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Diagnosis and treatment of periodic fever, aphthous stomatitis, pharyngitis, cervical adenitis syndrome

Research Project

Project/Area Number 22591155
Research Category

Grant-in-Aid for Scientific Research (C)

Allocation TypeSingle-year Grants
Section一般
Research Field Pediatrics
Research InstitutionShinshu University

Principal Investigator

AGEMATSU Kazunaga  信州大学, 医学部, 准教授 (60262721)

Co-Investigator(Kenkyū-buntansha) KOBAYASHI Norimoto  信州大学, 医学部, 助教 (00362129)
Project Period (FY) 2010 – 2012
Project Status Completed (Fiscal Year 2012)
Budget Amount *help
¥4,420,000 (Direct Cost: ¥3,400,000、Indirect Cost: ¥1,020,000)
Fiscal Year 2012: ¥910,000 (Direct Cost: ¥700,000、Indirect Cost: ¥210,000)
Fiscal Year 2011: ¥1,300,000 (Direct Cost: ¥1,000,000、Indirect Cost: ¥300,000)
Fiscal Year 2010: ¥2,210,000 (Direct Cost: ¥1,700,000、Indirect Cost: ¥510,000)
Keywords周期性発熱 / 自己炎症疾患 / CD64 / autoinflammatory disease / 炎症 / 発熱 / 治療
Research Abstract

The syndrome of periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) is the most common periodic fever disease in children. We examined clinical findings and the utility of CD64, namely Fcγ-RI expression on polymorphonuclear neutrophils and monocytes in diagnosing patients with PFAPA, Although CD64 was expressed at a similar level in the attack-free period of PFAPA and controls, CD64 expressions on both neutrophils and monocytes was dramatically increased during attacks. In addition, serum IFN-γ also increased in some PFAPA patients during flares, suggesting the involvement of T-cell activation. Our findings demonstrate that remarkable CD64 expression during PFAPA flares serves as a potential biomarker for the diagnosis.

Report

(4 results)
  • 2012 Annual Research Report   Final Research Report ( PDF )
  • 2011 Annual Research Report
  • 2010 Annual Research Report
  • Research Products

    (41 results)

All 2013 2012 2011 2010 Other

All Journal Article (33 results) (of which Peer Reviewed: 22 results) Presentation (3 results) (of which Invited: 1 results) Book (3 results) Remarks (2 results)

  • [Journal Article] The contribution of SAA1 polymorphisms to Familial Mediterranean fever susceptibility in the Japanese population.2013

    • Author(s)
      Migita K
    • Journal Title

      PLoS One.

      Volume: 8 Pages: 55227-55227

    • Related Report
      2012 Annual Research Report
    • Peer Reviewed
  • [Journal Article] Rapid Detection of Intracellular p47phox and p67phox by Flow Cytometry; Useful Screening Tests for Chronic Granulomatous Disease.2013

    • Author(s)
      Wada T
    • Journal Title

      J Clin Immunol.

      Volume: 33 Issue: 4 Pages: 857-864

    • DOI

      10.1007/s10875-012-9859-9

    • Related Report
      2012 Annual Research Report
    • Peer Reviewed
  • [Journal Article] Clonal expansion of Epstein-Barr virus (EBV)-infected γδ T cells in patients with chronic active EBV disease and hydroa vacciniforme-like eruptions.2012

    • Author(s)
      Wada T, Toga A, Sakakibara Y, Toma T, Shigemura T, Agematsu K, Yachie A.
    • Journal Title

      Int J Hematol

      Volume: 96(4) Issue: 4 Pages: 443-9

    • DOI

      10.1007/s12185-012-1156-0

    • NAID

      10031126735

    • Related Report
      2012 Annual Research Report 2012 Final Research Report
    • Peer Reviewed
  • [Journal Article] Clinical relevance of MEFV gene mutations in Japanese patients with unexplained fever.2012

    • Author(s)
      Migita K, Ida H, Moriuchi H, Agematsu K.
    • Journal Title

      J Rheumatol.

      Volume: 39 Issue: 4 Pages: 875-877

    • DOI

      10.3899/jrheum.110700

    • Related Report
      2012 Annual Research Report 2012 Final Research Report
    • Peer Reviewed
  • [Journal Article] Clinical relevance of MEFV gene mutations in Japanese patients with unexplained fever.2012

    • Author(s)
      Migita K
    • Journal Title

      J Rheumatol.

