CCL

Összesen 9 találat.
#/oldal:
Részletezés:
Rendezés:

1.

001-es BibID:BIBFORM035082
Első szerző:Cauwenberghs, Nancy
Cím:Fc-receptor Dependent Platelet Aggregation Induced by Monoclonal Antibodies against Platelet Glycoprotein Ib or von Willebrand Factor / Nancy Cauwenberghs, Agotha Schlammadinger, Stephan Vauterin, Susan Cooper, Gretel Descheemaeker, István Tornai, Hans Deckmyn
Dátum:2001
ISSN:0340-6245
Megjegyzések:In this paper we describe two pathways leading to platelet activation by crosslinking glycoprotein (GP) Ibalpha to the platelet Fc-receptor (FcgammaRII). First the monoclonal antibody (MoAb) 9C8, raised against human platelet GPIbalpha, dose-dependently induced platelet aggregation of citrate-anticoagulated platelet-rich plasma, an effect that can be inhibited by several activation inhibitors. The FcgammaRII-inhibitory MoAb IV.3 was able to prevent the aggregatory effects of MoAb 9C8, indicating that crosslinking of the antigen GPIbalpha to the FcgammaII-receptor is necessary for the activating effect. Secondly we observed a synergistic activating effect of two anti-von Willebrand factor (vWF) MoAbs IC1E7 and B724, both known to enhance vWF binding to GPIbalpha in the presence of shear or ristocetin. When these antibodies are added together to PRP, platelet aggregation is induced without further need for an additional modulator. This effect can be blocked by either MoAb IV.3 or an inhibitory anti-GPIb MoAb, indicating that again the platelet activation results from signaling through FcgammaRII crosslinked to vWF bound to GPIbalpha. In addition, both the anti-GPIb MoAb 9C8, or the two anti-vWF MoAbs 1C1E7 and B724 induce genuine platelet activation, as evidenced by the secretion of ATP and protein tyrosine phosphorylation. These findings with both anti-GPIb and anti-vWF MoAbs add further proof to recent reports demonstrating an interaction between the platelet receptors GPIb and FcgammaRII, suggesting a role for the FcgammaII-receptor in GPIb-related signaling.
Tárgyszavak:Orvostudományok Klinikai orvostudományok idegen nyelvű folyóiratközlemény külföldi lapban
Monoclonal antibody
von Willebrand factor
glycoprotein Ib
FeyII-receptor
külföldön készült közlemény
platelet activation
Megjelenés:Thrombosis and Haemostasis. - 85 : 4 (2001), p. 679-685. -
További szerzők:Schlammadinger Ágota (1971-) (belgyógyász, haematológus) Vauterin, Stephan Cooper, Susan Descheemaeker, Gretel Tornai István (1954-) (belgyógyász, gasztroenterológus) Deckmyn, Hans
Internet cím:Intézményi repozitóriumban (DEA) tárolt változat
Borító:

2.

