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Disturbance of Blood Coagulation in Juvenile Chronic Arthritis, and Epidcmiology, Clinical Studies on CINCA Syndrome.

Research Project

Project/Area Number 04670626
Research Category

Grant-in-Aid for General Scientific Research (C)

Allocation TypeSingle-year Grants
Research Field 小児科学
Research InstitutionNihon University

Principal Investigator

INAMO Yasuji  Departments of pediatrics, Shool of Medicine Nihon University, instructor, 医学部, 助手 (30167267)

Co-Investigator(Kenkyū-buntansha) OKUNI Masahiko  Departments of pediatrics, Shool of Medicine Nihon University, professor, 医学部, 教授 (70058832)
Project Period (FY) 1992 – 1994
Project Status Completed (Fiscal Year 1994)
Budget Amount *help
¥700,000 (Direct Cost: ¥700,000)
Fiscal Year 1993: ¥700,000 (Direct Cost: ¥700,000)
Keywordsjuvenile chronic arthritis / CINCA Syndrome / FVIIa : activated factor VIIa / vWF : Ag : von Willebrand fator antigen ; / JRA / 組織因子 / 成長障害 / 活性化VII因子 / TFPI
Research Abstract

We measured FVlla, vWF : Ag, D-dimer and TAT to study the abnormality of coagulation in patients with juvenile chronic arthritis (JCA). Our subjects included 14 systemic JCA,16 pauciarticular JCA and 16 polyarticular JCA without DIC,thrombosis and liver dysfunction. All types of JCA showed an increased of FVlla, D-dimer and TAT.In particular, only systemic JCA has characteristically an clevation of vWF : Ag. We concluded that all types of JCA constitute a atate of subclinical hypercoagulopathy caused by tissue damage and that additionally systemic JCA involves a prothrombotic state brought on by vasculitis.
We report a Japanese child of the CINCA syndrome. The case was accompanied by the severely growth retardation and no pubertal development. However the growth hormone secretion was normal. The growth hormone therapy failed to increase his height. He showed the decrease of the GH secretion by insulin stimulation test, it caused that the long term glucocorticoid therapy influenced the suppression of hypothalanus-hypophysis axis. However he main cause of this patient's growth retardation is the early closure of physis, because GH secretion by L-DOPA and GRF urinary GH and IGF-1 were normal, moreover he had no effect of GH therapy (0.5U/kg/week) during one year. It is the difference between JRA and CINCA syndrome on the effect of GH therapy, because GH therapy effects on growth velocity of JRA patients. On this point, CINCA syndrome is essentially differrent from JRA on the pathogenesis. He had the dificiency of IgG3 and IgG4 with the decrease of B-cell population.
Abbreviations : FVIIa : activated factor VIIa ; vWF : Ag : von Willebrand factor antigen ; TAT : thrombin-antithrombin III complex ; JCA : Juvenile Chronic Arthritis ; CINCA syndrome : the Chronic, Infantile, Neurological, Cutaneous and Articular Syndrome

Report

(3 results)
  • 1994 Final Research Report Summary
  • 1993 Annual Research Report
  • 1992 Annual Research Report
  • Research Products

    (23 results)

All Other

All Publications (23 results)

  • [Publications] 稲毛康司: "全身発症型にみられる血液凝固・線溶能亢進について" 小児内科. 24. 356-360 (1992)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1994 Final Research Report Summary
  • [Publications] Yasuji Inamo.: "Studies on the influence of pemostatic molecular markers and platelet-derived groruth foutor on endotheliol damage in Kawasaki diseace" Proceedings of fouth international symposium on kawasaki diseace.209-212 (1993)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1994 Final Research Report Summary
  • [Publications] Yasuji Inamo.: "Case report:Chronic,lnfoutile,Neurological,Cutaneous and Articular Syudrone in Japan" Clinical and Exper:mental Rheumatologg. 12. 447-449 (1994)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1994 Final Research Report Summary
  • [Publications] 稲葉康司: "CINCA症候群(小児慢性神経皮膚関節症候群)" 小児科. 1145-1149 (1994)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1994 Final Research Report Summary
  • [Publications] Yasuji Inamo: "Growth Failure in a Japanese Child with the chronic,Infautile,neurological,Cutaneus and Autiwlr syudre." Clin Pediatr Endocrinol.3. 225-227 (1994)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1994 Final Research Report Summary
  • [Publications] Yasuji Inamo: "Increase of FVIIa and Hemostatic Molecular Markers in Juvonele Chronic Arthrtis" British J.of Rheumatology. (印刷中).

