma vWF:GPIbR is more specific and accurate than ELISA. The vWF:GPIbR is involved in the paroxysm of IS, which could be used to evaluate the risk of thrombosis in IS patients.


Subject(s)
Animals , Brain Ischemia , Flow Cytometry , Humans , Prognosis , Sheep , Stroke , von Willebrand Diseases , von Willebrand Factor
10.
Article in English | WPRIM | ID: wpr-762440

ABSTRACT

BACKGROUND: von Willebrand disease (VWD), characterized by quantitative or qualitative defects of von Willebrand factor (VWF), is the most common inheritable bleeding disorder. Data regarding the genetic background of VWD in Korean patients is limited. To our knowledge, this is the first comprehensive molecular genetic investigation of Korean patients with VWD. METHODS: Twenty-two unrelated patients with VWD were recruited from August 2014 to December 2017 (age range 28 months–64 years; male:female ratio 1.2:1). Fifteen patients had type 1, six had type 2, and one had type 3 VWD. Blood samples were collected for coagulation analyses and molecular genetic analyses from each patient. Direct sequencing of all exons, flanking intronic sequences, and the promoter of VWF was performed. In patients without sequence variants, multiplex ligation-dependent probe amplification (MLPA) was performed to detect dosage variants. We adapted the American College of Medical Genetics and Genomics guidelines for variant interpretation and considered variants of uncertain significance, likely pathogenic variants, and pathogenic variants as putative disease-causing variants. RESULTS: VWF variants were identified in 15 patients (68%): 14 patients with a single heterozygous variant and one patient with two heterozygous variants. The variants consisted of 13 missense variants, one small insertion, and one splicing variant. Four variants were novel: p.S764Efs*16, p.C889R, p.C1130Y, and p.W2193C. MLPA analysis in seven patients without reportable variants revealed no dosage variants. CONCLUSIONS: This study revealed the spectrum of VWF variants, including novel ones, and limited diagnostic utility of MLPA analyses in Korean patients with VWD.


Subject(s)
Exons , Genetic Background , Genetics, Medical , Genomics , Hemorrhage , Humans , Introns , Korea , Molecular Biology , Multiplex Polymerase Chain Reaction , von Willebrand Disease, Type 3 , von Willebrand Diseases , von Willebrand Factor
11.
Article in Chinese | WPRIM | ID: wpr-776531

ABSTRACT

OBJECTIVE@#To investigate the vascular damage effects and possible mechanism of acute exposure to ozone (O) in male Wistar rats.@*METHODS@#One hundred and twenty male Wistar rats were randomly divided into six groups, 20 in each group. The experimental animals were placed in a gas poisoning cabinet, the control group was exposed to filtered air, and the treatment group was exposed to ozone at concentrations of 0.12 ppm, 0.5 ppm, 1.0 ppm, 2.0 ppm, and 4.0 ppm, respectively, for 4 hours. Arterial blood pressure data were obtained by PC-lab medical physiological signal acquisition system. Blood rheology indicators and blood biochemical indicators were detected by Tianjin Dean Diagnostic Laboratory. Serum endothelin-1 (ET-1), homocysteine (HCY), von Willebrand factor (vWF), 8-hydroxydeoxyguanosine (8-OhdG), interleukin (IL-6) and tumor necrosis factor alpha (TNF-α) were detected by enzyme-linked immunosorbent assay (ELISA) microplate assay. Oxidative stress indicators superoxide dismutase (SOD) activity and malondialdehyde (MDA) were determined by xanthine oxidase method, thiobarbituric acid (TBA) method, reduced glutathione (GSH) and nitric oxide (NO) were tested by using microplate colorimetry. Paraffin sections were prepared from thoracic aorta tissue, and vascular structure was observed by HE staining.@*RESULTS@#Acute exposure to 0.12 ppm ozone could cause a significant increase in arterial systolic blood pressure (SBP). Exposure to different concentrations of ozone could cause a significant increase in plasma viscosity, and the K value of the ESR equation was significantly increased in the 1.0 ppm ozone exposure group. Both the relative and reduced viscosities were significantly reduced at ozone concentrations of 0.5 ppm and 4.0 ppm, while the red blood cell deformation index was increased significantly at ozone concentrations of 0.12 ppm, 0.5 ppm, 1.0 ppm, and 2.0 ppm. Acute ozone exposure resulted in the decrease of total cholesterol content. The content of high-density lipoprotein cholesterol (HDL-C) was significantly reduced in the 0.12 ppm ozone exposure group. When the ozone concentration was higher than 1.0 ppm, the body may also had an inflammatory reaction (increased TNF-α) and oxidative stress (increased MDA, decreased GSH). Acute exposure to ozone could lead to elevated levels of ET-1 in the blood, with significant differences in the 4.0 ppm concentration group, while HCY levels were decreased firstly and then increased, reaching the highest in the 1.0 ppm concentration group. No obvious pathological changes were observed in the thoracic aorta.@*CONCLUSION@#Acute ozone exposure can affect arterial blood pressure, blood rheology and cholesterol metabolism in rats. The possible mechanism is that ozone exposure leads to inflammatory reaction and oxidative stress reaction, causing vascular endothelial function damage, and vascular endothelial cells increase with ozone exposure concentration.


