Database : MEDLINE
Search on : von and Willebrand and Diseases [Words]
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[PMID]: 29377939
[Au] Autor:Budzyn M; Iskra M; Turkiewicz W; Krasinski Z; Gryszczynska B; Kasprzak MP
[Ad] Address:Department of General Chemistry, Chair of Chemistry and Clinical Biochemistry, Poznan University of Medical Sciences, Poznan, Poland.
[Ti] Title:Plasma concentration of selected biochemical markers of endothelial dysfunction in women with various severity of chronic venous insufficiency (CVI)-A pilot study.
[So] Source:PLoS One;13(1):e0191902, 2018.
[Is] ISSN:1932-6203
[Cp] Country of publication:United States
[La] Language:eng
[Ab] Abstract:BACKGROUND: Although the endothelial dysfunction is considered to be implicated in the pathogenesis of chronic venous insufficiency (CVI) the endothelial status in patients with venous disorders is still not fully evaluated. Therefore the aim of the study was to measure the concentration of selected markers of endothelial dysfunction: von Willebrand factor (vWf), soluble P-selectin (sP-selectin), soluble thrombomodulin (sTM) and soluble VE-cadherin (sVE-cadherin) in CVI women who constitute the most numerous group of patients suffering from venous disease. MATERIALS AND METHODS: Forty four women with CVI were involved in the study and divided into subgroups based on CEAP classification. Concentration of vWf, sP-selectin, sTM and sVE-cadherin were measured and compared with those obtained in 25 healthy age and sex-matched women. RESULTS: It was found that the concentration of sTM increased and sVEcadherin decreased along with disease severity in CVI women. A significant rise of sTM was observed especially in CVI women, with the highest inflammation status reflected by hsCRP or elastase concentration, and in CVI women with a high oxidative stress manifested by an increased plasma MDA. A significant fall of circulating sVE-cadherin was reported in CVI women with moderate to highest intensity of inflammation and oxidative stress. There was no change in vWF and sP-selectin concentration at any stage of CVI severity. CONCLUSIONS: The results of the present study demonstrate the presence of endothelial dysfunction in women suffering from CVI which seems to progress with the disease severity and may be associated with inflammation and enhanced oxidative stress.
[Mh] MeSH terms primary: Biomarkers/blood
Endothelium, Vascular/physiopathology
Venous Insufficiency/blood
[Mh] MeSH terms secundary: Adult
Antigens, CD/blood
Cadherins/blood
Chronic Disease
Female
Humans
Middle Aged
P-Selectin/blood
Pilot Projects
Severity of Illness Index
Thrombomodulin/blood
von Willebrand Factor/metabolism
[Pt] Publication type:JOURNAL ARTICLE; RESEARCH SUPPORT, NON-U.S. GOV'T
[Nm] Name of substance:0 (Antigens, CD); 0 (Biomarkers); 0 (Cadherins); 0 (P-Selectin); 0 (Thrombomodulin); 0 (cadherin 5); 0 (von Willebrand Factor)
[Em] Entry month:1803
[Cu] Class update date: 180309
[Lr] Last revision date:180309
[Js] Journal subset:IM
[Da] Date of entry for processing:180130
[St] Status:MEDLINE
[do] DOI:10.1371/journal.pone.0191902

  2 / 8048 MEDLINE  
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[PMID]: 29369835
[Au] Autor:Jehangir A; Pathak R; Ukaigwe A; Donato AA
[Ad] Address:Department of Internal Medicine, Reading Health System, West Reading.
[Ti] Title:Association of aortic valve disease with intestinal angioectasia: data from the Nationwide Inpatient Sample.
[So] Source:Eur J Gastroenterol Hepatol;30(4):438-441, 2018 Apr.
