Prevalence and spectrum of von Willebrand disease from western India.
Artigo
em Inglês
| IMSEAR
| ID: sea-17358
ABSTRACT
BACKGROUND AND OBJECTIVE:
von Willebrand disease (VWD) is one of the most common inherited bleeding disorders in the west. Limited studies from India showed a prevalence of approximately 10 per cent of VWD among the cases with hereditary bleeding disorders. VWD remains an underdiagnosed entity in India. The prevalence of different subtypes of VWD is also not known which is essential for a proper management of these cases. The present study was thus undertaken to know the prevalence of VWD and its various subtypes in the western part of our country.METHODS:
A total of 796 consecutive patients presented with various bleeding manifestations were analysed. The initial screening and confirmation tests for the diagnosis of VWD included bleeding time (BT), screening coagulation tests i.e., prothrombin time (PT), activated partial thromboplastin time (APTT), thrombin time (TT), factor VIII C assay, ristocetin-induced platelet aggregation (RIPA) and VWF antigen (VWFAg) estimations. VWF multimer analysis, ristocetin cofactor activity (RCOF), VWF collagen binding assay (VWF CBA), factor VIII VWF binding assay were also done to classify and subtype these cases.RESULTS:
The patients were subtyped as per the International Society of Thrombosis and Haemostasis (ISTH) criteria. Of the 796 patients screened, 58 were diagnosed as VWD. Of the 15 families with a positive family history of bleeding, 26 additional cases were diagnosed as VWD. Majority of the patients were type 3 (59.5%) with severe clinical manifestations, about 18 per cent of type 1 VWD patients were detected in this group while the prevalence of the qualitative variants of VWD i.e., type 2 VWD was found to be 19 per cent and the prevalence of various subtypes were type 2A (9.52%), type 2B (4.76%), type 2M (1.2%), type 2N (3.6%). INTERPRETATION ANDCONCLUSION:
The high prevalence of type 3 and a low prevalence of type 1 VWD which is in contrast to the western reports, suggests the low awareness of the disease as also the underdiagnosis of the mild cases in our country.
Texto completo:
DisponíveL
Índice:
IMSEAR (Sudeste Asiático)
Assunto principal:
Tempo de Tromboplastina Parcial
/
Tempo de Protrombina
/
Tempo de Trombina
/
Doenças de von Willebrand
/
Testes de Coagulação Sanguínea
/
Feminino
/
Humanos
/
Masculino
/
Fator de von Willebrand
/
Ristocetina
Tipo de estudo:
Estudo de prevalência
/
Pesquisa qualitativa
/
Fatores de risco
País/Região como assunto:
Ásia
Idioma:
Inglês
Ano de publicação:
2005
Tipo de documento:
Artigo
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