scholarly journals Von Willebrand factor, ADAMTS13 and mortality in dialysis patients

2021 ◽  
Vol 22 (1) ◽  
Author(s):  
Gurbey Ocak ◽  
Mark Roest ◽  
Marianne C. Verhaar ◽  
Maarten B. Rookmaaker ◽  
Peter J. Blankestijn ◽  
...  

Abstract Background Von Willebrand Factor (VWF) multimers are cleaved into smaller and less coagulant forms by the metalloprotease ADAMTS13. The aim of this study was to investigate the association between VWF and ADAMTS13 and mortality in dialysis patients. Methods We prospectively followed 956 dialysis patients. VWF levels and ADAMTS13 activity were measured. Cox proportional hazard analyses were used to calculate hazard ratios (HRs) with 95 % confidence intervals (CIs) to investigate the association between quartiles of VWF levels and ADAMTS13 activity and all-cause mortality. HRs were adjusted for age, sex, body mass index, cardiovascular disease, dialysis modality, primary kidney disease, use of antithrombotic medication, systolic blood pressure, albumin, C-reactive protein and residual GFR. Results Of the 956 dialysis patients, 288 dialysis patients died within three years (mortality rate 151 per 1000 person-years). The highest quartile of VWF as compared with lower levels of VWF was associated with a 1.4-fold (95 %CI 1.1–1.8) increased mortality risk after adjustment. The lowest quartile of ADAMTS13 activity as compared with other quartiles was associated with a 1.3-fold (95 %CI 1.0-1.7) increased mortality risk after adjustment. The combination of the highest VWF quartile and lowest ADAMTS13 quartile was associated with a 2.0-fold (95 %CI 1.3-3.0) increased mortality risk as compared with the combination of the lowest VWF quartile and highest ADAMTS13 quartile. Conclusions High VWF levels and low ADAMTS13 activity were associated with increased mortality risks in dialysis patients.

2018 ◽  
Vol 118 (08) ◽  
pp. 1397-1408 ◽  
Author(s):  
Eszter Palyu ◽  
Jolan Harsfalvi ◽  
Tamas Tornai ◽  
Maria Papp ◽  
Miklos Udvardy ◽  
...  

Background There is an unstable balance between pro- and anti-haemostatic processes in patients with cirrhosis. We hypothesized, that in patients with acute decompensation (AD) the major alterations of von Willebrand factor (VWF) could contribute to the pro-thrombotic situation as compared to patients with stable (ST) cirrhosis. Patients and Methods We analysed different parameters of VWF, including detailed multimer distribution by densitometry and platelet adhesion, together with a disintegrin-like and metalloproteinase with thrombospondin type-1 motifs 13 (ADAMTS13) activity and antigen and C-reactive protein (CRP) levels in patients with ST cirrhosis (n = 99), with AD (n = 54) and controls (n = 92). Results VWF antigen, ristocetin co-factor as well as collagen-binding activities were elevated in both cirrhotic groups in a stepwise manner. There was a decrease in high and an increase in low molecular weight multimer ratios in the majority of ST cirrhosis. However, in 24 out of 54 AD patients, ultra-large VWF multimers (ultra-large molecular weight multimers [ULMWM]) were found. ADAMTS13 activity in ST and AD patients without ULMWM was similar to controls (median [interquartile range; IQR]%: 98 [67–132] and 91 [60–110] vs. 106 [88–117], respectively). The presence of ULMWM in AD patients was associated with low ADAMTS13 activity [33 (24–49)%] and high CRP level [23 (7.1–83.6) mg/L]. Adhesion of normal platelets showed a stepwise increase in the presence of cirrhotic plasmas, reaching the highest level in AD patients with ULMWM. Conclusion Characteristic changes of VWF parameters are seen in ST cirrhosis. In AD patients, highly increased VWF and reduced ADAMTS13 activity could be found, along with the presence of ULMWM, which are possible markers and contributors of the disease progression.


Hepatology ◽  
2021 ◽  
Author(s):  
Patrick Starlinger ◽  
Joseph C. Ahn ◽  
Aidan Mullan ◽  
Georg P. Gyoeri ◽  
David Pereyra ◽  
...  