001-es BibID:BIBFORM019249
Első szerző:Gézsi András
Cím:Accelerated clearance alone explains ultra-large multimers in von Willebrand disease Vicenza / A. Gézsi, U. Budde, I. Deák, E. Nagy, A. Mohl, Á. Schlammadinger, Z. Boda, T. Masszi, J. E. Sadler, I. Bodó
Dátum:2010
Megjegyzések:von Willebrand disease (VWD) Vicenza is characterized by low plasma von Willebrand factor (VWF) levels, the presence of ultra-large (UL) VWF multimersand less prominent satellite bands on multimer gels, and the heterozygous amino acid substitution R1205H in the VWFgene. The pathogenesis of VWD Vicenza has been elusive.Accelerated clearance is implicated as a cause of low VWF level. Objectives: We addressed the question, whether the presence of ultra-large multimers is a cause, or a result of accelerated VWF clearance, or whether it is an unrelated phenomenon. Patients/methods: We studied the detailed phenotype of three Hungarian patients with VWD Vicenza, expressed the mutant VWFR1205H in 293T cells and developed a mathematical model to simulate VWF synthesis and catabolism. Results: We found that the half-life of VWF after DDAVP was approximately one tenth of that after the administration of Haemate P, a source of exogenous wild-type (WT) VWF (0.81 ? 0.2 vs. 7.25 ? 2.38 h). An analysis of recombinant mutant VWF-R1205H showed that the biosynthesis and multimer structure ofWT and mutant VWF were indistinguishable. A mathematical model of the complex interplay of VWF synthesis, clearance and cleavageshowed that decreasing VWF half-life to one-tenth of normalreproduced all features of VWD Vicenza including low VWFlevel, ultra-large multimers and a decrease of satellite band intensity. Conclusion: We conclude that accelerated clearance alone may explain all features of VWD Vicenza.
Tárgyszavak:Orvostudományok Klinikai orvostudományok idegen nyelvű folyóiratközlemény külföldi lapban
clearance
von Willebrand disease
von Willebrand factor
Megjelenés:Journal of Thrombosis and Haemostasis. - 8 : 6 (2010), p. 1273-1280. -
További szerzők:Budde, U. Deák Ivett Nagy E. Mohl, A. Schlammadinger Ágota (1971-) (belgyógyász, haematológus) Boda Zoltán (1947-) (belgyógyász, haematologus, klinikai onkológus) Masszi Tamás Sadler, J. E. Bodó I.
Internet cím:DOI
Intézményi repozitóriumban (DEA) tárolt változat
Borító:

3.

001-es BibID:BIBFORM007183
Első szerző:Ilonczai Péter (orvos, belgyógyász, haematológus szakorvos)
Cím:Temporarily successful eradication therapy in acquired haemophilia with high inhibitor titer : a case report with a new protocol / Péter Ilonczai, Ágota Schlammadinger, Zsolt Oláh, Katalin Rázsó, Zsuzsanna Bereczky, Zoltán Boda
Dátum:2008
Tárgyszavak:Orvostudományok Klinikai orvostudományok idegen nyelvű folyóiratközlemény külföldi lapban
Megjelenés:Thombosis and Haemostasis. - 100 : 1 (2008), p. 149-150. -
További szerzők:Schlammadinger Ágota (1971-) (belgyógyász, haematológus) Oláh Zsolt (1974-) (belgyógyász) Molnárné Rázsó Katalin (1966-) (belgyógyász, haematológus, klinikai onkológus) Bereczky Zsuzsanna (1974-) (orvosi laboratóriumi diagnosztika szakorvos) Boda Zoltán (1947-) (belgyógyász, haematologus, klinikai onkológus)
Internet cím:DOI
elektronikus változat
Borító:

4.

