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Establishment of a new clinicopathological stratification method for focal segmental glomerulosclerosis

Research Project

Project/Area Number 18K07029
Research Category

Grant-in-Aid for Scientific Research (C)

Allocation TypeMulti-year Fund
Section一般
Review Section Basic Section 49020:Human pathology-related
Research InstitutionTokyo Women's Medical University

Principal Investigator

Ishizuka Kiyonobu  東京女子医科大学, 医学部, 助教 (80596560)

Co-Investigator(Kenkyū-buntansha) 張田 豊  東京大学, 医学部附属病院, 准教授 (10451866)
服部 元史  東京女子医科大学, 医学部, 教授 (50192274)
Project Period (FY) 2018-04-01 – 2022-03-31
Project Status Completed (Fiscal Year 2021)
Budget Amount *help
¥4,420,000 (Direct Cost: ¥3,400,000、Indirect Cost: ¥1,020,000)
Fiscal Year 2020: ¥1,560,000 (Direct Cost: ¥1,200,000、Indirect Cost: ¥360,000)
Fiscal Year 2019: ¥1,430,000 (Direct Cost: ¥1,100,000、Indirect Cost: ¥330,000)
Fiscal Year 2018: ¥1,430,000 (Direct Cost: ¥1,100,000、Indirect Cost: ¥330,000)
Keywords一次性巣状分節性糸球体硬化症 / 遺伝性巣状分節性糸球体硬化症 / 二次性巣状分節性糸球体硬化症 / 病因分類による層別化 / 腎移植後再発 / 移植腎機能予後 / 巣状分節性糸球体硬化症 / 糸球体上皮細胞足突起消失 / 腎移植再発 / 腎不全
Outline of Final Research Achievements

Focal segmental glomerulosclerosis (FSGS), an intractable nephrotic syndrome, is highly progressive to renal failure. FSGS may recur early after kidney transplantation, resulting in loss of transplanted kidney function. Post-transplant FSGS recurrence is limited to primary FSGS, whereas does not occur in such as hereditary FSGS, caused by genetic mutations of proteins comprising the glomeruli which is principal component of the filtration in the kidney, or secondary FSGS resulting maladaptive for renal hypoplasia. Therefore, the establishment of the differentiation of cause of FSGS is extremely important in order to identify appropriate therapeutic strategy before kidney transplantation. In this study, we combined and analyzed clinical, genetic and pathological information to accurately differentiate primary FSGS, at the same time extract FSGS patients, who should be performed genetic testing.

Academic Significance and Societal Importance of the Research Achievements

本研究は、小児末期腎不全の原因の第2位である巣状分節性糸球体硬化症(FSGS)について、その病因分類(一次性、遺伝性、二次性)によって層別化することで、発症後から腎移植までの適正な長期治療戦略の確立に寄与することが期待できる。一次性FSGSでは腎移植前に再発予防処置を講じることで腎移植後の原病再発の抑止と移植腎機能予後の改善が期待でき、一方で遺伝性FSGSや二次性FSGSでは不要な免疫抑制薬の投与を回避することが可能となる。このことで、限られた腎移植ドナー資源を、特に献腎移植について効率的に活用できることに貢献し、さらに医療経済負担の軽減に寄与するものと考えられる。

Report

(5 results)
  • 2021 Annual Research Report   Final Research Report ( PDF )
  • 2020 Research-status Report
  • 2019 Research-status Report
  • 2018 Research-status Report
  • Research Products

    (24 results)

All 2021 2020 2019 2018

All Journal Article (16 results) (of which Int'l Joint Research: 8 results,  Peer Reviewed: 13 results,  Open Access: 7 results) Presentation (8 results) (of which Int'l Joint Research: 2 results)

  • [Journal Article] Degree of foot process effacement in patients with genetic focal segmental glomerulosclerosis: a single-center analysis and review of the literature2021

    • Author(s)
      Ishizuka K, Miura K, Hashimoto T, Kaneko N, Harita Y, Yabuuchi T, Hisano M, Fujinaga S, Omori T, Yamaguchi Y, Hattori M.
    • Journal Title