2016 ◽  
Vol 39 (4) ◽  
pp. 111 ◽  
Author(s):  
Nuran Cetın ◽  
Nadide M Sav ◽  
Duran Karabel ◽  
Ali Yildirim ◽  
Bilal Yıldız

Purpose: Cardiovascular diseases are the main causes of morbidity and mortality in children with end-stage renal disease and the relationships among several relevant potential biomarkers were investigated in pediatric peritoneal dialysis patients. Methods: Serum homocysteine, von Willebrand factor (vWF), apolipoproteins A and B, lipoprotein-a, high sensitive-CRP, hemoglobin, phosphorus and parathyroid hormone (PTH) levels, systolic (SBP) and diastolic (DBP) blood pressure, carotid intima-media thickness (cIMT) and left ventricular mass index (LVMI) were measured in 21 pediatric peritoneal dialysis patients and control subjects. Results: All risk factors were higher in patients than controls. LVMI values were negatively correlated with hemoglobin and positively correlated with PTH and phosphorus levels (p=0.007, r= - 0.573; p=0.013, r= 0.532 and p=0.035, r= 0.461, respectively). cIMT was negatively associated with serum albumin and positively correlated with vWF levels and with SBP and DBP (p=0.006, r= - 0.578; p=0.039, r= 0.453; p=0.02, r= 0.503; p=0.024, r= 0.491, respectively). Robust regression analyses showed that hemoglobin was an independent predictor of LVMI and serum albumin was an independent predictor of cIMT. Conclusion: Only uremia-related factors were independent risk factors for predicting LVMI and cIMT. Hemoglobin level may be a critical factor in the development of left ventricular hypertrophy; therefore, effective treatment of anemia is crucial. Low serum albumin and high hsCRP and vWF levels, and their correlations with cIMT, indicate these patients could be at risk of developing malnutrition–inflammation–atherosclerosis syndrome and suggest that serum albumin and vWF levels may be useful markers for early detection of vascular damage.


2016 ◽  
Vol 68 (5) ◽  
pp. 726-732 ◽  
Author(s):  
Sanaz Sedaghat ◽  
Paul S. de Vries ◽  
Johan Boender ◽  
Michelle A.H. Sonneveld ◽  
Ewout J. Hoorn ◽  
...  

2020 ◽  
Vol Volume 15 ◽  
pp. 543-552 ◽  
Author(s):  
Lasse L Langholm ◽  
Sarah Rank Rønnow ◽  
Jannie MB Sand ◽  
Diana Julie Leeming ◽  
Ruth Tal-Singer ◽  
...  

2009 ◽  
Vol 101 (02) ◽  
pp. 305-311 ◽  
Author(s):  
János Rigó Jr ◽  
Tamás Bõze ◽  
Zoltán Derzsy ◽  
László Cervenak ◽  
Veronika Makó ◽  
...  

SummaryThe activity of ADAMTS13, the von Willebrand factor (VWF) cleaving protease is low in several conditions, including HELLP (haemolysis, elevated liver enzymes, and low platelet count) syndrome. As HELLP syndrome develops in most cases on the basis of preeclampsia, our aim was to determine whether plasma ADAMTS13 activity is decreased in preeclampsia. Sixty-seven preeclamptic patients, 70 healthy pregnant women and 59 healthy non-pregnant women were involved in this case-control study. Plasma ADAMTS13 activity was determined with the FRETS-VWF73 assay, while VWF antigen (VWF:Ag) levels with an enzyme-linked immunosorbent assay. The multimeric pattern of VWF was analyzed by SDS-agarose gel electrophoresis. There was no significant difference in plasma ADAMTS13 activity between the preeclamptic and the healthy pregnant and non-pregnant groups (median [25–75 percentile]: 98.8 [76.5–112.8] %, 96.3 [85.6–116.2] % and 91.6 [78.5–104.4] %, respectively; p>0.05). However, plasma VWF:Ag levels were significantly higher in preeclamptic patients than in healthy pregnant and non-pregnant women (187.1 [145.6–243.1] % versus 129.3 [105.1–182.8] % and 70.0 [60.2–87.3] %, respectively; p<0.001). The multimeric pattern of VWF was normal in each group. Primiparas had lower plasma ADAMTS13 activity than multi-paras (92.6 [75.8–110.6] % versus 104.2 [92.1–120.8] %; p=0.011). No other relationship was found between clinical characteristics, laboratory parameters and plasma ADAMTS13 activity in either study group. In conclusion, plasma ADAMTS13 activity is normal in preeclampsia despite the increased VWF:Ag levels. However, further studies are needed to determine whether a decrease in plasma ADAMTS13 activity could predis-pose preeclamptic patients to develop HELLP syndrome.


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