      Volume: 39 Pages: 875-877

    • Related Report
      2012 Annual Research Report
    • Peer Reviewed
  • [Journal Article] 遷延する関節炎がみられた家族性地中海熱の1女性例2012

    • Author(s)
      黒岩京子
    • Journal Title

      小児リウマチ

      Volume: 3 Pages: 77-80

    • Related Report
      2012 Annual Research Report
  • [Journal Article] 家族性地中海熱MEFV exon 3 variantsの1例2012

    • Author(s)
      福田 稔
    • Journal Title

      小児リウマチ

      Volume: 3 Pages: 23-26

    • Related Report
      2012 Annual Research Report
  • [Journal Article] 【小児疾患の診断治療基準(第4版)】 (第2部)疾患 生体防御・免疫不全 重症複合免疫不全症(解説/特集)2012

    • Author(s)
      上松 一永
    • Journal Title

      小児内科

      Volume: 44 Pages: 224-225

    • Related Report
      2012 Annual Research Report
  • [Journal Article] Muckle-Wells症候群の1例2012

    • Author(s)
      大橋 敦子
    • Journal Title

      日本小児皮膚科学会雑誌

      Volume: 31 Pages: 107-111

    • Related Report
      2012 Annual Research Report
  • [Journal Article] Defective IL-10 signaling in hyper-IgE syndrome results in impaired generation of tolerogenic dendritic cells and induced regulatory T cells2011

    • Author(s)
      Saito M,…, Takada H, et al
    • Journal Title

      J Exp Med

      Volume: 208 Issue: 2 Pages: 235-249

    • DOI

      10.1084/jem.20100799

    • Related Report
      2012 Final Research Report
    • Peer Reviewed
  • [Journal Article] Quantification of κ -deleting recombination excision circles in Guthrie cards for the identification of early B-cell maturation defects.2011

    • Author(s)
      Nakagawa N, Imai K, Kanegane H, Sato H, Yamada M, Kondoh K, Okada S, Kobayashi M, Agematsu K, Takada H, Mitsuiki N, Oshima K, Ohara O, Suri D, Rawat A, Singh S, Pan-Hammarstrom Q, Hammarstrom L, Reichenbach J, Seger R, Ariga T, Hara T, Miyawaki T, Nonoyama S
    • Journal Title

      J Allergy Clin Immunol.

      Volume: 28 Issue: 1 Pages: 223-225

    • DOI

      10.1016/j.jaci.2011.02.022

    • Related Report
      2012 Final Research Report
    • Peer Reviewed
  • [Journal Article] Expression of CD64 on polymorphonuclear neutrophils in patients with familial Mediterranean fever2011

    • Author(s)
      Migita K, Agematsu K, Yamazaki K, Suzuki A, Yazaki M, Jiuchi Y, Miyashita T, Izumi Y, Koga T, Kawakami A, Eguchi K.
    • Journal Title

      Clin Exp Immunol.

      Volume: 164 Issue: 3 Pages: 365-72

    • DOI

      10.1111/j.1365-2249.2011.04380.x

    • Related Report
      2012 Final Research Report
    • Peer Reviewed
  • [Journal Article] A case of Behcet's disease associated with myelodysplastic syndrome involving trisomy 8 and a gain-of-function mutation in SHP-2.2011

    • Author(s)
      Shigemura T, Sakashita K
    • Journal Title

      Rheumatology (Oxford)

      Volume: 50 Issue: 7 Pages: 1342-4

    • DOI

      10.1093/rheumatology/ker137

    • Related Report
      2012 Final Research Report
    • Peer Reviewed
  • [Journal Article] Monitoring serum IL-18 levels is useful for treatment of a patient with systemic juvenile idiopathic arthritis complicated by macrophage activation syndrome.2011

    • Author(s)
      Shigemura T, Yamazaki T, Hara Y, Ou JN, Stevens AM, Ochs HD, Koike K, Agematsu K.
    • Journal Title

      Pediatr Rheumatol Online J.

      Volume: 13;9(1) Issue: 1 Pages: 1-1

    • DOI

      10.1186/1546-0096-9-15

    • NAID

      120007110556

    • Related Report
      2012 Final Research Report
    • Peer Reviewed
  • [Journal Article] 家族性地中海熱の臨床2011

    • Author(s)
      右田清志、上松一永
    • Journal Title

      日本臨床免疫学会誌

      Volume: 34 Pages: 335-360

    • NAID

      10029897928

    • Related Report
      2012 Final Research Report 2011 Annual Research Report
  • [Journal Article] 自己炎症症候群の多様性 家族性地中海熱わが国と欧米の差異2011

    • Author(s)
      上松 一永, 角田 裕幸, 高本 雅哉
    • Journal Title

      炎症と免疫

    • Related Report
      2012 Final Research Report
  • [Journal Article] Monitoring serum IL-18 levels is useful for treatment of a patient with systemic juvenile idiopathic arthritis complicated by macrophage activation syndrome2011