[Is] ISSN:1473-5687
[Cp] Country of publication:England
[La] Language:eng
[Ab] Abstract:OBJECTIVE: Since the description of a correlation between aortic stenosis and angioectasia, controversy has persisted about whether these diseases are truly associated or coincidental findings of older age. Our objective was to determine the association of aortic valve disease and bleeding intestinal angioectasia from a large database. PATIENTS AND METHODS: We used the 2011 Nationwide Inpatient Sample database to identify hospitalizations in the USA in patients with bleeding intestinal angioectasia. International Classification of Diseases, 9th revision, Clinical Modification codes were used to identify patients with aortic valve disease, mitral valve disease, and known risk factors for angioectasia (including diagnosed von Willebrand disease, left ventricular assist device, and chronic kidney disease). Univariate and multivariate logistic regression were used to determine the odds of association between the valvular diseases and angioectasia. RESULTS: A total of 32 079 intestinal angioectasia-related hospitalizations were identified of which 7.02% (n=2253) cases had coexistent aortic valve disease. The unadjusted odds of aortic valve disease in association with bleeding intestinal angioectasia versus those without bleeding angioectasia was 4.95 [95% confidence interval: (CI): 4.43-5.54, P<0.001]. The association of intestinal angioectasia with mitral valve disease was not significant (odds ratio=1.56, 95% CI: 0.59-4.14, P=0.38). When adjusted for age and known risk factors, the odds of aortic valve disease in bleeding intestinal angioectasia was still significant (odds ratio=2.37, 95% CI: 2.10-2.66, P<0.001). CONCLUSION: Our findings support an important association between aortic valve disease and bleeding intestinal angioectasia, not identified in valvular heart valvular diseases with lower shear stress (mitral valve disease).
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1801
[Cu] Class update date: 180306
[Lr] Last revision date:180306
[St] Status:In-Data-Review
[do] DOI:10.1097/MEG.0000000000001068

  3 / 8048 MEDLINE  
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[PMID]: 29344653
[Au] Autor:Yang Q; Xu H; Luo J; Zhang Q; Xie B; Yi S; Rong X; Wang J; Qin Z; Jiang T; Lin L; Zuo Y; Fan X
[Ad] Address:Genetic and Metabolic Central Laboratory, Guangxi Maternal and Child Health Hospital, Nanning, Guangxi 530023, P.R. China.
[Ti] Title:A novel variant of osteogenesis imperfecta type IV and low serum phosphorus level caused by a Val94Asp mutation in COL1A1.
[So] Source:Mol Med Rep;17(3):4433-4439, 2018 Mar.
[Is] ISSN:1791-3004
[Cp] Country of publication:Greece
[La] Language:eng
[Ab] Abstract:Osteogenesis imperfecta (OI) is a rare congenital disorder characterized by bone fragility and fractures, and associated with bone deformity, short stature, dentin, ligament and blue­gray eye sclera. OI is caused by a heterozygous mutation in collagen α­1(I) chain (COL1A1) or collagen α­2(I) chain (COL1A2) genes that encode α chains of type I collagen. Collagen α chain peptide contains an N­propeptide, which has a role in assembly and processing of collagen. Point mutations in the N­propeptide domain appear to trigger OI. In the present study, a novel heterozygous missense mutation, c.281T>A (p.Val94Asp), was identified in the von Willebrand C domain of N­terminal of type I collagen in an individual with type IV OI. The majority of N­terminal mutations are associated with OI/Ehlers­Danlos syndrome (EDS); however, in the present study, the affected individual did not suffer from EDS and the level of serum phosphorus of the patient was low (0.67 mmol/l). A number of clinical phenotypes were observed at the same variation site or in the same region on the polypeptide chain of COL1A, which suggests that additional genetic and environmental factors may influence the severity of OI. The present study may provide insight into the phenotype­genotype association in collagen-associated diseases and improve clinical diagnosis of OI.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1801
[Cu] Class update date: 180227
[Lr] Last revision date:180227
[St] Status:In-Process
[do] DOI:10.3892/mmr.2018.8436

  4 / 8048 MEDLINE  
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[PMID]: 29288565
[Au] Autor:Portier I; Vanhoorelbeke K; Verhenne S; Pareyn I; Vandeputte N; Deckmyn H; Goldenberg DS; Samal HB; Singh M; Ivics Z; Izsvák Z; De Meyer SF
[Ad] Address:Laboratory for Thrombosis Research, KU Leuven Campus Kulak Kortrijk, Kortrijk, Belgium.
[Ti] Title:High and long-term von Willebrand factor expression after Sleeping Beauty transposon-mediated gene therapy in a mouse model of severe von Willebrand disease.
[So] Source:J Thromb Haemost;16(3):592-604, 2018 Mar.