001-es BibID:BIBFORM034335
Első szerző:Mohl, A.
Cím:Common large partial VWF gene deletion does not cause alloantibody formation in the Hungarian type 3 von Willebrand disease population / Mohl A., Boda Z., Jager R., Losonczy H., Marosi A., Masszi T., Nagy E., Nemes L., Obser T., Oyen F., Radványi G., Schlammadinger Á., Szélessy Zs., Várkonyi A., Vezendy K., Vilimi B., Schneppenheim R., Bodó I.
Dátum:2011
ISSN:1538-7933
Megjegyzések:BACKGROUND: Type 3 von Willebrand disease (VWD) is an autosomal recessive bleeding disorder, characterized by virtually undetectable plasma von Willebrand factor (VWF) and consequently reduced plasma factor VIII levels. Genetic mutations responsible for type 3 VWD are very heterogeneous, scattered throughout the VWF gene and show high variability among different populations. METHODS: Twenty-five severe VWD patients were studied by direct sequencing of the 51 coding exons of the VWF gene. The total number of VWD type 3 families in Hungary is 24, of which 23 were investigated. RESULTS: Fifteen novel mutations were identified in 31 alleles, five being nonsense mutations (p.Q1238X, p.Q1898X, p.Q1931X, p.S2505X and p.S2568X), four small deletions and insertions resulting in frame shifts (c.1992insC, c.3622delT, c.5315insGA and c.7333delG), one a large partial deletion (delExon1-3) of the 5'-region, four candidate missense mutations (p.C35R, p.R81G, p.C295S, p.C623T) and one a candidate splice site mutation (c.1730-10C>A). Six previously described mutations were detected in 17 alleles, including the repeatedly found c.2435delC, p.R1659X and p.R1853X. Only one patient developed alloantibodies to VWF, carrying a homozygous c.3622delT. CONCLUSION: We report the genetic background of the entire Hungarian type 3 VWD population. A large novel deletion, most probably due to a founder effect, seems to be unique to Hungarian type 3 VWD patients with high allele frequency. In contrast to previous reports, none of the five patients homozygous for the large partial deletion developed inhibitors to VWF. This discrepancy raises the possibility of selection bias in some of the reports.
Tárgyszavak:Orvostudományok Klinikai orvostudományok idegen nyelvű folyóiratközlemény külföldi lapban
genetic defect
type 3
von Willebrand disease
Megjelenés:Journal Of Thrombosis And Haemostasis. - 9 : 5 (2011), p. 945-952. -
További szerzők:Boda Zoltán (1947-) (belgyógyász, haematologus, klinikai onkológus) Jáger Rita Losonczy Hajna Marosi A. Masszi Tamás Nagy E. Nemes László Obser, T. Oyen, F. Radványi Gáspár Schlammadinger Ágota (1971-) (belgyógyász, haematológus) Szélessy Zs. Várkonyi Ágnes Vezendy K. Vilimi B. Schneppenheim, Reinhard Bodó I.
Internet cím:DOI
Intézményi repozitóriumban (DEA) tárolt változat
Borító:

5.

001-es BibID:BIBFORM035991
Első szerző:Réti Marienn
Cím:Complement activation in thrombotic thrombocytopenic purpura / Réti M., Farkas P., Csuka D., Rázsó K., Schlammadinger Á., Udvardy M. L., Madách K., Domján G., Bereczki C., Reusz G. S., Szabó A. J., Prohászka Z.
Dátum:2012
ISSN:1538-7933
Megjegyzések:Ultra-large von Willebrand factor and deficiency of its cleaving protease are important factors in the events leading to thrombotic microangiopathy; however, the mechanisms involved are only partly understood. Whereas pathological activation of the alternative complement pathway is linked to atypical hemolytic uremic syndrome, the role of complement activation in thrombotic thrombocytopenic purpura (TTP) is unknown. The aim of this study was to investigate whether signs of complement activation are characteristic of TTP. PATIENTS AND METHODS: Twenty-three patients with TTP (18 women, median age 38 years) and 17 healthy controls (13 women, median age 38 years) were included. Complement parameters (C3, Factors H, I, B and total alternative pathway activity) together with complement activation fragments (C3a) or complexes (C1rs-INH, C3bBbP, sC5b9) were measured by ELISA or RID. ADAMTS13 activity and anti-ADAMTS13 inhibitory antibodies were measured by the VWF-FRET73 assay. RESULTS: Increased levels of C3a, and SC5b9 were observed in TTP during acute episodes, as compared with healthy controls. Decreased complement C3 levels indicative of complement consumption occurred in 15% of acute TTP patients. Significant decrease of complement activation products C3a and SC5b9 was observed during plasma exchange (PEX). The sustained presence of anti-ADAMTS13 inhibitory antibodies in complete remission was associated with increased complement activation.
Tárgyszavak:Orvostudományok Klinikai orvostudományok idegen nyelvű folyóiratközlemény külföldi lapban
ADAMTS13 inhibitor
complement activation
TTP
Megjelenés:Journal Of Thrombosis And Haemostasis. - 10 : 5 (2012), p. 791-798. -
További szerzők:Farkas P. Csuka D. Molnárné Rázsó Katalin (1966-) (belgyógyász, haematológus, klinikai onkológus) Schlammadinger Ágota (1971-) (belgyógyász, haematológus) Udvardy Miklós László (1977-) (orvos, tudományos segédmunkatárs) Madách Krisztina Domján Gyula Bereczki Csaba Reusz György Szabó A. J. Prohászka Zoltán
Internet cím:Szerző által megadott URL
DOI
Intézményi repozitóriumban (DEA) tárolt változat
Borító:

6.

