Complement activation associated with ADAMTS13 deficiency may contribute to the characteristic glomerular manifestations in Upshaw-Schulman syndrome.

• Material Type: 博士論文
• Title: Complement activation associated with ADAMTS13 deficiency may contribute to the characteristic glomerular manifestations in Upshaw-Schulman syndrome.
• Author/Editor: Itami, Hiroe; Hara, Shigeo; Matsumoto, Masanori; Imamura, Shin; Kanai, Rie; Nishiyama, Kei; Ishimura, Masataka; Ohga, Shouichi; Yoshida, Makiko; Tanaka, Ryojiro; Ogawa, Yoshiyuki; Asada, Yujiro; Sekita-Hatakeyama, Yoko; Hatakeyama, Kinta; Ohbayashi, Chiho
• Author Heading: Itami, Hiroe; Hara, Shigeo; Matsumoto, Masanori; Imamura, Shin; Kanai, Rie; Nishiyama, Kei; Ishimura, Masataka; Ohga, Shouichi; Yoshida, Makiko; Tanaka, Ryojiro; Ogawa, Yoshiyuki; Asada, Yujiro; Sekita-Hatakeyama, Yoko; Hatakeyama, Kinta; Ohbayashi, Chiho
• Publication, Distribution, etc.: Elsevier
• Publication Date: 2018-10
• Publication Date (W3CDTF): 2018-10
• Alternative Title: Upshaw-Schulman症候群の糸球体障害には補体活性とADAMTS13欠損が関連している可能性がある
• Periodical title: Thrombosis research
• No. or year of volume/issue: 170
• Volume: 170
• Pages: 148-155
• ISSN (Periodical Title): ISSN : 0049-3848
• Degree grantor/type: 奈良県立医科大学
• Date Granted: 2021-03-15
• Dissertation Number: 甲第792号
• Degree Type: 博士（医学）
• Text Language Code: eng
• Subject Heading: Upshaw-Schulman syndrome (USS); Renal biopsy; ADAMTS13; C4d
• Target Audience: 一般
• Note (General): type:Thesis; Introduction: Upshaw-Schulman syndrome (USS) is a congenital form of thrombotic thrombocytopenic purpura (TTP) associated with loss-of-function mutations in the ADAMTS13 gene, possibly leading to aberrant complement activation and vascular injury. However, USS is extremely rare, and there have been no systematic studies correlating histopathological severity with local ADAMTS13 expression and complement activation. Materials and methods: Here, we compared histopathological features, ADAMTS13 immunoreactivity, and immunoreactivity of complement proteins C4d and C5b-9 among renal biopsy tissues from five USS cases, ten acquired TTP cases, and eleven controls. Results: Pathological analysis revealed chronic glomerular sclerotic changes in the majority of USS cases (4 of 5), with minor glomerular pathology in the remaining case. In two of these four severe cases, more than half of the glomerular segmental sclerosis area was localized in the perihilar region. The average number of ADAMTS13-positive cells per glomerulus was significantly lower in USS cases than controls (p < 0.05). Conversely, C4d staining was significantly more prevalent in the glomerular capillary walls of USS cases than controls (p < 0.05), while C5b-9 staining did not differ significantly among groups. Conclusions: These findings suggest that the severity of glomerular injury in USS is associated with deficient ADAMTS13 expression and local complement activation, particularly in vascular regions with higher endothelial shear stress. We suggest that C4d immunostaining provides evidence for complement-mediated glomerular damage in USS.; 博士（医学）・甲第792号・令和3年3月15日; Copyright © 2018 Elsevier Ltd. All rights reserved.; identifier:Thrombosis research Vol.170 p.148-155 (2018 Oct); identifier:00493848; identifier:http://ginmu.naramed-u.ac.jp/dspace/handle/10564/3916; identifier:Thrombosis research, 170: 148-155
• Source: fulltext; http://ginmu.naramed-u.ac.jp/dspace/bitstream/10564/3916/1/01%e7%94%b2792%e6%9c%ac%e6%96%87%e3%81%ae%e8%a6%81%e6%97%a8.pdf; http://ginmu.naramed-u.ac.jp/dspace/bitstream/10564/3916/2/02%e7%94%b2792%e5%af%a9%e6%9f%bb%e8%a6%81%e6%97%a8.pdf; http://ginmu.naramed-u.ac.jp/dspace/bitstream/10564/3916/3/03%e7%94%b2792%e6%9c%ac%e6%96%87.pdf
• Access Restrictions: インターネット公開
• Related Material (DOI): 10.1016/j.thromres.2018.08.020
• Periodical Title (NCID): AA00863148; https://ci.nii.ac.jp/ncid/AA00863148
• 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