1. Involvement of the ADAMTS13-VWF axis in acute Kawasaki disease and effects of intravenous immunoglobulin
- Author
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Tsujii, Nobuyuki, Nogami, Keiji, Matsumoto, Masanori, Yoshizawa, Hiroyuki, Takase, Toshio, Tanaka, Ichiro, Sakai, Toshiyuki, Fukuda, Kazuyoshi, Hayakawa, Masaki, Sakai, Kazuya, Isonishi, Ayami, Matsuura, Kayoko, Fujimura, Yoshihiro, and Shima, Midori
- Subjects
Kawasaki disease ,Isoelectro-focusing analysis ,hemic and lymphatic diseases ,von Willebrand factor ,ADAMTS13 ,Intravenous immunoglobulin - Abstract
INTRODUCTION: ADAMTS13 modulates shear-dependent platelet thrombus formation (PTF) by limited proteolysis of von Willebrand factor (VWF). A high-plasma-ratio of VWF antigen to ADAMTS13 activity (VWF:Ag/ADAMTS13:AC) promotes PTF and aggravates shear-induced inflammation mediated by VWF. A role of ADAMTS13 in Kawasaki disease (KD) remains unknown, however. We investigated the involvement of ADAMTS13-VWF axis in the acute-phase of KD (acute-KD). METHODS: VWF:Ag and ADAMTS13:AC in 77 KD infants were measured at three time-points; immediately before (Pre), one-week (1 W) and one-month (1 M) after intravenous-immunoglobulin (IVIG) treatment. VWF multimer (VWFM) distribution and ADAMTS13-isoelectrofocusing (IEF) patterns were compared between the responders and non-responders to IVIG. RESULTS: A high VWF:Ag (195.7 ± 85.6%, p, 博士(医学)・甲第720号・令和元年9月27日, Copyright © 2019 Elsevier Ltd. All rights reserved.
- Published
- 2019