001-es BibID:BIBFORM034354
Első szerző:Schlammadinger Ágota (belgyógyász, haematológus)
Cím:Comparison of the O'Brien filter test and the PFA-100 platelet analyzer in the laboratory diagnosis of von Willebrand's disease / Agota Schlammadinger, Adrienne Kerenyi, Laszlo Muszbek, Zoltan Boda
Dátum:2000
ISSN:0340-6245
Megjegyzések:Von Willebrand's disease (vWD) is the most common congenital haemorrhagic diathesis, characterized by the quantitative or qualitative disorder of von Willebrand factor (vWF). A number of methods have been used for the diagnosis of the disease, and the bleeding time determination is widely accepted as a screening test in spite of its low sensitivity. Our aim was to evaluate and compare the performance of two high shear systems (the O'Brien filter test and the PFA-100 device) in the screening and diagnosis of vWD. Thirty patients (n=13 type 1 with mild symptoms, n = 9 type 1 with severe symptoms, n = 2 type 2A, n = 3 type 2B and n = 3 type 3 vWD) and twenty controls were investigated. In mild vWD the platelet retention in the second phase of the filter test with citrated blood showed the highest sensitivity (91.6%). The sensitivity of the PFA-100 method with collagen-epinephrine cartridges in this group was 76.9%, while the bleeding time was prolonged only in 15.4% of the cases. In severe type 1, in type 2A and type 3 all functional tests reflected the bleeding tendency of the patients. In type 2B disease the bleeding time was prolonged only when the patient was thrombocytopenic, but both high shear systems revealed the disease independently of the presence of thrombocytopenia. The overall sensitivity of the bleeding time determination was 50% compared to the 80-90% sensitivity of the O'Brien filter test and the PFA-100 system. The sensitivity values of the filter test and the PFA-100 device with collagen-epinephrine cartridges were in the same range, but the collagen-ADP cartridges showed a lower (65.5%) sensitivity, though the results were specific and had high positive predictive value. We conclude that both high shear systems are suitable for the screening of vWD, and that they are superior to the traditional bleeding time determination in case of mild disease or type 2B vWD.
Tárgyszavak:Orvostudományok Klinikai orvostudományok idegen nyelvű folyóiratközlemény külföldi lapban
von Willebrand' disease
high shear induced platelet activation
O'Brien filter test
PFA-100 system
Megjelenés:Thrombosis And Haemostasis. - 84 : 1 (2000), p. 88-92. -
További szerzők:Kerényi Adrienne (1970-) (laboratóriumi szakorvos) Muszbek László (1942-) (haematológus, kutató orvos) Boda Zoltán (1947-) (belgyógyász, haematologus, klinikai onkológus)
Internet cím:Intézményi repozitóriumban (DEA) tárolt változat
Borító:

7.

001-es BibID:BIBFORM002138
Első szerző:Szántó Tímea
Cím:The A/T1381 polymorphism in the A1-domain of von Willebrand factor influences the affinity of von Willebrand factor for platelet glycoprotein Ibalpha / Szanto T., Schlammadinger A., Staelens S., De Meyer S. F., Freson K., Pareyn I., Vauterin S., Harsfalvi J., Deckmyn H., Vanhoorelbeke K.
Dátum:2007
Tárgyszavak:Orvostudományok Elméleti orvostudományok idegen nyelvű folyóiratközlemény külföldi lapban
Megjelenés:Thrombosis Haemostasis. - 98 : 1 (2007), p. 178-185. -
További szerzők:Schlammadinger Ágota (1971-) (belgyógyász, haematológus) Staelens, Stephanie De Meyer, Simon F. Freson, Kathleen Pareyn, Inge Vauterin, Stephan Deckmyn, Hans Vanhoorelbeke, Karen Hársfalvi Jolán (1949-) (klinikai biokémikus)
Internet cím:elektronikus változat
DOI
Borító:

8.

