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Please use this identifier to cite or link to this item: http://hdl.handle.net/10564/3916

Title: Complement activation associated with ADAMTS13 deficiency may contribute to the characteristic glomerular manifestations in Upshaw-Schulman syndrome.
Other Titles: 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
Authors: 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
Keywords: Upshaw-Schulman syndrome (USS)
Renal biopsy
ADAMTS13
C4d
Issue Date: Oct-2018
Publisher: Elsevier
Citation: Thrombosis research Vol.170 p.148-155 (2018 Oct)
Abstract: 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.
Description: 博士(医学)・甲第792号・令和3年3月15日
Copyright © 2018 Elsevier Ltd. All rights reserved.
URI: http://hdl.handle.net/10564/3916
ISSN: 00493848
Academic Degrees and number: 24601A792
Degree-granting date: 2021-03-15
Degree name: 博士(医学)
Degree-granting institutions: 奈良県立医科大学
Appears in Collections:2020年度

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