Establishment of diagnostic criteria and disease severity in Crow-Fukase syndrome
Project/Area Number |
16K09692
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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 |
Research Field |
Neurology
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Research Institution | Chiba University |
Principal Investigator |
Misawa Sonoko 千葉大学, 医学部附属病院, 准教授 (30375753)
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Project Period (FY) |
2016-04-01 – 2019-03-31
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Project Status |
Completed (Fiscal Year 2018)
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Budget Amount *help |
¥4,550,000 (Direct Cost: ¥3,500,000、Indirect Cost: ¥1,050,000)
Fiscal Year 2018: ¥2,210,000 (Direct Cost: ¥1,700,000、Indirect Cost: ¥510,000)
Fiscal Year 2017: ¥1,170,000 (Direct Cost: ¥900,000、Indirect Cost: ¥270,000)
Fiscal Year 2016: ¥1,170,000 (Direct Cost: ¥900,000、Indirect Cost: ¥270,000)
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Keywords | クロウ-深瀬症候群 / POEMS症候群 / 血管内皮増殖因子 / 形質細胞 / クロウ・深瀬症候群 / 診断基準 / 全国調査 / plasma cell dyscrasia / 内科 |
Outline of Final Research Achievements |
Crow-Fukase (POEMS) syndrome is a rare cause of demyelinating neuropathy based on plasma cell dyscrasia. The prognosis of Crow-Fukase syndrome has been improved by application of treatment for myeloma. However, appropriate diagnostic criteria and disease severity are essential to establish efficient therapeutic strategies. Therefore, we have suggested a new diagnostic criteria, which was statistically validated and had high diagnostic accuracy. In addition, we analyzed prevalence rate and current treatment status, and prognosis of Crow-Fukase syndrome in Japan based on the national survey conducted in 2015. We are now considering disease severity based on our database, the national survey data, and national registry database.
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Academic Significance and Societal Importance of the Research Achievements |
クロウ-深瀬(POEMS)症候群は全国の患者数が400例未満の超稀少疾患である。患者数が少ないが故に、保険適用のある治療がなく、治療方針も標準化されていない。本研究により、現時点の我が国におけるクロウ・深瀬症候群の罹病者数、疾患プロファイル、治療の実態が明確になった。本症候群は骨髄腫の治療が奏功する可能性が高く、診断基準の整備、治療の標準化により、予後の改善が大いに期待される。本研究成果により、クロウ・深瀬症候群の今後の予後改善の基盤が築かれた。
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Report
(4 results)
Research Products
(13 results)
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[Journal Article] Prevalence, clinical profiles, and prognosis of POEMS syndrome in Japanese nationwide survey2019
Author(s)
Tomoki Suichi, Sonoko Misawa, Minako Beppu, Sho Takahashi, Yukari Sekiguchi, Kazumoto Shibuya, Hiroshi Amino, Atsuko Tsuneyama, Yo-ichi Suzuki, Keigo Nakamura, Yasunori Sato, and Satoshi Kuwabara
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Journal Title
Neurology
Volume: in press
Related Report
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[Journal Article] Long-Term Evaluation of Physical Improvement and Survival of Autologous Stem Cell Transplantation in POEMS Syndrome.2018
Author(s)
Ohwada C, Sakaida E, Kawajiri-Manako C, Nagao Y, Oshima-Hasegawa N, Togasaki E, Muto T, Tsukamoto S, Mitsukawa S, Takeda Y, Mimura N, Takeuchi M, Shimizu N, Misawa S, Iseki T, Kuwabara S, Nakaseko C.
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Journal Title
Related Report
Peer Reviewed
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[Journal Article] Safety and efficacy of granulocyte-colony stimulating factor monotherapy for peripheral blood stem cell collection in POEMS Syndrome.2017
Author(s)
Muto T, Ohwada C, Takaishi K, Isshiki Y, Nagao Y, Hasegawa N, Kawajiri-Manako C, Togasaki E, Shimizu R, Tsukamoto S, Sakai S, Takeda Y, Mimura N, Takeuchi M, Sakaida E, Misawa S, Shimizu N, Iseki T, Kuwabara S, Nakaseko C.
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Journal Title
Biol Blood Marrow Transplant
Volume: 23
Issue: 2
Pages: 361-363
DOI
Related Report
Peer Reviewed
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[Journal Article] Safety and efficacy of thalidomide in patients with POEMS syndrome: a multicentre, randomised, double-blind, placebo-controlled trial.2016
Author(s)
Misawa S, Sato Y, Katayama K, Nagashima K, Aoyagi R, Sekiguchi Y, Sobue G, Koike H, Yabe I, Sasaki H, Watanabe O, Takashima H, Nishizawa M, Kawachi I, Kusunoki S, Mitsui Y, Kikuchi S, Nakashima I, et al; Japanese POEMS Syndrome for Thalidomide (J-POST) Trial Study Group.
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Journal Title
Lancet Neurol
Volume: 15
Issue: 11
Pages: 1129-37
DOI
Related Report
Peer Reviewed / Open Access / Int'l Joint Research
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