      Sci Rep

      Volume: 11 Issue: 1 Pages: 12008-12008

    • DOI

      10.1038/s41598-021-91520-9

    • NAID

      120007183298

    • Related Report
      2021 Annual Research Report
    • Peer Reviewed / Open Access / Int'l Joint Research
  • [Journal Article] Amount and selectivity of proteinuria may predict the treatment response in post-transplant recurrence of focal segmental glomerulosclerosis: a single-center retrospective study2021

    • Author(s)
      Ban Hideki、Miura Kenichiro、Kaneko Naoto、Shirai Yoko、Yabuuchi Tomoo、Ishizuka Kiyonobu、Chikamoto Hiroko、Akioka Yuko、Shimizu Satoru、Ishida Hideki、Tanabe Kazunari、Hattori Motoshi
    • Journal Title

      Pediatric Nephrology

      Volume: - Issue: 8 Pages: 2433-2442

    • DOI

      10.1007/s00467-021-04951-x

    • Related Report
      2021 Annual Research Report 2020 Research-status Report
    • Peer Reviewed / Int'l Joint Research
  • [Journal Article] 巣状分節性糸球体硬化症における遺伝子解析と腎移植後再発に関する検討2021

    • Author(s)
      三浦健一郎 ,金子直人 ,安藤太郎 ,石塚喜世伸 ,伴 英樹 ,橋本多恵子 ,服部元史
    • Journal Title

      日本臨床腎移植学会雑誌

      Volume: 9 Pages: 236-242

    • NAID

      40022769471

    • Related Report
      2021 Annual Research Report
    • Peer Reviewed / Open Access
  • [Journal Article] Morphologic Analysis of Urinary Podocytes in Focal Segmental Glomerulosclerosis2020

    • Author(s)
      Shirai Yoko、Miura Kenichiro、Yokoyama Takashi、Horita Shigeru、Nakayama Hideki、Seino Hiroshi、Ando Taro、Shiratori Atsutoshi、Yabuuchi Tomoo、Kaneko Naoto、Ishiwa Sho、Ishizuka Kiyonobu、Hara Masanori、Hattori Motoshi
    • Journal Title

      Kidney360

      Volume: 2 Issue: 3 Pages: 477-486

    • DOI

      10.34067/kid.0005612020

    • NAID

      120007183296

    • Related Report
      2021 Annual Research Report 2020 Research-status Report
    • Peer Reviewed / Open Access / Int'l Joint Research
  • [Journal Article] Rapid progression to end-stage renal disease in a child with IgA-dominant infection-related glomerulonephritis associated with parvovirus B192020

    • Author(s)
      Yoko Shirai, Kenichiro Miura, Tomoo Yabuuchi, Takeshi Nagasawa, Kiyonobu Ishizuka, Kazuhiro Takahashi, Sekiko Taneda, Kazuho Honda, Yutaka Yamaguchi, Hitoshi Suzuki, Yusuke Suzuki, Motoshi Hattori
    • Journal Title

      CEN Case Rep

      Volume: 9 Issue: 4 Pages: 423

    • DOI

      10.1007/s13730-020-00501-w

    • URL

      https://pure.teikyo.jp/en/publications/199afde7-34cd-44d7-a659-45559d9855eb

    • Related Report
      2020 Research-status Report
    • Peer Reviewed / Open Access
  • [Journal Article] Renal hypoplasia can be the cause of membranous nephropathy-like lesions.2020

    • Author(s)
      Keiichi Takizawa, Kenichiro Miura, Naoto Kaneko, Tomoo Yabuuchi, Kiyonobu Ishizuka, Shoichiro Kanda, Yutaka Harita, Yuko Akioka, Shigeru Horita, Sekiko Taneda, Kazuho Honda, Motoshi Hattori
    • Journal Title