[Is] ISSN:1538-7836
[Cp] Country of publication:England
[La] Language:eng
[Ab] Abstract:Essentials von Willebrand disease (VWD) is the most common inherited bleeding disorder. Gene therapy for VWD offers long-term therapy for VWD patients. Transposons efficiently integrate the large von Willebrand factor (VWF) cDNA in mice. Liver-directed transposons support sustained VWF expression with suboptimal multimerization. SUMMARY: Background Type 3 von Willebrand disease (VWD) is characterized by complete absence of von Willebrand factor (VWF). Current therapy is limited to treatment with exogenous VWF/FVIII products, which only provide a short-term solution. Gene therapy offers the potential for a long-term treatment for VWD. Objectives To develop an integrative Sleeping Beauty (SB) transposon-mediated VWF gene transfer approach in a preclinical mouse model of severe VWD. Methods We established a robust platform for sustained transgene murine VWF (mVWF) expression in the liver of Vwf mice by combining a liver-specific promoter with a sandwich transposon design and the SB100X transposase via hydrodynamic gene delivery. Results The sandwich SB transposon was suitable to deliver the full-length mVWF cDNA (8.4 kb) and supported supra-physiological expression that remained stable for up to 1.5 years after gene transfer. The sandwich vector stayed episomal (~60 weeks) or integrated in the host genome, respectively, in the absence or presence of the transposase. Transgene integration was confirmed using carbon tetrachloride-induced liver regeneration. Analysis of integration sites by high-throughput analysis revealed random integration of the sandwich vector. Although the SB vector supported long-term expression of supra-physiological VWF levels, the bleeding phenotype was not corrected in all mice. Long-term expression of VWF by hepatocytes resulted in relatively reduced amounts of high-molecular-weight multimers, potentially limiting its hemostatic efficacy. Conclusions Although this integrative platform for VWF gene transfer is an important milestone of VWD gene therapy, cell type-specific targeting is yet to be achieved.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1712
[Cu] Class update date: 180226
[Lr] Last revision date:180226
[St] Status:In-Data-Review
[do] DOI:10.1111/jth.13938

  5 / 8048 MEDLINE  
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[PMID]: 27778439
[Au] Autor:Randi AM; Laffan MA
[Ad] Address:National Heart and Lung Institute, Imperial College, London, UK.
[Ti] Title:Von Willebrand factor and angiogenesis: basic and applied issues.
[So] Source:J Thromb Haemost;15(1):13-20, 2017 01.
[Is] ISSN:1538-7836
[Cp] Country of publication:England
[La] Language:eng
[Ab] Abstract:The recent discovery that von Willebrand factor (VWF) regulates blood vessel formation has opened a novel perspective on the function of this complex protein. VWF was discovered as a key component of hemostasis, capturing platelets at sites of endothelial damage and synthesized in megakaryocytes and endothelial cells (EC). In recent years, novel functions and binding partners have been identified for VWF. The finding that loss of VWF in EC results in enhanced, possibly dysfunctional, angiogenesis is consistent with the clinical observations that in some patients with von Willebrand disease (VWD), vascular malformations can cause severe gastrointestinal (GI) bleeding. In vitro and in vivo studies indicate that VWF can regulate angiogenesis through multiple pathways, both intracellular and extracellular, although their relative importance is still unclear. Investigation of these pathways has been greatly facilitated by the ability to isolate EC from progenitors circulating in the peripheral blood of normal controls and patients with VWD. In the next few years, these will yield further evidence on the molecular pathways controlled by VWF and shed light on this novel and fascinating area of vascular biology. In this article, we will review the evidence supporting a role for VWF in blood vessel formation, the link between VWF dysfunction and vascular malformations causing GI bleeding and how they may be causally related. Finally, we will discuss how these findings point to novel therapeutic approaches to bleeding refractory to VWF replacement therapy in VWD.
[Mh] MeSH terms primary: Neovascularization, Physiologic
von Willebrand Factor/metabolism
[Mh] MeSH terms secundary: Angiodysplasia/metabolism
Animals
Blood Coagulation
Blood Platelets/metabolism
Endothelial Cells/metabolism
Gastrointestinal Hemorrhage/blood
Glycoproteins/metabolism
Hemorrhage
Hemostasis
Humans
Megakaryocytes/metabolism
Mice
Neovascularization, Pathologic
Signal Transduction
Stem Cells/metabolism
Vascular Endothelial Growth Factor Receptor-2/metabolism
von Willebrand Diseases/metabolism
[Pt] Publication type:JOURNAL ARTICLE; REVIEW
[Nm] Name of substance:0 (Glycoproteins); 0 (von Willebrand Factor); EC 2.7.10.1 (Vascular Endothelial Growth Factor Receptor-2)
[Em] Entry month:1801
[Cu] Class update date: 180222
[Lr] Last revision date:180222
[Js] Journal subset:IM
[Da] Date of entry for processing:161026
[St] Status:MEDLINE
[do] DOI:10.1111/jth.13551

  6 / 8048 MEDLINE  
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[PMID]: 28743060
[Au] Autor:Murata M; Adachi H; Oshima S; Kurabayashi M
[Ad] Address:Gunma Prefectural Cardiovascular Center, Department of Cardiology, 3-12 Kameizumimachi, Maebashi, Gunma 371-0004, Japan. Electronic address: yarukimanmann2000@yahoo.co.jp.
