TY - JOUR
T1 - von Willebrand Disease
T2 - Diagnostic Strategies and Treatment Options
AU - Ng, Christopher J.
AU - Di Paola, Jorge
N1 - Publisher Copyright:
© 2018 Elsevier Inc.
PY - 2018/6
Y1 - 2018/6
N2 - von Willebrand disease (VWD) is one of the most common inherited bleeding disorders. Since its first description in 1926, the diagnosis and management of VWD has significantly improved due to increasing scientific knowledge of the genetics and biology of von Willebrand factor (VWF). This article reviews the molecular structure and function of VWF as well as the clinical symptoms, laboratory-based diagnostic workup, and classification schema for VWD. It highlights current treatment options and state-of-the art research in VWF and VWD.
AB - von Willebrand disease (VWD) is one of the most common inherited bleeding disorders. Since its first description in 1926, the diagnosis and management of VWD has significantly improved due to increasing scientific knowledge of the genetics and biology of von Willebrand factor (VWF). This article reviews the molecular structure and function of VWF as well as the clinical symptoms, laboratory-based diagnostic workup, and classification schema for VWD. It highlights current treatment options and state-of-the art research in VWF and VWD.
KW - Mucocutaneous bleeding
KW - von Willebrand disease
KW - von Willebrand factor
UR - http://www.scopus.com/inward/record.url?scp=85047439508&partnerID=8YFLogxK
U2 - 10.1016/j.pcl.2018.02.004
DO - 10.1016/j.pcl.2018.02.004
M3 - Review article
C2 - 29803281
AN - SCOPUS:85047439508
SN - 0031-3955
VL - 65
SP - 527
EP - 541
JO - Pediatric Clinics of North America
JF - Pediatric Clinics of North America
IS - 3
ER -