INVESTIGADORES
AMARAL Maria Marta
congresos y reuniones científicas
Título:
Von Willebrand Factor And ADAMTS-13 In 23 Doberman Pinschers Dogs
Autor/es:
VERA, EZEQUIEL; KRIMER, ALEJANDRO; FARÍAS, CRISTINA; AMARAL MARÍA M; POWAZNIAK, YANINA; KEMPFER, ANA C; LAZZARI MARÍA A
Lugar:
Sydney
Reunión:
Congreso; XX CONGRESS. THE INTERNATIONAL SOCIETY ON THROMBOSIS AND HAEMOSTASIS AND 51st ANNUAL SSC METTING; 2005
Institución organizadora:
THE INTERNATIONAL SOCIETY ON THROMBOSIS AND HAEMOSTASIS
Resumen:
Objective: To study type-1 von Willebrand disease (VWD) phenotype and genotype and ADAMTS-13 values in 23 Doberman Pinschers dogs. Methods: Genotyping for von Willebrand factor (VWF). VWF:Ag by ELISA. Ultralarge VWF multimers (% ULVWF) by SDS- gel electrophoresis analyzed by densitometric scanning. ADAMTS-13 as previously described (Kempfer, BCF 2003 and Gerritsen, TH 1999). The VWF:Ag and ADAMTS-13 values were compared with normal human values (NHV) and expressed as mean ± SD (U/dL). Results: Genotype analysis: 4 dogs (17.4%) were homozygous (Ho1- Ho4) for vWD, 17 (73.9%) were heterozygous (He1-He17), and 2 (8.7%) did not have the defect (X1, X2). Some dogs are consanguineous: groups of three He, two He and He1 is stepbrother of X1. VWF:Ag results were: Ho=10±4, He=36±12 and X=53±15 (NHV=104±41, n=27). Ho showed statistical differences (p1. Dog Age (months) Sex VWF:Ag (U/dL) ULVWF (%) ADAMTS-13 (U/dL) X1 37 M 63 37 18 X2 - F 42 33 33 He1 7 F 36 25 30 Conclusion: there is a strong association between VWF:Ag and homozygous state only. Three out of 23 (13%) have some laboratory characteristics of thrombotic thrombocytopenic purpura (TTP). These findings suggest that these dogs can be an excellent tool to study the pathogenesis and treatment of TTP.