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001-es BibID:	BIBFORM073777
Első szerző:	Pályu Eszter
Cím:	Major changes of von Willebrand factor multimer distribution in cirrhotic patients with stable disease or acute decompensation / Eszter Palyu, Jolan Harsfalvi, Tamas Tornai, Maria Papp, Miklos Udvardy, Katalin Szekeres-Csiki, Lajos Pataki, Karen Vanhoorelbeke, Hendrik B. Feys, Hans Deckmyn, Istvan Tornai
Dátum:	2018
ISSN:	0340-6245
Megjegyzések:	Background and aims: 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 prothrombotic situation as compared to patients with stable (ST) cirrhosis. Methods: We analyzed different parameters of VWF, including detailed multimer distribution by densitometry and platelet adhesion together with ADAMTS13 activity and antigen and C-reactive protein (CRP) levels in patients with ST (n=99), with AD (n=54) and controls (n=92). Results: VWF antigen, ristocetin cofactor 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 (ULMWM) were found. ADAMTS13 activity in ST and AD patients without ULMWM was similar to controls [median, (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.
Tárgyszavak:	Orvostudományok Klinikai orvostudományok idegen nyelvű folyóiratközlemény külföldi lapban cirrhosis acute decompensation systemic inflammation von Willebrand factor multimers trhombotic microangiopathy
Megjelenés:	Thrombosis and Haemostasis. - 118 : 8 (2018), p. 1397-1408. -
További szerzők:	Hársfalvi Jolán (1949-) (klinikai biokémikus) Tornai Tamás István (1984-) (belgyógyász) Papp Mária (1975-) (belgyógyász, gasztroenterológus) Udvardy Miklós (1947-) (belgyógyász, haematológus) Szekeres-Csiki Katalin (1979-) (orvos) Pataki Lajos Vanhoorelbeke, Karen Feys, Hendrik B. Deckmyn, Hans Tornai István (1954-) (belgyógyász, gasztroenterológus)
Internet cím:	Intézményi repozitóriumban (DEA) tárolt változat DOI
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