[Ti] Title:Glucose fluctuation and the resultant endothelial injury are correlated with pancreatic ß cell dysfunction in patients with coronary artery disease.
[So] Source:Diabetes Res Clin Pract;131:107-115, 2017 Sep.
[Is] ISSN:1872-8227
[Cp] Country of publication:Ireland
[La] Language:eng
[Ab] Abstract:AIMS: We evaluated whether glucose fluctuation (GF) causes vascular endothelial injury and affects glucometabolic factors during lengthy oral glucose tolerance test (OGTT). METHODS: We enrolled consecutive 116 patients with coronary artery disease (CAD) who were performed coronary angiography and 4-h OGTT. Blood samples were collected before and 4h after glucose load to measure endothelial injury factor [von Willebrand factor (vWF) and vWF/a disintegrin-like and metalloproteinase with thrombospondin type-1 motifs 13 (ADAMTS-13) ratio]. GF was defined as maximum - minimum blood glucose levels during 4-h OGTT. We estimated the relationship between GF and glucometabolic factors. RESULTS: vWV and vWF/ADAMTS-13 ratio were significantly correlated with GF during 4-h OGTT. GF was significantly correlated with homeostasis model to assess insulin resistance (HOMA-IR) (R=0.262), Matsuda index (R=-0.405), insulinogenic index (R=-0.336), HbA1c (R=0.281) and disposition index (R=-0.672). When dividing patients into impaired and preserved category groups according to the average value of GF (122mg/dL), adjusted to age, sex, HOMA-ß, insulinogenic index, HOMA-IR, Matsuda index and HbA1c, disposition index was an independent risk factor for impaired GF [odds ratio (95% confidence interval): 2.87 (1.70-4.83), P<0.001]. CONCLUSION: Pancreatic ß cell dysfunction is associated with GF and causes endothelial injury in CAD patients.
[Mh] MeSH terms primary: Blood Glucose/metabolism
Coronary Artery Disease/etiology
Endothelial Cells/pathology
Glucose Tolerance Test/methods
Insulin-Secreting Cells/metabolism
[Mh] MeSH terms secundary: Aged
Coronary Artery Disease/pathology
Female
Humans
Insulin-Secreting Cells/pathology
Male
[Pt] Publication type:JOURNAL ARTICLE
[Nm] Name of substance:0 (Blood Glucose)
[Em] Entry month:1802
[Cu] Class update date: 180209
[Lr] Last revision date:180209
[Js] Journal subset:IM
[Da] Date of entry for processing:170726
[St] Status:MEDLINE

  7 / 8048 MEDLINE  
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[PMID]: 29297854
[Au] Autor:Hoore M; Rack K; Fedosov DA; Gompper G
[Ad] Address:Theoretical Soft Matter and Biophysics, Institute of Complex Systems and Institute for Advanced Simulation, Forschungszentrum Jülich, 52425 Jülich, Germany.
[Ti] Title:Flow-induced adhesion of shear-activated polymers to a substrate.
[So] Source:J Phys Condens Matter;30(6):064001, 2018 Feb 14.
[Is] ISSN:1361-648X
[Cp] Country of publication:England
[La] Language:eng
[Ab] Abstract:Adhesion of polymers and proteins to substrates plays a crucial role in many technological applications and biological processes. A prominent example is the von Willebrand factor (VWF) protein, which is essential in blood clotting as it mediates adhesion of blood platelets to the site of injury at high shear rates. VWF is activated by flow and is able to bind efficiently to damaged vessel walls even under extreme flow-stress conditions; however, its adhesion is reversible when the flow strength is significantly reduced or the flow is ceased. Motivated by the properties and behavior of VWF in flow, we investigate adhesion of shear-activated polymers to a planar wall in flow and whether the adhesion is reversible under flow stasis. The main ingredients of the polymer model are cohesive inter-monomer interactions, a catch bond with the adhesive surface, and the shear activation/deactivation of polymer adhesion correlated with its stretching in flow. The cohesive interactions within the polymer maintain a globular conformation under low shear stresses and allow polymer stretching if a critical shear rate is exceeded, which is directly associated with its activation for adhesion. Our results show that polymer adhesion at high shear rates is significantly stabilized by catch bonds, while at the same time they also permit polymer dissociation from a surface at low or no flow stresses. In addition, the activation/deactivation mechanism for adhesion plays a crucial role in the reversibility of its adhesion. These observations help us better understand the adhesive behavior of VWF in flow and interpret its adhesion malfunctioning in VWF-related diseases.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1801
[Cu] Class update date: 180207
[Lr] Last revision date:180207
[St] Status:In-Process
[do] DOI:10.1088/1361-648X/aaa4d5

  8 / 8048 MEDLINE  
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[PMID]: 29378354
[Au] Autor:Pan X; Gong YY; Xu Y; Ariens RAS; Routledge MN
[Ad] Address:Environmental Epidemiology Group, Leeds Institute for Cardiovascular and Metabolic Medicine, School of Medicine, University of Leeds, Leeds, United Kingdom.
