Establishment of a new clinicopathological stratification method for focal segmental glomerulosclerosis
Project/Area Number |
18K07029
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Research Category |
Grant-in-Aid for Scientific Research (C)
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Allocation Type | Multi-year Fund |
Section | 一般 |
Review Section |
Basic Section 49020:Human pathology-related
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Research Institution | Tokyo Women's Medical University |
Principal Investigator |
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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)
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Project Period (FY) |
2018-04-01 – 2022-03-31
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Project Status |
Completed (Fiscal Year 2021)
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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)
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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.
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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。
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Report
(5 results)
Research Products
(24 results)
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[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.
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Journal Title
Sci Rep
Volume: 11
Issue: 1
Pages: 12008-12008
DOI
NAID
Related Report
Peer Reviewed / Open Access / Int'l Joint Research
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[Journal Article] Rapid progression to end-stage renal disease in a child with IgA-dominant infection-related glomerulonephritis associated with parvovirus B19.2020
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
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Journal Title
CEN Case Rep
Volume: 9
Issue: 4
Pages: 423-430
DOI
Related Report
Peer Reviewed / Open Access
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[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
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Journal Title
Clin Exp Nephrol
Volume: 24
Issue: 9
Pages: 813-820
DOI
Related Report
Peer Reviewed / Open Access
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[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.
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Journal Title
Pediatr Nephrol
Volume: Mar 3
Issue: 3
DOI
Related Report
Peer Reviewed / Int'l Joint Research
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[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.
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Journal Title
J Am Soc Nephrol
Volume: 31
Issue: 1
Pages: 139-147
DOI
Related Report
Peer Reviewed / Open Access / Int'l Joint Research
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[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.
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Journal Title
Kidney Int Rep
Volume: 4(9)
Issue: 9
Pages: 1312-1322
DOI
Related Report
Peer Reviewed / Open Access / Int'l Joint Research
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