Involvement of the ADAMTS13-VWF axis in acute Kawasaki disease and effects of intravenous immunoglobulin.

• Material Type: 博士論文
• Title: Involvement of the ADAMTS13-VWF axis in acute Kawasaki disease and effects of intravenous immunoglobulin.
• Author/Editor: 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; Shima, Midori
• Author Heading: 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; Shima, Midori
• Publication, Distribution, etc.: Elsevier
• Publication Date: 2019-07
• Publication Date (W3CDTF): 2019-07
• Alternative Title: 川崎病急性期におけるa disintegrin and metalloproteinase with thrombospondin type 1 motif, member 13 (ADAMTS13) の動態
• Periodical title: Thrombosis research
• No. or year of volume/issue: 179
• Volume: 179
• Pages: 1-10
• ISSN (Periodical Title): ISSN : 0049-3848
• Degree grantor/type: 奈良県立医科大学
• Date Granted: 2020-09-27
• Dissertation Number: 甲第720号
• Degree Type: 博士（医学）
• Text Language Code: eng
• Subject Heading: Kawasaki disease; ADAMTS13; von Willebrand factor; Intravenous immunoglobulin; Isoelectro-focusing analysis
• Target Audience: 一般
• Note (General): type:Thesis; 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 < 0.05), low ADAMTS13:AC (60.3 ± 23.8%, p < 0.05) and high VWF:Ag/ADAMTS13:AC ratio (3.70 ± 2.12, p < 0.05) at Pre were seen compared to control plasmas. These parameters returned to normal levels time-dependently after IVIG treatment. Non-responders to IVIG demonstrated high VWF:Ag and low ADAMTS13:AC at Pre, and high VWF:Ag/ADAMTS13:AC ratio at 1 W compared to responders, but there were no significant differences in VWFM distribution between both groups. IEF analyses revealed the decreased free form of ADAMTS13 and increased complex form with ADAMTS13 and high-molecular-weight-VWFM at Pre in non-responders. A high VWF:Ag/ADAMTS13:AC ratio was associated with increased white blood cell counts, together with decreased serum albumin and sodium at Pre and 1 W. CONCLUSIONS: A high VWF:Ag/ADAMTS13:AC ratio in acute-KD persisted after primary treatment in non-responders, and unbalanced substrate-to-enzyme ratio appeared to associate with vascular endothelial damage. Analysis of existing mode of ADAMTS13 may help to clarify pathogenesis of IVIG resistance in acute-KD.; 博士（医学）・甲第720号・令和元年9月27日; Copyright © 2019 Elsevier Ltd. All rights reserved.; identifier:Thrombosis research Vol.179 p.1-10 (2019 Jul); identifier:00493848; identifier:http://ginmu.naramed-u.ac.jp/dspace/handle/10564/3665; identifier:Thrombosis research, 179: 1-10
• Access Restrictions: インターネット公開
• Data Provider (Database): 国立情報学研究所 : 学術機関リポジトリデータベース（IRDB）（機関リポジトリ）; https://irdb.nii.ac.jp
• Original Data Provider (Database): 奈良県立医科大学 : 奈良県立医科大学機関リポジトリ GINMU

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http://ginmu.naramed-u.ac.jp/dspace/bitstream/10564/3665/1/01%e7%94%b2720%e6%9c%ac%e6%96%87%e3%81%ae%e8%a6%81%e6%97%a8.pdf fulltext

http://ginmu.naramed-u.ac.jp/dspace/bitstream/10564/3665/2/02%e7%94%b2720%e5%af%a9%e6%9f%bb%e8%a6%81%e6%97%a8.pdf fulltext

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