[Ti] Title:Urban Particulate Matter Induces Changes in Gene Expression in Vascular Endothelial Cells that Are Associated with Altered Clot Structure In Vitro.
[So] Source:Thromb Haemost;118(2):266-278, 2018 Feb.
[Is] ISSN:2567-689X
[Cp] Country of publication:Germany
[La] Language:eng
[Ab] Abstract:BACKGROUND: Particulate matter contained in ambient air pollution has been associated with cardiovascular diseases in several epidemiological studies. OBJECTIVE: The aim of this study was to investigate the potential for urban particulate matter to induce changes in clot structure through interaction with vascular endothelial cells. METHODS: We examined the structure of clots formed on human umbilical vascular endothelial cells that had been treated with various types of particles versus those formed on untreated cells. Particles used were standard reference particulate matter from diesel engine emissions (SRM2975) and urban ambient collection (SRM2787). RESULTS: There was a dose-dependent increase in fibre density in clots formed on particle-treated endothelial cells. It was also found that exposure to the particles induced increased expression of tissue factor and reduced expression of thrombomodulin genes as measured by real-time polymerase chain reaction and increased expression of von Willebrand factor and plasminogen activation inhibitor-1 as measured by ELISA. CONCLUSION: These changes are consistent with increased procoagulant activity of air pollution particulate matter-treated endothelial cells and suggest that particulate matter has the potential to promote clot formation through changes induced in endothelial genes controlling clot formation.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1801
[Cu] Class update date: 180129
[Lr] Last revision date:180129
[St] Status:In-Data-Review
[do] DOI:10.1160/TH17-05-0362

  9 / 8048 MEDLINE  
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[PMID]: 28744907
[Au] Autor:Wu Y; Zhu L; Luo Y
[Ad] Address:Institute of Biomedical Manufacturing and Life Quality Engineering, School of Mechanical Engineering Shanghai Jiao Tong University, Shanghai, China.
[Ti] Title:Design and Hemocompatibility Analysis of a Double-Suction Injection Suspension Blood Pump Using Computational Fluid Dynamics Methods.
[So] Source:Artif Organs;41(11):979-987, 2017 Nov.
[Is] ISSN:1525-1594
[Cp] Country of publication:United States
[La] Language:eng
[Ab] Abstract:The blood pump has become a possible solution to heart diseases. For the prevention of device failure and hemocompatibility problems, a rotary pump with suspended bearing is a preferred solution. In our previous work, a novel injection suspension method has been introduced to levitate the rotor. The suspension method is totally passive. This study aims to apply this suspension method to a double-suction pump, and the property of the pump was investigated using computational fluid dynamics (CFD) methods. The flow field of the pump is simulated based on the SST k-ω turbulent model. The characteristic curves of the pump were calculated. At the nominal working point of 5 L/min, 100 mm Hg, the suspension force acting on the rotor was detected, which could reach 0.46 N with a gap of 150 µm. We compared the pump with a previously developed single-suction injection pump to evaluate the blood compatibility of the double-suction design. The average scalar shear stress values were 3.13 Pa for the double-suction pump and 7.10 Pa for the single-suction pump. Larger volumes in the single-suction pump were exposed to shear stresses higher than 10 Pa. Thresholds for the von Willebrand factor cleavage, platelet activation, and hemolysis were defined to be 9 Pa, 50 Pa, and 150 Pa, respectively. The volume fractions for the double-suction pump are lower for all thresholds. The normalized index of hemolysis (NIH) values for the two pumps were calculated to be 0.008 g/100 L and 0.016 g/100 L. Results proved that the double-suction pump has a better hemocompatibility compared with the single-suction pump.