      Clin Exp Nephrol

      Volume: 24 Issue: 9 Pages: 813-820

    • DOI

      10.1007/s10157-020-01902-y

    • Related Report
      2020 Research-status Report
    • Peer Reviewed / Open Access
  • [Journal Article] Kidney function of Japanese children undergoing kidney transplant with preemptive therapy for cytomegalovirus infection2020

    • Author(s)
      Gotoh Yoshimitsu、Shishido Seiichiro、Hamasaki Yuko、Watarai Yoshihiko、Hattori Motoshi、Miura Kenichiro、Ishizuka Kiyonobu、Fujita Naoya、Saito Kazuhide、Nakagawa Yuki、Hotta Kiyohiko、Hataya Hiroshi、Hamada Riku、Sato Hiroyuki、Kitayama Hirotsugu、Ishikura Kenji、Honda Masataka、Uemura Osamu
    • Journal Title

      Transplant Infectious Disease

      Volume: Feb 28 Issue: 3

    • DOI

      10.1111/tid.13271

    • Related Report
      2020 Research-status Report 2019 Research-status Report
    • Peer Reviewed / Int'l Joint Research
  • [Journal Article] Individualized concept for the treatment of autosomal recessive polycystic kidney disease with end-stage renal disease.2020

    • Author(s)
      Miura K, Sato Y, Yabuuchi T, Kaneko N, Ishizuka K, Chikamoto H, Akioka Y, Nawashiro Y, Hisano M, Imamura H, Miyai T, Sakamoto S, Kasahara M, Fuchinoue S, Okumi M, Ishida H, Tanabe K, Hattori M.
    • Journal Title

      Pediatr Nephrol

      Volume: Mar 3 Issue: 3

    • DOI

      10.1111/petr.13690

    • Related Report
      2020 Research-status Report 2019 Research-status Report
    • Peer Reviewed / Int'l Joint Research
  • [Journal Article] Long-term outcome of renal transplantation in childhood-onset anti-neutrophil cytoplasmic antibody-associated vasculitis.2020

    • Author(s)
      Nagasawa T, Miura K, Kaneko N, Yabuuchi T, Ishizuka K, Chikamoto H, Akioka Y, Hisano M, Hattori M.
    • Journal Title

      Pediatr Nephrol.

      Volume: Mar;24(2) Issue: 2

    • DOI

      10.1111/petr.13656

    • Related Report
      2020 Research-status Report 2019 Research-status Report
    • Peer Reviewed / Int'l Joint Research
  • [Journal Article] Deletion in the cobalamin synthetase W domain-containing protein 1 gene is associated with congenital anomalies of the kidney and urinary tract.2020

    • Author(s)
      Kanda S, Ohmuraya M, Akagawa H, Horita S, Yoshida Y, Kaneko N, Sugawara N, Ishizuka K, Miura K, Harita Y, Yamamoto T, Oka A, Araki K, Furukawa T, Hattori M.
    • Journal Title

      J Am Soc Nephrol

      Volume: 31 Issue: 1 Pages: 139-147

    • DOI

      10.1681/asn.2019040398

    • Related Report
      2020 Research-status Report 2019 Research-status Report
    • Peer Reviewed / Open Access / Int'l Joint Research
  • [Journal Article] In Vivo Expression of NUP93 and Its Alteration by NUP93 Mutations Causing Focal Segmental Glomerulosclerosis.2019

    • Author(s)
      Hashimoto T, Harita Y, Takizawa K, Urae S, Ishizuka K, Miura K, Horita S, Ogino D, Tamiya G, Ishida H, Mitsui T, Hayasaka K, Hattori M.
    • Journal Title

      Kidney Int Rep

      Volume: 4(9) Issue: 9 Pages: 1312-1322

    • DOI

      10.1016/j.ekir.2019.05.1157

    • Related Report
      2019 Research-status Report
    • Peer Reviewed / Open Access / Int'l Joint Research
  • [Journal Article] Clinical characteristics of Campylobacter enteritis after pediatric renal transplantation: A retrospective analysis from single center2019