Subject(s)
Animals , Blood Vessels , Wounds and Injuries , Deoxyguanosine , Blood , Endothelin-1 , Blood , Homocysteine , Blood , Interleukin-6 , Blood , Male , Malondialdehyde , Oxidative Stress , Ozone , Toxicity , Rats , Rats, Wistar , Superoxide Dismutase , Tumor Necrosis Factor-alpha , Blood , von Willebrand Factor
12.
Article in English | WPRIM | ID: wpr-739668

ABSTRACT

BACKGROUND: Venous thromboembolism is a common complication in patients with glioma. The clotting factor von Willebrand factor (VWF) is a highly adhesive procoagulant molecule that mediates platelet adhesion to endothelial and subendothelial surfaces. In the current analysis, we examined The Cancer Genome Atlas (TCGA) data to assess the VWF gene in patients with lower grade gliomas. METHODS: For newly diagnosed gliomas, we evaluated the association between VWF and overall survival in the Genomic Data Commons TCGA Lower Grade Glioma (LGG) dataset in TCGA. Simple statistics were calculated to identify patterns of mutual exclusivity or co-occurrence of VWF mutations. For each pair of query genes an odds ratio was calculated that indicates the likelihood that the mutations in the two genes are mutually exclusive or co-occurrent across the selected cases. To determine whether the identified relationship was significant for a gene pair, Fisher's exact test was performed. RESULTS: Lower grade gliomas with less VWF gene expression had significantly better survival than those with more VWF gene expression (hazard ratio 0.64, 95% confidence interval 0.44 to 0.92, p=0.015 log rank test). When we analyzed the data with Cox regression, VWF expression had a significant effect on survival (p=0.02) that was unrelated to the effect of IDH1 expression (p=0.062), TP53 expression (p=0.135), independent of ATRX expression (p=0.021) and histology (astrocytoma versus oligoastrocytoma and oligodendroglioma, p=0.002). VWF mutations significantly co-occur with mutations in TP53 and ATRX (p<0.001). CONCLUSION: The deleterious prognostic effect of VWF expression and its co-occurrent mutations with TP53 and ATRX in lower grade gliomas are not surprising, given VWF's role in other cancers. Therefore, VWF gene expression may be a clinically important risk marker in lower grade glioma.


Subject(s)
Adhesives , Blood Platelets , Dataset , Gene Expression , Genes, vif , Genome , Glioblastoma , Glioma , Humans , Odds Ratio , Oligodendroglioma , Venous Thromboembolism , von Willebrand Factor
13.
Acta cir. bras ; 33(8): 664-672, Aug. 2018. tab, graf
Article in English | LILACS | ID: biblio-949374

ABSTRACT

Abstract Purpose: To investigate the correlation of inhaled nitric oxide (NO) on plasma levels of cardiac troponin I (cTnI) and von Willebrand factor (vWF), glycoprotein (GP) IIb/IIIa, granule membrane protein 140 (GMP-140) in rabbits with acute massive pulmonary embolism (PE). Methods: Thirty apanese white rabbits were divided into 3 groups, thrombus were injected in model group (n = 10), NO were inhalated for 24 h after massive PE in NO group (n = 10), saline were injected in control group (n = 10). The concentrations of vWF, GP IIb/IIIa, GMP-140 and cTnI were tested at 4, 8, 12, 16, 20, and 24 h, Correlation analyses were conducted between cTnI and vWF, GP IIb/IIIa, and GMP-140 by Pearson's correlation. Results: The concentration of cTnI and vWF, GP IIb/IIIa, and GMP-140 was increased in the model group, compared to control group. In the inhaled group, the concentrations of cTnI, vWF, GP IIb/IIIa, and GMP-140 were reduced compared to model group. There was a positive correlation between cTnI and vWF, GP IIb/IIIa, and GMP-140. Conclusion: Inhaled nitric oxide can lead to a decrease in levels of cardiac troponin I, von Willebrand factor, glycoprotein, and granule membrane protein 140, after an established myocardial damage, provoked by acute massive pulmonary embolism.


