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A Histopathological Study on the Pathophysiology of Pseudo-Hirschsprung's Disease

Research Project

Project/Area Number 08672062
Research Category

Grant-in-Aid for Scientific Research (C)

Allocation TypeSingle-year Grants
Section一般
Research Field 小児外科
Research InstitutionHyogo College of Medicine

Principal Investigator

TOYOSAKA Akihiro  Hyogo College of Medicine, 1st Dept.of Surgery, Professor, 医学部, 助教授 (20068498)

Co-Investigator(Kenkyū-buntansha) NAKAI Yoshiyuki  Hyogo College of Medicine, 1st Dept.of Surgery, Instructor, 医学部, 講師 (50198024)
Project Period (FY) 1996 – 1998
Project Status Completed (Fiscal Year 1998)
Budget Amount *help
¥2,300,000 (Direct Cost: ¥2,300,000)
Fiscal Year 1998: ¥500,000 (Direct Cost: ¥500,000)
Fiscal Year 1997: ¥800,000 (Direct Cost: ¥800,000)
Fiscal Year 1996: ¥1,000,000 (Direct Cost: ¥1,000,000)
Keywordspseudo-Hirschsprung's disease / immaturity of ganglia / hypoganglionosis / oligoganglionosis / meconium disease / immature ganglionosis / meconium ileus / meconium ileus / Hirshsprung病類縁疾患 / meconium ilcus
Research Abstract

Pseudo-Hirschsprung's disease is defined as congenital motor dysfunction of the intestinal tract resembling Hirschsprung's disease, but harbouring intramural ganglion cells as far as the terminal rectum.
Symptons of this disease are mainly related to non-mechanical ileus and manifested in the neonatal period.
The patients were divided into two groups based on the presence of histologic abnormalities in the intramural ganglia. The group with morphologic abnormalities was divided into three groups : that is 1) hypoganglionosis or oligoganglionosis (a reduction in size of ganglion cells), and 3) others (intestinal neuronal dysplasia etc.). In the groups without morphologic abnormalities there were ClIPS and MMIHS.
This disease was dinicopathologically investigated concerning age at onset, symptoms, barium enema findings, rectoanal reflex, Ach-E activity, pathology of intramural ganglia, operative procedures prognosis and nutritional status. In the groups with morphologic abnormalities, most … More cases had meconium disease-like appearances in the operative findings. The morphologic abnormalities such as hypoganglionosis or immaturity of the myenteric plexus in the intestine seems to be the main etiologic factor in meconium ileus without mucoviscidosis. In most cases impaired intestine extended proximally beyond the colon for a variable distance, and none had an abnormality of a short segment or segmental abnormality. These findings indicate that pseudo-Hirschsprung's disease remains a serious disease of childhood, but immature ganglionosis has a good prognosis due to maturation of the ganglion cells.
Our results indicate that Hirselisprung's disease and pseudo-Hirschsprung's disease with and without morphologic abnormalities of the intramural nervous system can be separated on the basis of Ach-E activity rectoanal reflex, and barium enemia findings.
The detection of c-kit/SCF system and cathepsin D in the gastrointestinal tract may be useful in the diagnosis of pseudo-Hirschsprung's disease. Less

Report

(4 results)
  • 1998 Annual Research Report   Final Research Report Summary
  • 1997 Annual Research Report
  • 1996 Annual Research Report
  • Research Products

    (27 results)

All Other

All Publications (27 results)