    • Author(s)
      Shigemura T, Yamazaki T, Hara Y, Ou JN, Stevens AM, OchS HD, Koike K, Agematsu K
    • Journal Title

      Pediatr Rheumatol

      Volume: 13 Pages: 9-12

    • NAID

      120007110556

    • Related Report
      2011 Annual Research Report
    • Peer Reviewed
  • [Journal Article] A case of Behcet's disease associated with myelodysplastic syndrome involving trisomy 8 and a gain-of-function mutation in SHP-22011

    • Author(s)
      Shigemura T, Agematsu K, Yamazaki T, Sakashita K, Nakayama Y, Higuchi Y, Matsuda K, Koike K
    • Journal Title

      Rheumatology

      Volume: 50 Pages: 1342-1344

    • Related Report
      2011 Annual Research Report
    • Peer Reviewed
  • [Journal Article] Expression of CD64 on polymorphonuclear neutrophils in patients with familial Mediterranean fever2011

    • Author(s)
      Migita K, Agematsu K, Yamazaki K, Suzuki A, Yazaki M, Jiuchi Y, Miyashita T, Izumi Y, Koga T, Kawakami A, Eguchi K
    • Journal Title

      Clin Exp Immunol

      Volume: 164 Pages: 365-372

    • Related Report
      2011 Annual Research Report
    • Peer Reviewed
  • [Journal Article] Quantification of κ-deleting recombination excision circles in Guthrie cards for the identification of early B-cell maturation defects2011

    • Author(s)
      Nakagawa N, Imai K, Kanegane H, Sato H, Yamada M, Kondoh K, Okada S, Kobayashi M, Agematsu K, Takada H, Mitsuiki N, Oshima K, Ohara O, Suri D, Rawat A, Singh S, Pan-Hammarstrom Q, Hammarstrom L, Reichenbach J, Seger R, Ariga T, Hara T, Miyawaki T, Nonoyama S.
    • Journal Title

      J Allergy Clin Immunol

      Volume: 28 Pages: 223-225

    • Related Report
      2011 Annual Research Report
    • Peer Reviewed
  • [Journal Article] Expression of CD64 on polymorphonuclear neutrophils in patients with familial Mediterranean fever2011

    • Author(s)
      Saito M, Nagasawa M, Takada H, Hara T, Tsuchiya S, Agematsu K, Yamada M, Kawamura N, Ariga T, Tsuge I, Nonoyama S, Karasuyama H, Minegishi Y
    • Journal Title

      J Exp Med

      Volume: 208 Pages: 235-249

    • Related Report
      2011 Annual Research Report
    • Peer Reviewed
  • [Journal Article] A case of Familial Mediterranean fever associated with compound heterozygosity for the pyrin variant L110P-E148Q/M680I in Japan.2010

    • Author(s)
      Koichi Oshima, Kazuko Yamazaki, Yoichi Nakajima, Akari Kobayashi, Tomochika Kato, Osamu Ohara, Kazunaga Agematsu.
    • Journal Title

      Modern Rheumatology

      Volume: 20 Issue: 2 Pages: 193-5

    • DOI

      10.1007/s10165-009-0249-y

    • NAID

      10027169046

    • Related Report
      2012 Final Research Report
    • Peer Reviewed
  • [Journal Article] Potentiation of TLR9 responses for human naive B-cell growth through RP105 signaling2010

    • Author(s)
      Yamazaki K, Yamazaki T, Taki S, Miyake K, Hayashi T, Ochs HD, Agematsu K
    • Journal Title

      J. Clin. Immunol.

      Volume: 135 Issue: 1 Pages: 125-136

    • DOI

      10.1016/j.clim.2009.12.013

    • Related Report
      2012 Final Research Report
    • Peer Reviewed
  • [Journal Article] Prominent eosinophilia but less eosinophil activation in a patient with Omenn syndrome2010

    • Author(s)
      Seki M, Kimura H, Mori A, Shimada A, Yamada Y, Maruyama K, Hayashi Y, Agematsu K, Morio T, Yachie A, Kato M.
    • Journal Title

      Pediatr Int

      Volume: 52 Issue: 4 Pages: 96-9

    • DOI

      10.1111/j.1442-200x.2010.03135.x

    • Related Report
      2012 Final Research Report
    • Peer Reviewed
  • [Journal Article] 周期性の発熱を呈し臨床的にTNF receptorassociated periodic syndrome(TRAPS) と診断した成人女性の 1例.2010