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1994 Final Research Report Summary
  • [Publications] Yasuji Inamo: "Haemostasis abnormalities in systemic oncet JRA." Shouni-Naika (in Japanese). vol.24, no.3. 356-360 (1992)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1994 Final Research Report Summary
  • [Publications] Yasuji Inamo: "Studies on the influence of hemostatic molecular markers and plateletderived growth factor on endothelial damage in Kawasaki disease." Proceedings of the Fourth international symposium on Kawasaki disease ISBN 0-87493-200-9, American Heart Association, American Heart Association National Center, Dallas, TX.Masato TAKAHASHI,Kathryn Taubert. 209-212 (1993)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1994 Final Research Report Summary
  • [Publications] Yasuji Inamo: "Case report : Chronic, Infantile, Neurological, Cutaneous and Articular Syndrome in Japan." Clinical and Experimental Rheumatology. vol.12 no.3. 447-449 (1994)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1994 Final Research Report Summary
  • [Publications] Yasuji Inamo: "CINCA syndrome" Shounika (in Japanese). vol.35, no.9. 1145-1149 (1994)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1994 Final Research Report Summary
  • [Publications] Yasuji Inamo: "Growth Failure in a Japanese Child with the Chronic, Infantile, neurological, Cutaneous and Articular syndrome (CINCA syndrome)" ClinPediatr Endocrinol. vol.3, Suppl 5. 225-227 (1994)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1994 Final Research Report Summary
  • [Publications] Yasuji Inamo: "Increase of Flla and Haemostatic Molecular Markers in Juvenile Chronic Arthritis" British J.of Rheumatology. (in press).

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1994 Final Research Report Summary
  • [Publications] 稲毛康司: "若年性関節リウマチおよびSLEにみられた血液凝固・線溶能亢進状態についての検討" リウマチ. 33. 31-31 (1991)

    • Related Report
      1993 Annual Research Report
  • [Publications] 稲毛康司: "若年性関節リウマチ(全身発症型)にみられる血液凝固異常について、とくにDIC発症例の一例と凝固亢進状態にある一例からの検討" 日本小児科学会雑誌. 95. 552-552 (1991)

    • Related Report
      1993 Annual Research Report
  • [Publications] 稲毛康司: "Stevens-Tohnson症候群と多型〓出性〓〓(軽症型)における顆粒球エラスターゼの関与とウリナスタチンの治療効果について" 小児科臨床. 45. 381-385 (1992)

    • Related Report
      1993 Annual Research Report
  • [Publications] 稲毛康司: "若年性関節リウマチにおける血液障害の全身発症型にみられる血液凝固・線溶能亢進について" 小児内科. 24. 356-360 (1992)

    • Related Report
      1993 Annual Research Report
  • [Publications] 稲毛康司: "CINCA症候群と考えられる2例" リウマチ. 32. 697-697 (1992)

    • Related Report
      1993 Annual Research Report
  • [Publications] Yasuji Inamo: "Studies on the cnfluence of hemostatic molecular markers and PDGF on endotterisal damage in Kawasaki disene" Pivceedings of the Fourth Internatonal Symposiume on Kawasaki disease.209-212 (1993)

    • Related Report
      1993 Annual Research Report
  • [Publications] 稲毛 康司: "若年性関節リウマチおよびSLEにみられた血液凝固・線溶能亢進状態についての検討" リウマチ. 33. 31-31 (1991)

    • Related Report
      1992 Annual Research Report
  • [Publications] 稲毛 康司: "若年性関節リウマチ(全身発症型)にみられる血液凝固異常について. とくにDIC発症例の一例と凝固亢進状態にある一例からの検討" 日本小児科学会雑誌. 95. 552-552 (1991)

    • Related Report
      1992 Annual Research Report
  • [Publications] 稲毛 康司: "Stevens-Johnson 症候群と多型滲出性紅斑(軽症型)における顆粒球エラスターゼね関与とウリナスタチンの治療効果について" 小児科臨床. 45. 381-385 (1992)

    • Related Report
      1992 Annual Research Report
  • [Publications] 稲毛 康司: "若年性関節リウマチにおける血液障害の全身発症型にみられる血液凝固・線溶能亢進について" 小児内科. 24. 356-360 (1992)

    • Related Report
      1992 Annual Research Report
  • [Publications] 稲毛 康司: "CINCA症候群と考えられる2例" リウマチ. 32. 697-697 (1992)

    • Related Report
      1992 Annual Research Report

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Published: 1993-04-01   Modified: 2016-04-21  

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