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2003 Fiscal Year Final Research Report Summary

ANALYSIS OF THE PATHOGENESIS OF THE SERONEGATIVE AUTOIMUNE DISEASE

Research Project

Project/Area Number 13670846
Research Category

Grant-in-Aid for Scientific Research (C)

Allocation TypeSingle-year Grants
Section一般
Research Field Pediatrics
Research InstitutionTOHO UNIVERSITY

Principal Investigator

SHINOMIYA Noriaki  TOHO UNI., SCHOOL OF MEDICINE, PROF., 医学部, 教授 (10104225)

Co-Investigator(Kenkyū-buntansha) UNO Taku  TOHO UNI., SCHOOL OF MEDICINE, INSTRUCTOR, 医学部, 助手 (30318240)
YAMAGUCHI Yukitoshi  TOHO UNI., SCHOOL OF MEDICINE, ASSISTANT PROF, 医学部, 講師 (30277339)
NIHEI Kouichi  TOHO UNI., SCHOOL OF MEDICINE, ASSISTANT PROF, 医学部, 講師 (20218241)
Project Period (FY) 2001 – 2003
Keywordschildhood-onset myasthenia gravis / ant-acetylcholine receptor autoantibody / seronegative autoimrnmue / HLA DR / DO allele / latent general type / DRB1^*1302 / DOA1^*0102 / DOB1^*0604
Research Abstract

As most patients with myasthenia gravis(MG)of childhood onset have low or negative ant-acetylcholine receptor(AChR) autoantibody titier, the role of anti-AChR autoantibodies as the cause of muscle weakness remains as open question.As for the prevalence of child-hood MG in Japan, the largest onset-age group was found to be under three years.To elucidate whether the Japanese patients with childhood-onset MG is a variant type, the correlation between clinical features and HLA DR/DQ allele frequencies was examined in 87 Japanese patients with childhood-onset disease.HLA genotypes DRB1^*1302/DQA1^*0102/DQB1^*0604 and DRB1^*1302/DQA1^*0102/DQB1^*0604 were signiticant higher in patients than in healthy controls(Pc<0.0001, RR=8.5 ; Pc<0.0001, RR=5.5, for two genotypes, respectively).Patients who had a significant higher likelihood of the HLA types DRB1^*1302/DQA1^*0102/DQB1^*0604 or DRB1^*1302/DQA1^*0102/DQB1^*0604 belonged to the latent general type(LG) of MG ; this is clinically ocular type, but shows myasthenic electromyographic findings in extremity muscles.The LG type of MG was observed in 78% of patients exhibiting the clinically ocular type ; this group comprised approximately 75% of patients with childhood-onset MG.These data suggested that the LG type with childhood-onset associated with HLA types DRB1^*1302/DQA1^*0102/DQB1^*0604 is a specific clinical type of childhood-onset MG which may carry a specific genes predisposing toward the disease.

  • Research Products

    (17 results)

All Other

All Publications (17 results)

  • [Publications] Uno T, Shinomiya N: "EYE1 Gene Nonsense Mutation in a Japanese Family with Brachio-Oto Renal(BOR) Syndrome"Pediatr Int.. 46. (2004)

    • Description
      「研究成果報告書概要(和文)」より
  • [Publications] Shinomiya N: "A variant of childhood-onset myasthenia gravis : HLA typing and clinical characteristics in Japan"Clin.Immunol. 110. 154-158 (2004)

    • Description
      「研究成果報告書概要(和文)」より
  • [Publications] 四宮範明: "重症筋無力症患者末梢血および胸腺内CD4+細胞ラインにおけるTCRVβレパートリーの検討"Neuroimmunology. 12. 50 (2004)

    • Description
      「研究成果報告書概要(和文)」より
  • [Publications] Nihei K, Shinomiya N: "Wolff-Parkinson-White(WPW) syndrome in isolated noncompaction of the ventricular myocardium"Circulation J. 68. 82-84 (2004)

    • Description
      「研究成果報告書概要(和文)」より
  • [Publications] 井沢雅子, 四宮範明: "乳児期発症アトピー性皮膚炎患者におけるTH1/TH2の検討"日児誌. 107. 757-762 (2003)

    • Description
      「研究成果報告書概要(和文)」より
  • [Publications] 四宮範明: "セロネガティブ自己免疫疾患の免疫学的病因の解析"日臨免疫会誌. 25. 302-309 (2002)

    • Description
      「研究成果報告書概要(和文)」より
  • [Publications] Uno T, Shinomiya N: "EYEL Gene Nonsense Mutation in a Japanese Family with Branchio-Oto-Renal(BOR)Syndrome"Pedatr Int. (in press). (2004)

    • Description
      「研究成果報告書概要(欧文)」より
  • [Publications] Shinomiya, N: "A Variant of Childhood Myasthenia Gravis : HLA Typing and Clinical Characteristicsin Japan"Clin Immunol. 110. 154-158 (2004)

    • Description
      「研究成果報告書概要(欧文)」より
  • [Publications] Shinomiya N: "TCRVβ Repatoir of CD4+ T cell Lines from Periheral Blood or Thymus of Patients with Myasthenia Gravis."Neuroimmunol. 12. 50 (2004)

    • Description
      「研究成果報告書概要(欧文)」より
  • [Publications] Nichei K, Shinomiya N: "Wolff-Parkinson-White(WPW)Syndrome in Isolated Noncompaction of Ventricular Myocaldium(INVM)"Cir J. 68. 82-84 (2004)

    • Description
      「研究成果報告書概要(欧文)」より
  • [Publications] Izawa M, Shinomiya N: "TH1/TH2 Balance from the Peripheral Blood in Infantile Atopic Dermatitis"Pediatr Japomca. 107. 757-762 (2003)

    • Description
      「研究成果報告書概要(欧文)」より
  • [Publications] Shinomiya N: "A variant of Childhood Myasthenia Gravis : HLA Typing and Clinical Characteristics in Japan"J Allergy Clin Immuno. 111(suppl12). 316 (2003)

    • Description
      「研究成果報告書概要(欧文)」より
  • [Publications] Shinomiya N: "Analysis of the Pathogenesis of the Seronegative Autoimmune Disease."Jpn J Clin Immuno. 25. 302-309 (2002)

    • Description
      「研究成果報告書概要(欧文)」より
  • [Publications] Shinomiya N: "Increased CCR4+CD4+ Lymphocytes in Peripheral blood from Infantile Atopic Dermatitis patient"J Allergy Clin Immuno. 111(suppl12). 316 (2002)

    • Description
      「研究成果報告書概要(欧文)」より
  • [Publications] Kishida M, Shinomiya N: "Mouthpiece versus Facemask for Delivery of Salbutanol in Excerebated Childhood Asthma."J Asthma. 39. 337-339 (2002)

    • Description
      「研究成果報告書概要(欧文)」より
  • [Publications] Shinomiya N: "Analysis of the Pathogenesis of the Seronegative Autoimmune Disease.----Model of Childhood Myasthenia Gravis"J Med Soc Toho. 49. 37-40 (2002)

    • Description
      「研究成果報告書概要(欧文)」より
  • [Publications] Shinomiya N: "Analysis of CD4+ T cell TCR Vβ or Vα repertoir From Peripheral Blood of Patients with chilodhood Myasthenia gravis."Neuroimmunol. 10. 76-78 (2002)

    • Description
      「研究成果報告書概要(欧文)」より

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Published: 2005-04-19  

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