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001-es BibID:	BIBFORM034625
Első szerző:	Bonnefoy, Arnaud
Cím:	The subendothelium of the HMEC-1 cell line supports thrombus formation in the absence of von Willebrand factor and collagen types I, III and VI / Bonnefoy A., Harsfalvi J., Pfliegler G., Fauvel-Lafeve F., Legrand C.
Dátum:	2001
ISSN:	0340-6245
Megjegyzések:	The macromolecular composition of the extracellular matrix (ECM) produced by the human microvascular endothelial cell line (HMEC-1) was determined by ELISA and its thrombogenicity was studied in blood perfusion assays. Results were compared with those obtained with the ECM produced by human umbilical vein endothelial cells (HUVEC). The HMEC-1's ECM contains collagen type IV, fibronectin, laminin and thrombospondin, but no detectable levels of collagen types I, III and VI, or von Willebrand factor (vWF), whereas all these components were found in the ECM synthesized by HUVEC. HMEC-1's ECM was perfused with low-molecular-weight heparin-anticoagulated blood at two wall shear rates (650/s and 2,600/s), representative of moderate and high arterial wall shear rates, in parallel plate flow chambers for 5 min. This resulted in the formation of large platelet aggregates, compared to essentially a monolayer of adherent platelets on HUVEC's ECM. Interestingly, large thrombi were formed at 2,600/s when HMEC-1's ECM was perfused with the blood of a patient with severe type III von Willebrand disease lacking both plasma and platelet vWF, indicating that vWF was not absolutely required for thrombus formation on this matrix. Thrombin generated on the HMEC-1's ECM contributed importantly to the large platelet thrombi formed, shown by performing blood perfusion experiments in the presence of thrombin inhibitors. Our results indicate that 1) platelet adhesion and aggregate formation on a subendothelium may occur at a high shear rate (2600/s) without the participation of collagen types I, III and VI, and vWF; and 2) the HMEC-1 cell line may prove useful for in vitro studies of the thrombogenic properties of microvascular subendothelium which in most cases does not contain fibrillar collagens and vWF.
Tárgyszavak:	Orvostudományok Klinikai orvostudományok idegen nyelvű folyóiratközlemény külföldi lapban Thrombus formation von Willebrand factor collagen HMEC-1 shear rate extra cellular matrix (ECM)
Megjelenés:	Thrombosis And Haemostasis. - 85 : 3 (2001), p. 552-559. -
További szerzők:	Hársfalvi Jolán (1949-) (klinikai biokémikus) Pfliegler György (1949-) (belgyógyász, hematológus, labor szakorvos) Fauvel-Lafeve, Francoise Legrand, Chantal
Internet cím:	Intézményi repozitóriumban (DEA) tárolt változat
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001-es BibID:	BIBFORM014375
Első szerző:	Fodor Mariann (szemész)
Cím:	Transient visual loss triggered by scuba diving in a patient with a petrous epidermoid and combined thrombotic risk factors / Fodor Mariann, Facskó Andrea, Berényi Ervin, Sziklai István, Berta András, Pfliegler György
Dátum:	2007
Megjegyzések:	A 25-year-old woman who developed transient neurological abnormalities after scuba diving is reported. The subsequent day she experienced transient left-side monocular blindness. Arterial ocular occlusion in apparently healthy young women is unusual, and a search for the cause of this devastating vascular event is mandatory. Occlusion of the left branch retinal artery, total occlusion of the left internal carotid artery, and a petrous apex epidermoid were found, together with a shortened prothrombin time (INR: 0.73), a slightly elevated serum cholesterol level (6.1 mmol/l) and combined thrombophilia (elevated FVIIIC plus type 2 sticky platelet syndrome). This case underlines the complex mechanism of thromboembolic diseases, and the importance of the acquired trigger (in the present case scuba diving) in addition to the long-term anatomical and biochemical risk factors.
