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Establishment of diagnostic criteria and disease severity in Crow-Fukase syndrome

Research Project

Project/Area Number 16K09692
Research Category

Grant-in-Aid for Scientific Research (C)

Allocation TypeMulti-year Fund
Section一般
Research Field Neurology
Research InstitutionChiba University

Principal Investigator

Misawa Sonoko  千葉大学, 医学部附属病院, 准教授 (30375753)

Project Period (FY) 2016-04-01 – 2019-03-31
Project Status Completed (Fiscal Year 2018)
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)
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.

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。

Report

(4 results)
  • 2018 Annual Research Report   Final Research Report ( PDF )
  • 2017 Research-status Report
  • 2016 Research-status Report
  • Research Products

    (13 results)

All 2019 2018 2017 2016

All Journal Article (10 results) (of which Int'l Joint Research: 2 results,  Peer Reviewed: 8 results,  Acknowledgement Compliant: 1 results,  Open Access: 2 results) Presentation (3 results) (of which Invited: 2 results)

  • [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
    • Journal Title

      Neurology

      Volume: in press

    • Related Report
      2018 Annual Research Report
  • [Journal Article] Proposal of new clinical diagnostic criteria for POEMS syndrome2018

    • Author(s)
      Suichi Tomoki、Misawa Sonoko、Sato Yasunori、Beppu Minako、Sakaida Emiko、Sekiguchi Yukari、Shibuya Kazumoto、Watanabe Keisuke、Amino Hiroshi、Kuwabara Satoshi
    • Journal Title

      Journal of Neurology, Neurosurgery & Psychiatry

      Volume: 90 Issue: 2 Pages: 133-137

    • DOI

      10.1136/jnnp-2018-318514

    • Related Report
      2018 Annual Research Report
    • Peer Reviewed
  • [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.
    • Journal Title

      Blood

      Volume: 印刷中

    • Related Report
      2017 Research-status Report
    • Peer Reviewed
  • [Journal Article] Reduction of Optic Disc Oedema by Bortezomib and Dexamethasone Followed by Autologous Peripheral Blood Stem Cell Transplantation in Patient with POEMS Syndrome2017

    • Author(s)
      Iwase T, Yokouchi H, Misawa S, Oshitari T, Baba T, Kuwabara S, Yamamoto S.
    • Journal Title

      Neuroophthalmology

      Volume: 42 Issue: 1 Pages: 25

    • DOI

      10.1080/01658107.2017.1318406

    • Related Report
      2017 Research-status Report
  • [Journal Article] Pain-related evoked potentials after intraepidermal electrical2017

    • Author(s)
      Omori S, Isose S, Misawa S, Watanabe K, Sekiguchi Y, Shibuya K, Beppu M, Amino
    • Journal Title

      Neuroscience Research

      Volume: 印刷中 Pages: 43-48

    • DOI

      10.1016/j.neures.2017.03.007

    • Related Report
      2016 Research-status Report
    • Peer Reviewed / Int'l Joint Research / Acknowledgement Compliant
  • [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.
    • Journal Title

      Biol Blood Marrow Transplant

      Volume: 23 Issue: 2 Pages: 361-363

    • DOI

      10.1016/j.bbmt.2016.10.024

    • Related Report
      2016 Research-status Report
    • Peer Reviewed
  • [Journal Article] Changes in subfoveal choroidal thickness and reduction of serum levels of vascular endothelial growth factor in patients with POEMS syndrome2017

    • Author(s)
      Yokouchi H, Baba T, Misawa S, Kitahashi M, Oshitari T, Kuwabara S, Yamamoto S.
    • Journal Title

      Br J Ophthalmol.

      Volume: 101 Issue: 6 Pages: 786

    • DOI

      10.1136/bjophthalmol-2016-309319

    • Related Report
      2016 Research-status Report
    • Peer Reviewed
  • [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.
    • Journal Title

      Lancet Neurol

      Volume: 15 Issue: 11 Pages: 1129-37

    • DOI

      10.1016/s1474-4422(16)30157-0

    • Related Report
      2016 Research-status Report
    • Peer Reviewed / Open Access / Int'l Joint Research
  • [Journal Article] Correlation between peripapillary retinal thickness and serum level of vascular endothelial growth factor in patients with POEMS syndrome2016

    • Author(s)
      Yokouchi H, Baba T, Misawa S, Sawai S, Kitahashi M, Oshitari T, Kuwabara S, Yamamoto S.
    • Journal Title

      Br J Ophthalmol.

      Volume: 100 Issue: 7 Pages: 897

    • DOI

      10.1136/bjophthalmol-2015-307068

    • Related Report
      2016 Research-status Report
    • Peer Reviewed
  • [Journal Article] POEMS syndrome and calciphylaxis: an unrecognized cause of abnormal small vessel calcification.2016

    • Author(s)
      Araki N, Misawa S, Shibuya K, Ota S, Oide T, Kawano A, Beppu M, Nakatani Y, Kuwabara S.
    • Journal Title

      Orphanet J Rare Dis.

      Volume: 11 Issue: 1 Pages: 35-35

    • DOI

      10.1186/s13023-016-0421-3

    • Related Report
      2016 Research-status Report
    • Peer Reviewed / Open Access
  • [Presentation] Current therapeutic strategies for POEMS syndrome.2016

    • Author(s)
      Misawa S
    • Organizer
      第57回 日本神経学会学術集会
    • Place of Presentation
      神戸国際会議場(兵庫県神戸市)
    • Year and Date
      2016-05-18
    • Related Report
      2016 Research-status Report
    • Invited
  • [Presentation] Late breaking symposium: Efficacy and safety of thalidomide in POEMS syndrome A multicentre, randomised, double-blind, placebo-controlled trial.2016

    • Author(s)
      Misawa S
    • Organizer
      第57回 日本神経学会学術集会
    • Place of Presentation
      神戸国際会議場(兵庫県神戸市)
    • Year and Date
      2016-05-18
    • Related Report
      2016 Research-status Report
    • Invited
  • [Presentation] POEMS症候群の新規診断基準の提唱2016

    • Author(s)
      三澤園子、別府美奈子、関口縁、岩井雄太、渡辺慶介、網野 寛、桑原 聡
    • Organizer
      第57回 日本神経学会学術集会
    • Place of Presentation
      神戸国際会議場(兵庫県神戸市)
    • Year and Date
      2016-05-18
    • Related Report
      2016 Research-status Report

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Published: 2016-04-21   Modified: 2020-03-30  

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