[Mh] MeSH terms primary: Biocompatible Materials
Computer-Aided Design
Heart-Assist Devices
Hemodynamics
Models, Cardiovascular
Prosthesis Design
[Mh] MeSH terms secundary: Heart-Assist Devices/adverse effects
Hemolysis
Hydrodynamics
Materials Testing
Regional Blood Flow
Reproducibility of Results
Stress, Mechanical
[Pt] Publication type:JOURNAL ARTICLE; VALIDATION STUDIES
[Nm] Name of substance:0 (Biocompatible Materials)
[Em] Entry month:1801
[Cu] Class update date: 180129
[Lr] Last revision date:180129
[Js] Journal subset:IM
[Da] Date of entry for processing:170727
[St] Status:MEDLINE
[do] DOI:10.1111/aor.12888

  10 / 8048 MEDLINE  
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[PMID]: 27771173
[Au] Autor:Farkas P; Csuka D; Mikes B; Sinkovits G; Réti M; Németh E; Rácz K; Madách K; Gergely M; Demeter J; Prohászka Z
[Ad] Address:3rd Department of Internal Medicine, Research Laboratory and Füst György Complement Diagnostic Laboratory, Semmelweis University, Budapest, Hungary.
[Ti] Title:Complement activation, inflammation and relative ADAMTS13 deficiency in secondary thrombotic microangiopathies.
[So] Source:Immunobiology;222(2):119-127, 2017 02.
[Is] ISSN:1878-3279
[Cp] Country of publication:Netherlands
[La] Language:eng
[Ab] Abstract:BACKGROUND: The secondary forms of hemolytic uremic syndrome/thrombotic thrombocytopenic purpura (secondary TMA) emerge as complications of coexisting diseases. OBJECTIVES: We hypothesized that secondary TMA could be characterized by the presence of relative ADAMTS13 deficiency and complement activation, and this relationship may have a prognostic value for outcome. PATIENTS AND METHODS: Fifty-three patients with thrombotic microangiopathy (TMA) and coexisting disease (such as malignancies, sepsis, heart surgery with extracorporeal circulation, solid organ transplantation, systemic autoimmune disorders), 41 patient controls, and 34 healthy controls were enrolled in our case-control study with 30days follow-up. Complement profile (from serum) and activation products, von Willebrand factor (VWF, from EDTA plasma), and ADAMTS13 activity were determined. RESULTS: ADAMTS13 activity was reduced, while VWF level was elevated in secondary TMA patients. The activity of the classical, lectin and alternative pathways, as well as the levels of C3, C4, and Factor H were significantly lower in secondary TMA patients, and were accompanied by high activation product levels (C3a and sC5b-9). Factor H concentration correlated to relative ADAMTS13 deficiency (i.e. VWF/ADAMTS13 ratio (r=-0.368, p=0.019)). 28/53 patients (53%) died during the follow-up period. Increased sC5b-9, C3a, and C reactive protein levels were all associated with a poor patient outcome. CONCLUSIONS: Our results indicate that the secondary TMA syndrome and its poor outcome is characterized by relative ADAMTS13 deficiency, inflammation, and complement activation with consumption via the classical and alternative pathways. It is yet to be determined whether complement inhibition could be a possible therapeutic option for patients with secondary TMA.
[Mh] MeSH terms primary: ADAMTS13 Protein/deficiency
Complement Activation/immunology
Complement System Proteins/immunology
Inflammation/complications
Thrombotic Microangiopathies/etiology
[Mh] MeSH terms secundary: Adolescent
Adult
Aged
Aged, 80 and over
Biomarkers
Case-Control Studies
Child
Child, Preschool
Comorbidity
Complement Activation/genetics
Complement System Proteins/metabolism
Female
Humans
Male
Middle Aged
Prognosis
Proportional Hazards Models
Thrombotic Microangiopathies/diagnosis
Thrombotic Microangiopathies/metabolism
Thrombotic Microangiopathies/mortality
Young Adult
[Pt] Publication type:JOURNAL ARTICLE; RESEARCH SUPPORT, NON-U.S. GOV'T
[Nm] Name of substance:0 (Biomarkers); 9007-36-7 (Complement System Proteins); EC 3.4.24.87 (ADAMTS13 Protein)
[Em] Entry month:1712
[Cu] Class update date: 180125
[Lr] Last revision date:180125
[Js] Journal subset:IM
[Da] Date of entry for processing:161025
[St] Status:MEDLINE


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