Tárgyszavak:	Orvostudományok Klinikai orvostudományok idegen nyelvű folyóiratközlemény külföldi lapban Transient monocular blindness Internal carotid artery occlusion Petrous epidermoid Platelet hyperaggregability Elevated FVIIIC Scuba diving
Megjelenés:	Pathophysiology of Haemostasis and Thrombosis. - 36 : 6 (2007), p. 311-314. -
További szerzők:	Facskó Andrea (1953-) (szemész) Berényi Ervin (1964-) (radiológus) Sziklai István (1954-) (fül-orr-gégész) Berta András (1955-) (szemész, gyermekszemész) Pfliegler György (1949-) (belgyógyász, hematológus, labor szakorvos)
Internet cím:	Szerző által megadott URL DOI Intézményi repozitóriumban (DEA) tárolt változat
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001-es BibID:	BIBFORM063574
035-os BibID:	(WoS)000374979000010 (Scopus)84959145645
Első szerző:	Gindele Réka (molekuláris biológus)
Cím:	Founder effect is responsible for the p.Leu131Phe heparin-binding-site antithrombin mutation common in Hungary : phenotype analysis in a large cohort / R. Gindele, Z. Olah, P. Ilonczai, M. Speker, A. Udvari, A. Selmeczi, G. Pfliegler, E. Marjan, B. Kovacs, Z. Boda, L. Muszbek, Z. Bereczky
Dátum:	2016
ISSN:	1538-7933
Megjegyzések:	Background: Antithrombin (AT) is a key regulatorof the coagulation. In type II deficiency, the heparinbinding-site defect (type II HBS) is considered to be relativelylow thrombosis risk. Objectives: Our aims were tosearch for SERPINC1 mutation(s) and to describe the clinicaland laboratory phenotype of a large number of ATBudapest3 (ATBp3, p.Leu131Phe) carriers and confirm thepresence of a founder effect. Patients/Methods: AT-deficientpatients were recruited and carriers of ATBp3,n = 102 (63 families) were selected. To investigate the foundereffect, eight intragenic single nucleotide polymorphisms,a 50-length dimorphism, and five microsatellitemarkers were detected. Clinical and laboratory data of thepatients were collected and analyzed. Results: In AT deficiency,16 different causative mutations were found, andthe great majority of patients were of type II HBS subtype.Most of them (n = 102/118, 86.5%) carried the ATBp3mutation. The ATBp3 mutant allele was associated withone single haplotype, while different haplotypes weredetected in the case of normal allele. The anti?factor Xa?based AT activity assay that we used could detect allATBp3 patients with high sensitivity in our cohort. ATBp3homozygosity (n = 26) was associated with thrombosis at ayoung age and conferred a high thrombotic risk. Half ofthe heterozygotes (n = 41/76, 53.9%) also had venous and/or arterial thrombosis, and pregnancy complications werealso recorded. Conclusion: In Hungary, the founder mutation,ATBp3, is the most common AT deficiency. Ourstudy is the first in which the clinical characterization ofATBp3 mutation was executed in a large population.
Tárgyszavak:	Orvostudományok Elméleti orvostudományok idegen nyelvű folyóiratközlemény külföldi lapban folyóiratcikk antithrombin III deficiency antithrombin III thrombophilia founder effect venous thromboembolism
Megjelenés:	Journal Of Thrombosis And Haemostasis. - 14 : 4 (2016), p. 704-715. -
További szerzők:	Oláh Zoltán Ilonczai Péter (1977-) (orvos, belgyógyász, haematológus szakorvos) Speker Marianna Udvari Á. Selmeczi Anna (1982-) (orvos) Pfliegler György (1949-) (belgyógyász, hematológus, labor szakorvos) Marján Erzsébet Kovács Bettina (1975-) (orvos) Boda Zoltán (1947-) (belgyógyász, haematologus, klinikai onkológus) Muszbek László (1942-) (haematológus, kutató orvos) Bereczky Zsuzsanna (1974-) (orvosi laboratóriumi diagnosztika szakorvos)
Pályázati támogatás:	PD101120 OTKA K116228 OTKA TÁMOP-4.2.2.A-11/1/KONV-2012-0045 TÁMOP Trombózis Kutató Központ Kutatócsoport 11003 MTA TKI227 MTA
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