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Maturation therapy of enteric ganglia for pseudo-Hirschsprung's disease.

Research Project

Project/Area Number 07457424
Research Category

Grant-in-Aid for Scientific Research (B)

Allocation TypeSingle-year Grants
Section一般
Research Field 小児外科
Research InstitutionKyoto prefectural University of Medicine

Principal Investigator

IWAI Naomi  Kyoto Prefect Univ.of Med.Professor, 医学部, 教授 (90128695)

Co-Investigator(Kenkyū-buntansha) SHIMOTAKE Takashi  Kyoto Prefect Univ.of Med.Associate, 医学部, 助手 (90254341)
YANAGIHARA Jun  Kyoto Prefect Univ.of Med.Associate Prefes, 医学部, 助教授 (30158026)
Project Period (FY) 1995 – 1997
Project Status Completed (Fiscal Year 1997)
Budget Amount *help
¥7,600,000 (Direct Cost: ¥7,600,000)
Fiscal Year 1997: ¥1,700,000 (Direct Cost: ¥1,700,000)
Fiscal Year 1996: ¥1,800,000 (Direct Cost: ¥1,800,000)
Fiscal Year 1995: ¥4,100,000 (Direct Cost: ¥4,100,000)
KeywordsHirschsprung / neural cell / enteric ganglia / maturation / 消化管神経叢 / ヒルシュスプリング病類縁疾患 / 神経栄養因子
Research Abstract

In this research project, possible maturation therapy of enteric ganglia for children with Hirschsprung's disease and its related disorders was studied. Mutational analysis of the RET proto-oncogene in children with hereditary and sporadic Hirschsprung's disease was performed. Germline mutations of the RET proto-oncogene have been described also in pedigrees with multiple endocrine neoplasia (MEN) type 2A,MEN type 2B,and hereditary medullary thyroid carcinoma (FMTC). Pathology of neural crest-derived cells is supposed to be intimately associated with these diseases. Further investigations are required to examine possible association between the RET mutational pattern and type of disease expression as intestinal aganglionosis. These variations of mutational pattern of RET may be apllied to make a genetic diagnosis of high risk carriers for these diseases and also to make therapeutic approach for these disease entities.

Report

(4 results)
  • 1997 Annual Research Report   Final Research Report Summary
  • 1996 Annual Research Report
  • 1995 Annual Research Report
  • Research Products

    (35 results)

All Other

All Publications (35 results)

  • [Publications] T.Shimotake, N.Iwai et al: "Germline mutation of the RET proto-oncogene in children with total intestinal aganglinosis." Journal of Pediatric Surgery. 32(3). 498-500 (1997)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1997 Final Research Report Summary
  • [Publications] T.Shimotake, N.Iwai et al: "Germline mutation of the RET proto-oncogene in pedigree with MEN type 2A." Journal of Pediatric Surgery. 31(6). 779-781 (1996)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1997 Final Research Report Summary
  • [Publications] T.Shimotake, N.Iwai et al: "Absence of rectoanal inhibitory reflex in children with multiple endocrine neoplasia type 2B." Journal of Pediatric Surgery. 33 IN-PRESS.

