Subtypes of von Willebrand Disease Based on vWF Multimer Analysis in Korea / 대한소아혈액종양학회지
Korean Journal of Pediatric Hematology-Oncology
;
: 42-49, 2000.
Artigo
em Coreano
| WPRIM
| ID: wpr-8482
ABSTRACT
PURPOSE:
von Willebrand disease is a common inherited bleeding disorder characterized by high degree of variable clinical presentation due to either quantitative or qualitative defects in von Willebrand factor. Its incidence in Korea is not well studied while that in western countries is extensively studied.METHODS:
We classified 16 cases of vWD from 14 unrelated families based on vWF antigen, ristocetin cofactor activity, factor VIII activity and vWF multimeric patterns analysed by agarose gel electrophoresis, according to a revised classification by ISTH.RESULTS:
There were 12 cases (75%) of type 1 vWD or 2M/2N with normal multimeric pattern, 3 cases (18.75%) of type 2 vWD lacking high molecular weight multimers and only 1 case of type 3 vWD with no multimers.CONCLUSION:
The proportion of each vWD subtype in Korea is similar to that in western countries, however, accurate diagnosis based on ristocetin induced platelet aggregation test, factor VIII binding assay and molecular genetic diagnosis seems to be necessary for a more complete classification of vWD.
Texto completo:
DisponíveL
Índice:
WPRIM (Pacífico Ocidental)
Assunto principal:
Doenças de von Willebrand
/
Fator VIII
/
Fator de von Willebrand
/
Ristocetina
/
Agregação Plaquetária
/
Incidência
/
Classificação
/
Diagnóstico
/
Eletroforese em Gel de Ágar
/
Doença de von Willebrand Tipo 3
Tipo de estudo:
Estudo diagnóstico
/
Estudo de incidência
/
Estudo prognóstico
/
Pesquisa qualitativa
Limite:
Humanos
País/Região como assunto:
Ásia
Idioma:
Coreano
Revista:
Korean Journal of Pediatric Hematology-Oncology
Ano de publicação:
2000
Tipo de documento:
Artigo
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