001-es BibID:BIBFORM002137
Első szerző:Szántó Tímea
Cím:Type 2B von Willebrand disease in seven individuals from three different families : phenotypic and genotypic characterization / Szanto T., Schlammadinger A., Salles I., Pareyn I., Vauterin S., Harsfalvi J., Vanden Bulcke A. M., Deckmyn H., Vanhoorelbeke K.
Dátum:2007
Tárgyszavak:Orvostudományok Elméleti orvostudományok idegen nyelvű folyóiratközlemény külföldi lapban
Megjelenés:Thrombosis Haemostasis. - 98 : 1 (2007), p. 251-254. -
További szerzők:Schlammadinger Ágota (1971-) (belgyógyász, haematológus) Salles, Isabelle Pareyn, Inge Vauterin, Stephan Vanden Bulcke, Anne-Marie Deckmyn, Hans Vanhoorelbeke, Karen Hársfalvi Jolán (1949-) (klinikai biokémikus)
Internet cím:elektronikus változat
DOI
Borító:

9.

001-es BibID:BIBFORM035072
Első szerző:Tornai István (belgyógyász, gasztroenterológus)
Cím:Acquired Bernard-Soulier syndrome : a case with necrotizing vasculitis and thrombosis / Istvan Tornai, Zoltan Boda, Agota Schlammadinger, Attila Juhasz, Nancy Cauwenberghs, Hans Deckmyn, Jolan Harsfalvi
Dátum:1999
Megjegyzések:We describe a patient with positive antinuclear antibodies, polyclonal gammopathy and high level of circulating immunocomplexes, resulting in vascular purpura. In addition, the patient had a slightly prolonged bleeding time and an isolated defect of ristocetin-induced platelet aggregation (RIPA) in platelet-rich plasma (PRP). The patient's plasma also inhibited RIPA in normal PRP and in normal platelet suspension. The activity and multimeric structure of plasmatic von Willebrand factor showed no alteration. We could demonstrate an autoantibody against platelet membrane glycoprotein (GP) Ib, using an ELISA-type assay. These data suggest an acquired Bernard-Soulier syndrome. We suggest that the patient had an immunocomplex-mediated leukocytoclastic vasculitis accompanied by production of antinuclear autoantibodies as well as the presence of an autoantibody against GPIb. The titer of the anti-GPIb antibody, however, was too low to induce significant platelet-type bleeding tendency, only laboratory alterations were found. Moreover, in a later stage of her disease, she developed a severe necrotizing vasculitis which was followed by a deep venous thrombosis.
Tárgyszavak:Orvostudományok Klinikai orvostudományok idegen nyelvű folyóiratközlemény külföldi lapban
egyetemen (Magyarországon) készült közlemény
Megjelenés:Haemostasis. - 29 : 4 (1999), p. 229-236. -
További szerzők:Boda Zoltán (1947-) (belgyógyász, haematologus, klinikai onkológus) Schlammadinger Ágota (1971-) (belgyógyász, haematológus) Juhász Attila (1970-) (szakorvos, klinikai mikrobiológus) Cauwenberghs, Nancy Deckmyn, Hans Hársfalvi Jolán (1949-) (klinikai biokémikus)
Internet cím:Intézményi repozitóriumban (DEA) tárolt változat
Borító:
Rekordok letöltése1 
Corvina könyvtári katalógus v8.2.27 © 2023 Monguz kft. Minden jog fenntartva.