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1997 Final Research Report Summary
  • [Publications] Y.Kubota T.Shimotake et al: "Development' of anorectal malformations in mice model using etretinate." Journal of Pediatric Surgery. 33(1). 127-129 (1998)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1997 Final Research Report Summary
  • [Publications] T.Shimotake, N.Iwai et al: "Impaired proliferative activity of mesenchymal cells affects the migratory pathway for neural crest cells." Journal of Pediatric Surgery. 30(3). 445-447 (1995)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1997 Final Research Report Summary
  • [Publications] N.Iwai, T.Shimotake et al: "Is a new biofeed back therapy for fecal incontinence patients who have anorectal malformations?" Journal of Pediatric Surgery. 32(11). 1626-1629 (1997)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1997 Final Research Report Summary
  • [Publications] 下竹 孝志, 岩井 直躬: "岡本英三監修Hirschspung病類縁疾患病態解明と診断治療の研究" 永井書店, 226 (1996)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1997 Final Research Report Summary
  • [Publications] Shimotake T,Iwai N,Inoue K,Kimura T,Ichikawa D,Abe T,Inazawa J.: "Germline mutation of the RET proto-oncogene in children with total intestinal aganglionosis." Journal of Pediatric Surgery. 32(3). 498-500 (1997)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1997 Final Research Report Summary
  • [Publications] Shimotake T,Iwai N,Inoue K,Inazawa J,Nishisho I.: "Germline mutations of the RET proto-oncogene in pedigree with MEN type 2A-DNA analysis and its implications for pediatric surgery." Journal of Pediatric Surgery. 31(6). 779-781 (1996)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1997 Final Research Report Summary
  • [Publications] Shimotake T., Kubota Y,Yanagihara J,Iwai N.: "Absence of rectoanal inhibitory reflex in children with multiple endocrine neoplasia type 2B." Journal of Pediatric Surgery. (in press).

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1997 Final Research Report Summary
  • [Publications] Kubota Y,Shimotake T., Yanagihara J,Iwai N.: "Development of anorectal malformations in mice model using etretinate." Journal of Pediatric Surgery. 33(1). 127-129 (1998)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1997 Final Research Report Summary
  • [Publications] Shimotake T,Iwai N,Yanagihara J,Deguchi E,Fushiki S.: "Impaired proliferative activity of mesenchymal cells affects the migratory pathway for neural crest cells in the developing gut of mutant murine embryos (Is/Is)." Journal of Pediatric Surgery. 30(3). 445-447 (1995)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1997 Final Research Report Summary
  • [Publications] Iwai N,Iwata G,Kimura O,Yanagihara J.: "Is a new biofeed back therapy for fecal incontinence in patients who have anorectal malformations?" Journal of Pediatric Surgery. 32(11). 1626-1629 (1997)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1997 Final Research Report Summary
  • [Publications] Shimotake T,Iwai N,Yanagihara J,Inoue K,Fushiki S.: "Innervation patterns in congenital biliary dilatation." European Journal of Pediatric Surgery. 5(5). 265-270 (1995)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1997 Final Research Report Summary
  • [Publications] Shimotake T,Jikihara R,Yanagihara J,Iwai N.: "Successful management of bilateral congenital eventration of the diaphragm." Pediatric Surgery International. 10(3). 173-174 (1995)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1997 Final Research Report Summary
  • [Publications] Yanagihara J,Iwai N,Tokiwa K,Deguchi E,Shimotake T.: "Results of a modified Duhamel Operation for Hirschsprung's disease using the GIA stapler." European Journal of Pediatric Surgery. 7(2). 77-79 (1997)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1997 Final Research Report Summary
  • [Publications] T.Shimotake, N.Iwai et al: "Germline mutation of the RET proto-oncogene in children with total intestinal aganglinosis." Journal of Pediatric Surgery. 32(3). 498-500 (1997)

    • Related Report
      1997 Annual Research Report
  • [Publications] T.Shimotake, N.iwai, et al: "Germline mutation of the RET proto-oncogene in pedigree with MEN type 2A." Journal of pediatric Surgery. 31(6). 779-781 (1996)

    • Related Report
      1997 Annual Research Report
  • [Publications] T.Shimotake, N.Iwai, et al: "Absence of rectoanal inhibitory reflex in children with multiple endocrine neoplasia type 2B." Journal of Pediatric Surgery. 33. IN-PRESS

    • Related Report
      1997 Annual Research Report
  • [Publications] Y.Kubota, T.shimotake, et al: "Development of anorectal malformations in mice model using etretinate." Journal of Pediatric Surgery. 33(1). 127-129 (1998)

