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Study of the pathophysiology of congenital pseudarthrosis of the tibia.

Research Project

Project/Area Number 14571362
Research Category

Grant-in-Aid for Scientific Research (C)

Allocation TypeSingle-year Grants
Section一般
Research Field Orthopaedic surgery
Research InstitutionThe University of Tokyo

Principal Investigator

HIRAOKA Hisatada (2004)  The University of Tokyo, Faculty of Medicine, Assistant, 医学部附属病院, 助手 (10262007)

滝川 一晴 (2003)  東京大学, 医学部附属病院, 助手 (80360866)

筋野 隆 (2002)  東京大学, 医学部附属病院, 助手 (40344452)

Co-Investigator(Kenkyū-buntansha) HOSHI Kazuto  The University of Tokyo, Faculty of Medicine, Associate Professor, 医学部附属病院, 寄付講座教員(客員助教授) (30344451)
MATSUDAIRA Koh  The University of Tokyo, Faculty of Medicine, Assistant, 医学部附属病院, 助手 (10302697)
NAKAMURA Kozo  The University of Tokyo, Faculty of Medicine, Professor, 医学部附属病院, 教授 (60126133)
岡崎 裕司  東京大学, 医学部附属病院, 講師 (30241988)
平岡 久忠  東京大学, 医学部附属病院, 助手 (10262007)
滝川 一晴  東京大学, 医学部附属病院, 助手
Project Period (FY) 2002 – 2004
Project Status Completed (Fiscal Year 2004)
Budget Amount *help
¥3,700,000 (Direct Cost: ¥3,700,000)
Fiscal Year 2004: ¥500,000 (Direct Cost: ¥500,000)
Fiscal Year 2003: ¥500,000 (Direct Cost: ¥500,000)
Fiscal Year 2002: ¥2,700,000 (Direct Cost: ¥2,700,000)
KeywordsOsteoclast / RANKL / congenital pseudoarthrosis / Bone histomorphometry / 先天性脛骨偽関節賞 / 小児難治性疾患 / 先天性脛骨偽関節症 / ランクリガンド
Research Abstract

This project investigated the possible pathophysiology of congenital pseudarthrosis of the tibia, one of the most controversial pediatric entities in terms of etiopathogenesis, pathology, and treatment. Eight patients with this disease and seven adult patients with post-traumatic pseudarthrosis as a control were reviewed histologically, using pathologic materials. The area of congenital pseudarthrosis was divided into three parts with different morphological features ; a highly cellular, fibromatosis area, a cartilagenous area and an osseous area. Interestingly, a marked number of osteoclasts, tartrate resistant acid phosphatase-positive multinucleated cells were detected on the surface of the bone and cartilage, and even in the fibrous area apart from bone surfaces. Bone histomorphometric analysis revealed that in congenital pseudarthrosis, osteoclast number (N. Oc/BS) and osteoclast surface (Oc. S/BS) were 2.66 +/-0.92 [/mm](mean +/-SD) and 10.67 +/-4.86%, respectively, while in the case of adult pseudarthrosis, N. Oc/BS and Oc. S/BS were 0.62 +/-0.33 [/mm] and 2.28 +/-1.20%, respectively. Furthermore, immunohistochemical study showed that RANK ligand, an essential factor for osteoclastogenesis, was highly expressed not only in the fibroblastic cells but also osteoclasts themselves in congenital pseudarthrosis. RT-PCR analysis also revealed the higher expression of RANK ligand in congenital pseudarthrosis of the tibia than in post-traumatic pseudoarthrosis. Taken together, the enhanced osteoclastogenesis is at least in part involved in the pathophysiology of congenital pseudarthrosis of the tibia. In addition, RANK ligand, an autocrine and paracrine factor for osteoclast differentiation and activation, might be one of the therapeutic targets for this refractory disease.

Report

(4 results)
  • 2004 Annual Research Report   Final Research Report Summary
  • 2003 Annual Research Report
  • 2002 Annual Research Report
  • Research Products

    (10 results)

All 2005 2003 Other

All Journal Article (4 results) Publications (6 results)

  • [Journal Article] Treatment of Congenital Pseudarthrosis of the Tibia : A Multicenter Study in Japan.2005

    • Author(s)
      Ohnishi T, Sato W, Matsuyama J, Yajima H, Haga N, Kamegaya M, Minami A, Sato M, Yoshino S, Oki T, Nakamura K.
    • Journal Title

      J Peediatr Orthop 25

      Pages: 219-224

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      2004 Annual Research Report 2004 Final Research Report Summary
  • [Journal Article] Treatment of Congenital Pseudarthrosis of the Tibia : A Multicenter Study in Japan.2005

    • Author(s)
      Ohnishi T, Sato W, Matsuyama J, Yajima H, Haga N, Kamegaya M, Minami A, Sato M, Yoshino S, Oki T, Nakamura K.
    • Journal Title

      J Pediatr Orthop 25

      Pages: 219-224

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      2004 Final Research Report Summary
  • [Journal Article] Signal transduction pathways regulating osteoclast differentiation and function.2003

    • Author(s)
      Tanaka, S., Nakamura, I., Inoue, J., Oda, I-I., Nakamura, K.
    • Journal Title

      J Bone Miner Metab 21

      Pages: 123-133

    • NAID

      10014444230

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      2004 Final Research Report Summary
  • [Journal Article] Signal transduction pathways regulating osteoclast differentiation and function.2003

    • Author(s)
      Tanaka, S., Nakamura, I., Inoue, J., Oda, H., Nakamura, K.
    • Journal Title

      J Bone Miner Metab 21

      Pages: 123-133

    • NAID

      10014444230

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      2004 Final Research Report Summary
  • [Publications] A.Yamamoto: "Suppression of arthritic bone destruction by adenovirus-mideiated dominant-negative Ras gene transfer to synoviocytes and osteoclasts."Arthr.Rheumat. 48. 2682-2692 (2003)

    • Related Report
      2003 Annual Research Report
  • [Publications] 藤原清香, 仲村一郎, 田尻康人, 星地亜都司, 中村耕三, 織田弘美, 肥後隆三郎: "頸椎手術後の後療法中に呼吸困難をきたしたRAの1症例"関東リウマチ. 36. 7-14 (2003)

    • Related Report
      2003 Annual Research Report
  • [Publications] 仲村一郎, 田中 栄: "骨吸収と破骨細胞"Arthritis. 1. 6-11 (2003)

    • Related Report
      2003 Annual Research Report
  • [Publications] S.Tanaka, I.Nakamura, J.-I.Inoue, H.Oda, K.Nakamura: "Signal transduction pathways regulating osteoclast differentiation and function"J.Bone Miner.Metab. 21. 123-133 (2003)

    • Related Report
      2003 Annual Research Report
  • [Publications] I.Nakamura, Y.Kadono, H.Takayanagi, E.Jimi, T.Miyazaki, H.Oda, K.Nakamura, S.Tanaka, G.A.Rodan, L.T.Duong: "Interlenkin-1 regulates cytoskeletal organization in osteoclasts via TRAF6/c-Sre compilex."J. Immunol. 168. 5103-5109 (2002)

    • Related Report
      2002 Annual Research Report
  • [Publications] S.Fujiwara, I.Nakamura, T.Goto, M.Toru, S.Yokokura, K.Nakamura: "Intracortical chondromyxoid fibroma of humerus."Skeletal Radiol. (in press). (2003)

    • Related Report
      2002 Annual Research Report

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Published: 2002-04-01   Modified: 2016-04-21  

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