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

• 資料種別: 博士論文
• タイトル: Involvement of the ADAMTS13-VWF axis in acute Kawasaki disease and effects of intravenous immunoglobulin.
• 著者・編者: 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
• 著者標目: 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
• 出版事項: Elsevier
• 出版年月日等: 2019-07
• 出版年（W3CDTF）: 2019-07
• 並列タイトル等: 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
• タイトル（掲載誌）: Thrombosis research
• 巻号年月日等（掲載誌）: 179
• 掲載巻: 179
• 掲載ページ: 1-10
• ISSN（掲載誌）: ISSN : 0049-3848
• 授与機関名: 奈良県立医科大学
• 授与年月日: 2020-09-27
• 報告番号: 甲第720号
• 学位: 博士（医学）
• 本文の言語コード: eng
• 件名標目: Kawasaki disease; ADAMTS13; von Willebrand factor; Intravenous immunoglobulin; Isoelectro-focusing analysis
• 対象利用者: 一般
• 一般注記: 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
• オンライン閲覧公開範囲: インターネット公開
• 連携機関・データベース: 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
• 提供元機関・データベース: 奈良県立医科大学 : 奈良県立医科大学機関リポジトリ GINMU

目次

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

http://ginmu.naramed-u.ac.jp/dspace/bitstream/10564/3665/3/03%e7%94%b2720%e6%9c%ac%e6%96%87.pdf fulltext