    • Related Report
      1997 Annual Research Report
  • [Publications] T.Shimotake, N.Iwai, et al: "Impaired proliferative activity of mesenchymal cells affects the migratory for pathway neural crest cells." Journal of Pediatric Surgery. 33(1). 127-129 (1998)

    • Related Report
      1997 Annual Research Report
  • [Publications] N.Iwai, G.Iwata, et al: "Is a new biofeed back therapy for fecal incontinence in patients who have anorectal malformations?" 32(11). 1626-1629 (1997)

    • Related Report
      1997 Annual Research Report
  • [Publications] 下竹孝志, 岩井直躬: "岡本英三監修 Hirschsprung病類縁疾患 病態解明と診断治療の研究" 永井書店, 226 (1996)

    • Related Report
      1997 Annual Research Report
  • [Publications] T. Shimotake, N. Iwai et al.: "Germline mutetions of the RET proto-oncogene in children with total intestinal aganglionosis" Journal of Pediatric Surgery. 32 (in press). (1997)

    • Related Report
      1996 Annual Research Report
  • [Publications] T. Shimotake, N. Iwai et al.: "Germline mutation of the RET proto-oncogene in pedigree with MEN type 2A" Journal of Pediatric Surgery. 31 (6). 779-781 (1996)

    • Related Report
      1996 Annual Research Report
  • [Publications] T. Shimotake, N. Iwai et al.: "Impaired proliferative activity of mesenchymal cells affects the migratory pathway for neural crest cells in the developing gut" Journal of Pediatric Surgery. 30 (3). 445-447 (1995)

    • Related Report
      1996 Annual Research Report
  • [Publications] T. Shimotake, N. Iwai et al.: "Innervation patterns in congenital biliary dilatation" European Journal of Pediatric Surgey. 5 (5). 265-270 (1995)

    • Related Report
      1996 Annual Research Report
  • [Publications] T. Shimotake, N. Iwai et al.: "Successful management of bilateral congenital eventration of the diaphagm" Pediatric Surgery International. 10 (2). 173-174 (1995)

    • Related Report
      1996 Annual Research Report
  • [Publications] 下竹孝志,井上勝裕,久保田良浩,柳原 潤,岩井直躬: "ヒルシュスプルング病類縁疾患におけるcranio-caudalmigration theoryの意義" 小児外科. 28 (9). 1053-1057 (1996)

    • Related Report
      1996 Annual Research Report
  • [Publications] 下竹孝志,岩井直躬: "岡本英三監修Hirschsprung病類縁疾患 病態解明と診断・治療の研究" 永井書店, 226 (1996)

    • Related Report
      1996 Annual Research Report
  • [Publications] 下竹孝志: "阿部達生監修臨床FISHプロトコール 目で見る染色体・遺伝子診断法" 秀潤社, 204 (1997)

    • Related Report
      1996 Annual Research Report
  • [Publications] Shimotake T,et al.: "Impaired proliferative activity of mesenchymal cells affects the migratory pathway for neural crest cells in the developing gut of mutant murine embryos." Journal of Pediatric Surgery. 30. 445-447 (1995)

    • Related Report
      1995 Annual Research Report
  • [Publications] Shimotake T,et al.: "Innervation patterns in congenital biliary dilatation." European Journal of Pediatric Surgery. 5. 265-270 (1995)

    • Related Report
      1995 Annual Research Report
  • [Publications] Shimotake T,et al.: "Successful management of congenital bilateral eventration of the diaphragm" Pediatric Surgery International. 10. 173-174 (1995)

    • Related Report
      1995 Annual Research Report
  • [Publications] Shimotake T,et al.: "Germline mutation of the RET proto-oncogene in multiple endocrine neoplasia type 2A:DNA diagnosis and its implications for pediatric surgeons" Journal of Pediatric Surgery. 31(in press). (1996)

    • Related Report
      1995 Annual Research Report

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Published: 1995-04-01   Modified: 2016-04-21  

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