Subject(s)
Animals , Rabbits , Pulmonary Embolism/blood , von Willebrand Factor/analysis , Platelet Glycoprotein GPIIb-IIIa Complex/analysis , Platelet Glycoprotein GPIIb-IIIa Complex/drug effects , P-Selectin/blood , Troponin I/blood , Nitric Oxide/administration & dosage , Pulmonary Embolism/pathology , Pulmonary Embolism/drug therapy , Reference Values , Time Factors , Administration, Inhalation , von Willebrand Factor/drug effects , Reproducibility of Results , Treatment Outcome , P-Selectin/drug effects , Troponin I/drug effects , Disease Models, Animal , X-Ray Microtomography , Heart Ventricles/pathology , Myocardium/pathology
14.
Article in English | WPRIM | ID: wpr-715000

ABSTRACT

Chronic kidney disease is a major global health problem affecting millions of people; kidney tissue engineering provides an opportunity to better understand this disease, and has the capacity to provide a cure. Two-dimensional cell culture and decellularised tissue have been the main focus of this research thus far, but despite promising results these methods are not without their shortcomings. Polymer fabrication techniques such as electrospinning have the potential to provide a non-woven path for kidney tissue engineering. In this experiment we isolated rat primary kidney cells which were seeded on electrospun poly(lactic acid) scaffolds. Our results showed that the scaffolds were capable of sustaining a multipopulation of kidney cells, determined by the presence of: aquaporin-1 (proximal tubules), aquaporin-2 (collecting ducts), synaptopodin (glomerular epithelia) and von Willebrand factor (glomerular endothelia cells), viability of cells appeared to be unaffected by fibre diameter. The ability of electrospun polymer scaffold to act as a conveyor for kidney cells makes them an ideal candidate within kidney tissue engineering; the non-woven path provides benefits over decellularised tissue by offering a high morphological control as well as providing superior mechanical properties with degradation over a tuneable time frame.


Subject(s)
Animals , Aquaporin 2 , Cell Culture Techniques , Global Health , Kidney , Polymers , Rats , Renal Insufficiency, Chronic , Tissue Engineering , von Willebrand Factor
15.
Article in Chinese | WPRIM | ID: wpr-689586

ABSTRACT

<p><b>OBJECTIVE</b>To detect the serum levels of platelet microparticle (PMP), fibronectin (FN), and von Willebrand Factor (vWF) in acute leukemia (AL) patients with thrombocytopenic and to analyze the relationship of the serum levels of PMP, FN and vWF with bleeding degree.</p><p><b>METHODS</b>One hundred and one newly diagnosed AL patients from May 2014 to May 2017 were enrolled the AL group. According to the WHO standard of bleeding stratification, 101 AL patients were divided into 5 sub groups: 0, 1, 2, 3 and 4 score groups; 52 normal persons subjected to physical examination were enrolled in control group. The PMP level was detected by flow cytometry; the FN and vWF levels were detected by ELISA. The levels of PMP, FN and vWF were compared between the AL group and the control group. The serum levels of PMP, FN and vWF were compared according to bleeding degree group. The relationship of bleeding degree with the serum levels of PMP, FN and vWF was analyzed.</p><p><b>RESULTS</b>The patients with newly diagnosed acute leukemia aged 18 to 60, and accounted for 61.39%. The degree of bleeding was mainly 1 score, which accounted for 38.61%. The serum levels of PMP, vWF and FN AL groups were significantly higher than those in control group (6.06%±4.38% vs 0.89%±0.50%, 205.82±24.89 vs 58.04±13.35 µg/L, 398.29±46.93 vs 311.37±26.02 µg/L)(P<0.001). The serum levels of PMP, FN and vWF were different among 5 subgroup (P<0.01); the level of PMP and FN were the highest in 0 score group and the lowest in 4 score group; the vWF level was the highest in 4 score groups and the lowest in 0 score group. The bleeding degree in the patients with acute leukemia negatively correlated with PMP level, and positively with NF and vWF levels (r=-0.753, r=0.648, r=0.805).</p><p><b>CONCLUSION</b>According to the relationship of the bleeding degree with serum levels of PMP, FN, vWF in patients, the detection of PMP, vWF and FN levels can help to evaluale the bleeding degree in the patients.</p>


