Laboratory Diagnosis and Management of von Willebrand Disease in Turkey: Izmir Experience
Küçük Resim Yok
Tarih
2011
Yazarlar
Dergi Başlığı
Dergi ISSN
Cilt Başlığı
Yayıncı
Thieme Medical Publ Inc
Erişim Hakkı
info:eu-repo/semantics/closedAccess
Özet
von Willebrand disease (VWD) is caused by a deficiency or dysfunction of von Willebrand factor (VWF). The pathophysiology, classification, diagnosis, and management of VWD are relatively complex, but their understanding is important for proper diagnosis and management of patients with VWD. There are inherent difficulties in both the identification and classification of VWD because of clinical uncertainty and the limitations in the test processes and test panels typically used by laboratories. The most common test panel employed by laboratories, particularly in the geographic regions covered by the mutational studies, would comprise factor VIII coagulant (FVIII:C), VWF protein (antigen; VWF:Ag), and ristocetin cofactor (VWF:RCo). In our center, use of a desmopressin challenge with our core four-test panel (i.e., VWF:Ag, VWF:RCo, FVIII:C, and PFA-100) is expected to further assist laboratory diagnosis of VWD in Turkey. Molecular genetics is a rather new approach for Turkey, with gene analyses related to VWD being initiated in one center and the results used for confirmation of diagnosis in limited cases.
Açıklama
Anahtar Kelimeler
Diagnosis, Turkey, von Willebrand disease, DDAVP
Kaynak
Seminars in Thrombosis and Hemostasis
WoS Q Değeri
Q1
Scopus Q Değeri
N/A
Cilt
37
Sayı
5
