Prevalence and spectrum of von Willebrand disease from western India.

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dc.contributor.authorTrasi, Suchetaen_US
dc.contributor.authorShetty, Shrimatien_US
dc.contributor.authorGhosh, Kanjakshaen_US
dc.contributor.authorMohanty, Dipikaen_US
dc.date.accessioned2005-05-07en_US
dc.date.accessioned2009-05-27T06:40:07Z
dc.date.available2005-05-07en_US
dc.date.available2009-05-27T06:40:07Z
dc.date.issued2005-05-07en_US
dc.description.abstractBACKGROUND 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 (VWF:Ag) 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 AND CONCLUSION: 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.en_US
dc.description.affiliationInstitute of Immunohaematology, Mumbai, India.en_US
dc.identifier.citationTrasi S, Shetty S, Ghosh K, Mohanty D. Prevalence and spectrum of von Willebrand disease from western India. Indian Journal of Medical Research. 2005 May; 121(5): 653-8en_US
dc.identifier.urihttps://imsear.searo.who.int/handle/123456789/17358
dc.language.isoengen_US
dc.source.urihttps://icmr.nic.in/ijmr/ijmr.htmen_US
dc.subject.meshAdulten_US
dc.subject.meshBlood Coagulation Testsen_US
dc.subject.meshChilden_US
dc.subject.meshCollagen --metabolismen_US
dc.subject.meshFemaleen_US
dc.subject.meshHumansen_US
dc.subject.meshIndia --epidemiologyen_US
dc.subject.meshMaleen_US
dc.subject.meshPartial Thromboplastin Timeen_US
dc.subject.meshPrevalenceen_US
dc.subject.meshProthrombin Timeen_US
dc.subject.meshRistocetinen_US
dc.subject.meshThrombin Timeen_US
dc.subject.meshvon Willebrand Disease --classificationen_US
dc.subject.meshvon Willebrand Factor --metabolismen_US
dc.titlePrevalence and spectrum of von Willebrand disease from western India.en_US
dc.typeComparative Studyen_US
dc.typeJournal Articleen_US
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