    • Author(s)
      Ban Hideki、Miura Kenichiro、Ishizuka Kiyonobu、Kaneko Naoto、Taniguchi Yohei、Nagasawa Takeshi、Shirai Yoko、Yabuuchi Tomoo、Takagi Yoko、Goto Aeko、Hattori Motoshi
    • Journal Title

      Transplant Infectious Disease

      Volume: 21 Issue: 2

    • DOI

      10.1111/tid.13040

    • Related Report
      2018 Research-status Report
    • Peer Reviewed
  • [Journal Article] エビデンスに基づくCKD診療ガイドライン20182018

    • Author(s)
      岡田浩一、服部元史、石塚喜世伸、他.
    • Journal Title

      日本腎臓学会誌

      Volume: 60 Pages: 1073-1193

    • Related Report
      2018 Research-status Report
    • Peer Reviewed
  • [Journal Article] 低・中分子生化学物質 シュウ酸、シスチン2018

    • Author(s)
      石塚喜世伸、服部元史
    • Journal Title

      腎と透析

      Volume: 84(増刊号) Pages: 64-65

    • Related Report
      2018 Research-status Report
  • [Journal Article] Outcomes of pediatric ABO-incompatible living kidney transplantations from 2002 to 2015: An analysis of the Japanese Kidney Transplant Registry.2018

    • Author(s)
      Hattori M, Mieno M, Shishido S, et al.
    • Journal Title

      Transplantation

      Volume: 102 Pages: 1934-1942

    • Related Report
      2018 Research-status Report
  • [Journal Article] 小児腎移植の成績と課題.2018

    • Author(s)
      服部元史
    • Journal Title

      腎と透析

      Volume: 85 Pages: 486-492

    • Related Report
      2018 Research-status Report
  • [Presentation] 移植腎長期生着に向けた小児腎移植の特徴と現状の課題2019

    • Author(s)
      石塚喜世伸、三浦健一郎、服部元史
    • Organizer
      日本泌尿器科学会
    • Related Report
      2019 Research-status Report
  • [Presentation] 維持期における腎移植例に対するエベロリムス併用の3年間の使用経験2019

    • Author(s)
      石塚喜世伸、三浦健一郎、安藤太郎、石和翔、 白鳥孝俊、飯田貴也、白井陽子、薮内智朗、 金子直人、服部元史
    • Organizer
      日本小児腎臓病学会
    • Related Report
      2019 Research-status Report
  • [Presentation] 一次性および二次性巣状分節性糸球体硬化症の臨床病理学的判別2018

    • Author(s)
      石塚喜世伸
    • Organizer
      第53回日本小児腎臓病学会学術集会
    • Related Report
      2018 Research-status Report
  • [Presentation] FSGS固有腎電顕所見による1次性/2次性判別の試み2018

    • Author(s)
      石塚喜世伸
    • Organizer
      第61回日本腎臓学会学術集会
    • Related Report
      2018 Research-status Report
  • [Presentation] FSGS移植後再発における超早期の足突起構成分子変化についての分子生物学的検討2018

    • Author(s)
      石塚喜世伸
    • Organizer
      第40回日本小児腎不全学会
    • Related Report
      2018 Research-status Report
  • [Presentation] 一次性および遺伝性FSGSの臨床病理学的判別2018

    • Author(s)
      石塚喜世伸
    • Organizer
      第40回日本小児腎不全学会
    • Related Report
      2018 Research-status Report
  • [Presentation] Hyperacute changes of podocytes in posttransplant recurrence of primary focal segmental glomerulosclerosis2018

    • Author(s)
      Kiyonobu Ishizuka
    • Organizer
      ISN Frontiers Meeting 2018
    • Related Report
      2018 Research-status Report
    • Int'l Joint Research
  • [Presentation] Clinicopathological discrimination of primary and genetic focal segmental glomerulosclerosis in children.2018

    • Author(s)
      Kiyonobu Ishizuka
    • Organizer
      Annual Meeting of American Society of Nephrology 2018
    • Related Report
      2018 Research-status Report
    • Int'l Joint Research

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Published: 2018-04-23   Modified: 2023-01-30  

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