Subject(s)
Acute Disease , Adolescent , Adult , Cell-Derived Microparticles , Hemorrhage , Humans , Leukemia , Middle Aged , Young Adult , von Willebrand Factor
16.
Journal of Experimental Hematology ; (6): 1230-1234, 2018.
Article in Chinese | WPRIM | ID: wpr-689500

ABSTRACT

Thrombotic thrombocytopenic purpura (TTP) is an acute, potentially life-threatening thrombotic microangiopathies (TMA). TTP has mainly been diagnosed by clinical findings, such as thrombocytopenia and microangiopathic haemolytic anemia. In addition, the reduced activity of von Willebrand factor-cleaving metalloproteinase ADAMTS13 below 10% has been accepted internationally as a diagnostic criterion for TTP. In clinic, the accurate diagnosis and early initiation of therapy can significantly improve the survival rate of patients. Therapy should be focused on increasing ADAMTS13 activity and eliminating or inhibiting ADAMTS13 antibody by the infusion or exchange of therapeutic plasma and corticosteroids. Both the administration of recombinant ADAMTS13 and reducing the release of human neutrophil peptides (HNPs) would be novel promising strategies for the prevention of platelet-vWFinteraction. This review briefly summarizes the recent advances in terms of pathogenesis, diagnosis and treatment of TTP.


Subject(s)
ADAMTS13 Protein , Anemia, Hemolytic , Blood Platelets , Humans , Purpura, Thrombotic Thrombocytopenic , von Willebrand Factor
17.
Chinese Medical Journal ; (24): 1780-1785, 2018.
Article in English | WPRIM | ID: wpr-688107

ABSTRACT

<p><b>Background</b>Although much attention has been paid to the pharmacokinetics (PKs) of different factor VIII (FVIII) concentrates in persons with hemophilia A (HA), limited information is available in young boys with severe HA. In this study, we aimed to assess the PK parameters of FVIII products in boys with severe HA in China.</p><p><b>Methods</b>A total of 36 boys (plasma-derived [pd]-FVIII, n = 15; recombinant [r] FVIII, n = 21) were enrolled between January 2015 and May 2016 in Beijing Children's Hospital. PK characteristics of FVIII products were studied according to a reduced 4-sampling time point design (1 h, 9 h, 24 h, and 48 h postinfusion).</p><p><b>Results</b>The mean FVIII half-life (t) was 10.99 ± 3.45 h (range 5.52-20.02 h), the mean in vivo recovery (IVR) was 2.01 ± 0.42 IU/dl per IU/kg (range 1.24-3.02 IU/dl per IU/kg) and mean clearance (CL) of FVIII is 4.34 ± 1.58 ml·kg·h (range 2.29-7.90 ml·kg·h). We also analyzed the influence of several parameters that potentially modulate FVIII PK. The age was closely associated with FVIII half-life (R = 0.32, P < 0.01). The tof FVIII increased by 0.59 h per year. Besides age, von Willebrand factor antigen (VWF:Ag) also was associated with FVIII half-life (R = 0.52, P < 0.01). Patients with blood Group O had a shorter FVIII half-life than patients with non-O blood group (9.40 ± 0.68 h vs. 12.3 ± 0.79 h, t = 2.70, P = 0.01). The FVIII IVR correlated with age (R = 0.21, P < 0.01) and VWF:Ag level (R = 0.28, P < 0.01). CL rates were faster in young patients and in those with low-VWF:Ag levels. CL rates of FVIII are higher in blood Group O versus non-blood Group O persons (5.02 ± 0.38 vs. 4.00 ± 0.32 ml·kg·h, t = 2.53, P = 0.02).</p><p><b>Conclusions</b>Chinese boys with severe HA have similar PK values to other ethnic groups and large differences in FVIII PK between individual patients. Age, blood group, and VWF:Ag levels are important determining factors for FVIII CL.</p>


Subject(s)
Adolescent , Blood Coagulation Tests , Child , Child, Preschool , China , Factor VIII , Pharmacokinetics , Hemophilia A , Drug Therapy , Humans , Male , von Willebrand Factor
18.
Article in Korean | WPRIM | ID: wpr-158037

ABSTRACT

Thrombotic thrombocytopenic purpura (TTP) is a rare blood disorder with a mortality rate of over 90% without prompt treatment. It is caused by congenital, idiopathic, or secondary diseases; idiopathic TTP is mainly associated with deficiency of ADAMTS13, a von Willebrand factor cleaving protease or ADAMTS13 inhibitors. The long-term survival rate of TTP has improved since the introduction of therapeutic plasma exchange (TPE), and the therapeutic aims have also been established. However, deciding on the end-point and appropriate treatment method requires careful assessment of clinical conditions of patients. The present study reports a case of a 33-year-old male patient with reduced ADAMTS13 activity and ADAMTS13 inhibitor, who developed symptoms after an early termination of TPE with improved symptoms, which finally improved with retreatment and additionally corticosteroid. We report our case with relevant literature review on TPE in TTP with this case.