    • Author(s)
      大谷寛, 中西里永子, 山田秀樹, 向山新, 布村眞季, 山崎和子, 上松一永.
    • Journal Title

      日本内科学会雑誌

      Volume: 99 Pages: 1058-1060

    • NAID

      10026331455

    • Related Report
      2012 Final Research Report
    • Peer Reviewed
  • [Journal Article] 日本小児科学会教育講演総説 自己炎症性疾患の最近の話題2010

    • Author(s)
      上松一永, 角田裕幸, 小池健一
    • Journal Title

      日本小児科学会誌

      Volume: 114 Pages: 1613-1614

    • Related Report
      2012 Final Research Report
  • [Journal Article] Potentiation of TLR9 responses for human nave B-cell growth through RP105 signaling.2010

    • Author(s)
      Yamazaki, et al.
    • Journal Title

      Clinical Immunol

      Volume: 135 Pages: 125-136

    • Related Report
      2010 Annual Research Report
    • Peer Reviewed
  • [Journal Article] A case of familial Mediterranean fever associated with compound heterozygosity for the ovrin variant L110P-E148Q/M6801 in Japan.2010

    • Author(s)
      Oshima, et al.
    • Journal Title

      Mod.Rheumatol

      Volume: 20 Pages: 193-195

    • Related Report
      2010 Annual Research Report
    • Peer Reviewed
  • [Journal Article] 家族性地中海熱2010

    • Author(s)
      上松一永
    • Journal Title

      医学のあゆみ

      Volume: 12 Pages: 1159-1163

    • Related Report
      2010 Annual Research Report
  • [Journal Article] 自己免疫疾患の概念2010

    • Author(s)
      小林法元
    • Journal Title

      小児科診療

      Volume: 12 Pages: 2083-2088

    • Related Report
      2010 Annual Research Report
  • [Journal Article] 自己炎症性疾患の最近の話題2010

    • Author(s)
      上松一永
    • Journal Title

      日本小児科学会雑誌

      Volume: 114 Pages: 1534-1541

    • NAID

      10027478045

    • Related Report
      2010 Annual Research Report
  • [Journal Article] 分類不能型免疫不全症の病態解析の最近の話題2010

    • Author(s)
      山崎崇志
    • Journal Title

      血液フロンティア

      Volume: 20 Pages: 1991-1998

    • Related Report
      2010 Annual Research Report
  • [Journal Article] 周期性の発熱を呈し臨床的にTNF receptor-associated periodic sy ndrome(TRAPS)と診断した成入女性の1例2010

    • Author(s)
      大谷寛
    • Journal Title

      日本内科学会誌

      Volume: 99 Pages: 11058-1060

    • Related Report
      2010 Annual Research Report
    • Peer Reviewed
  • [Presentation] 家族性地中海熱とその鑑別疾患について2012

    • Author(s)
      上松一永
    • Organizer
      大阪免疫研究会
    • Place of Presentation
      大阪
    • Year and Date
      2012-12-08
    • Related Report
      2012 Final Research Report
  • [Presentation] 自己炎症疾患の最近の話題2010

    • Author(s)
      上松一永
    • Organizer
      第113回日本小児科学会学術集会
    • Place of Presentation
      盛岡
    • Related Report
      2010 Annual Research Report
  • [Presentation] 家族性地中海熱とその鑑別疾患につて

    • Author(s)
      上松一永
    • Organizer
      膠原病の臓器障害研究会
    • Place of Presentation
      大阪
    • Related Report
      2012 Annual Research Report
    • Invited
  • [Book] 周期性発熱・アフタ性口内炎・咽頭炎・頸部リンパ節炎症候群 (PFAPA) 自己炎症性疾患・自然免疫不全症・近縁疾患2012

    • Author(s)
      山崎崇志、伯耆原祥、上松一永.
    • Publisher
      診断と治療社 東京
    • Related Report
      2012 Final Research Report
  • [Book] 周期性発熱・アフタ性口内炎・咽頭炎・リンパ節炎症候群の1女児例. 自己炎症性疾患・自然免疫不全症・近縁疾患2012

    • Author(s)
      伯耆原祥、山崎崇志、上松一永
    • Publisher
      診断と治療社 東京
    • Related Report
      2012 Final Research Report
  • [Book] 今日の治療指針最気管支炎2010

    • Author(s)
      上松一永
    • Total Pages
      1096
    • Publisher
      医学書院
    • Related Report
      2010 Annual Research Report
  • [Remarks] e-血液

    • URL

      http://www.e-ketsueki.com/

    • Related Report
      2012 Final Research Report
  • [Remarks] e-免疫.com 家族性地中海熱性

    • Related Report
      2012 Final Research Report

URL: 

Published: 2010-08-23   Modified: 2019-07-29  

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