Subject(s)
Adult , Humans , Male , Methods , Mortality , Plasma Exchange , Plasma , Plasmapheresis , Purpura, Thrombotic Thrombocytopenic , Retreatment , Survival Rate , von Willebrand Factor
19.
Article in Korean | WPRIM | ID: wpr-197957

ABSTRACT

BACKGROUND: Von Willebrand disease (VWD) is the most common inherited bleeding disorder. Surgery, even relatively minor procedures, in patients with moderate to severe qualitative and quantitative deficiencies of von Willebrand factor (VWF) can be associated with a life-threatening risk of excessive bleeding. The purpose of this study was to evaluate the safety and efficacy of VWF/FVIII in patients with von Willebrand disease before surgery and determine the efficacy of VWF/FVIII. METHODS: We reviewed the results of surgical procedures in patients with VWD at Kyung Hee University Hospital at Gangdong, between September 2009 and January 2016. VWF/FVIII concentrates were administrated preoperatively to all patients. RESULTS: Between September 2009 and January 2016 at our center, eight surgical procedures were performed successfully and no severe complications were observed in the seven patients with VWD. Four orthopedic procedures, one laparoscopic appendectomy, one ovary cystectomy, one strabotomy, and one dental extraction were performed. The median duration of hospitalization was seven days. VWF/FVIII concentrates were administered prior to all procedures, including the dental extraction. In all cases, uncontrolled bleeding and thromboembolic complications were not observed. CONCLUSION: Patients with VWD who require surgery can be treated efficiently and safely with VWF/FVIII concentrates. There is excellent tolerance, efficacy and safety in preventing excessive bleeding during surgery. When administering VWF/FVIII concentrates, treatment should be monitored with VWF Ag, VWF:RCo and FVIII plasma levels.


Subject(s)
Appendectomy , Cystectomy , Female , Hemorrhage , Hospitalization , Humans , Orthopedic Procedures , Ovary , Plasma , von Willebrand Diseases , von Willebrand Factor
20.
Article in English | WPRIM | ID: wpr-8646

ABSTRACT

Von Willebrand factor (vWF) is a glycoprotein with a crucial role in the formation of platelet thrombi, and ADAMTS13 is the main enzyme responsible for vWF cleavage. Both are important in the relationship between diabetic nephropathy, hypercoagulability, and cardiovascular disease. This study evaluated a potential relationship between vitamin D (vitD) levels, vWF, ADAMTS13 activity, and inflammation in diabetic patients on chronic hemodialysis (HD). Blood samples from 52 diabetic patients on chronic HD were obtained to determine vitD levels, vWF, and ADAMTS13 activity, and inflammatory markers. HD patients were grouped according to 25-hydroxyvitamin D [25(OH) VitD]25 nmol/L (n=36). vWF antigen and vWF activity were elevated in both groups, with an average of 214.3±82.6% and 175.8±72.6%, respectively. Average ADAMTS13 activity was within the normal range in both groups. Blood samples from the vitD <25 nmol/L group showed a positive correlation between c-reactive protein (CRP) and vWF levels (P=0.023; r=0.564; 95% confidence interval=0.095-0.828), with a negative correlation between HbA1c and 25(OH) VitD (P=0.015; r=-0.337; 95% confidence interval=-0.337-0.19). Diabetic patients on chronic HD had elevated vWF levels and activity with no significant change in ADAMTS13 activity. The correlation between CRP and vWF levels in the 25(OH) VitD<25 nmol/L group suggests inflammatory-related endothelial dysfunction in these patients.


Subject(s)
ADAMTS13 Protein/metabolism , Aged , C-Reactive Protein/analysis , Diabetes Mellitus, Type 2/complications , Female , Glycated Hemoglobin A/analysis , Humans , Male , Middle Aged , Renal Dialysis , Renal Insufficiency, Chronic/complications , Vitamin D/analogs & derivatives , von Willebrand Factor/metabolism
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