  • [Publications] 豊坂昭弘: "Hirschsprung病類縁疾患-病態解明と診断・治療の研究-" 永井書店. 1-226 (1996)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1998 Final Research Report Summary
  • [Publications] 豊坂昭弘: "ヒルシュスプルング病類縁疾患-特に壁内神経細胞減少症について-" 小児外科. 28・9. 1037-1047 (1996)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1998 Final Research Report Summary
  • [Publications] 豊坂昭弘: "腸壁内神経細胞未熟症とmeconium disease" Hirschsprung病類縁疾患(永井書店). 67-80 (1996)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1998 Final Research Report Summary
  • [Publications] 岡本英三: "ヒルシュスプルング病類縁疾患-定義・各称・分類-" 小児外科. 28(9). 1017-1022 (1996)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1998 Final Research Report Summary
  • [Publications] 豊坂昭弘: "Hirschsprung病および類縁疾患" 小児科学(白木和夫編)、医学書院. 1000-1002 (1997)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1998 Final Research Report Summary
  • [Publications] 豊坂昭弘: "Hirschsprung病類縁疾患の病態と診断・治療" 日小教会誌. 15(1). 67-78 (1999)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1998 Final Research Report Summary
  • [Publications] Toyosaka A.et al.: "Hypoganglionosis of the intestine : A clinicopathologic study." Jpn.J.Pediatr.Surg.28(9). 1037-1047 (1996)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1998 Final Research Report Summary
  • [Publications] Toyosaka A., et al.: "Immature ganglionosis and meconium disease" pseudo-Hirschsprung's disease. (Nakai Co.). 67-80 (1996)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1998 Final Research Report Summary
  • [Publications] Okamoto E.and Toyosaka A.: "Pseudo-Hirschsprung's disease - The difinition, Name and Clasification-" Jpn.J.Pediatr.Surg.28(9). 1017-1022 (1996)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1998 Final Research Report Summary
  • [Publications] Toyosaka A.: "Pseudo-Hirschsprung's disease-The pathophysiology, diagnosis and treatment-" J.Jap.Soc.Pediatr.Rad.15(1). 67-78 (1999)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1998 Final Research Report Summary
  • [Publications] TOYOSAKA A.et al.: Pseudo-Hirschsprung's disease- Research on the pathophysiology, diagnosis and treatment -, Edited by Toyosaka. Nagai co., 1-226 (1996)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1998 Final Research Report Summary
  • [Publications] Toyosaka A.: Hirschsprung's disease and pseudo-Hirschsprung's disease. Pediatrics (edited by Shiraki). IGAKUSHOIN, 1000-1002 (1997)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1998 Final Research Report Summary
  • [Publications] 豊坂 昭弘: "Hirschsprung病類縁疾患-病能解明と診断・治療の研究-" 永井書店. 1-226 (1996)

    • Related Report
      1998 Annual Research Report
  • [Publications] 豊坂 昭弘: "ヒルシュスプルング病類縁疾患-特に壁内神経細胞減少症について-" 小児外科. 28・9. 1037-1047 (1996)

    • Related Report
      1998 Annual Research Report
  • [Publications] 豊坂 昭弘: "腸壁内神経細胞未熟症とmeconjum disease" Hirchsprung病類縁疾患 (永井書店). 67-80 (1996)

    • Related Report
      1998 Annual Research Report
  • [Publications] 岡本 英三: "ヒルシュスプルング病類縁疾患-定義・名称・分類-" 小児外科. 28(9). 1017-1022 (1996)

    • Related Report
      1998 Annual Research Report
  • [Publications] 豊坂 昭弘: "Hirschsprung病および類縁疾患" 小児科学 (白木和夫編),医学書院. 1000-1002 (1997)

    • Related Report
      1998 Annual Research Report
  • [Publications] 豊坂昭弘: "Hirschsprung病類縁疾患の病態と診断・治療" 日小放会誌. 15(1). 67-78 (1999)

    • Related Report
      1998 Annual Research Report
  • [Publications] 豊坂 昭弘: "Hirschsprung病類縁疾患-病能解明と診断・治療の研究-" 永井書店. 1-226 (1996)

    • Related Report
      1997 Annual Research Report
  • [Publications] 豊坂 昭弘: "ヒルシュスプルング病類縁疾患-特に壁内神経細胞減少症について-" 小児外科. 28・9. 1037-1047 (1996)

    • Related Report
      1997 Annual Research Report
  • [Publications] 豊坂 昭弘: "腸壁内神経細胞未熟症とmeconium disease" Hirschsprung病類縁疾患(永井書店). 67-80 (1996)

    • Related Report
      1997 Annual Research Report
  • [Publications] 岡本 英三: "ヒルシュスプルング病類縁疾患-定義・各称・分類-" 小児外科. 28(9). 1017-1022 (1996)

    • Related Report
      1997 Annual Research Report
  • [Publications] 豊坂 昭弘: "Hirschsprung病および類縁疾患" 小児科学(白木和夫編)、医学書院. 1000-1002 (1997)

    • Related Report
      1997 Annual Research Report
  • [Publications] 豊坂昭弘: "Hirschsprung病類縁疾患-病能解明と診断治療の研究-" 永井書店. 1-226 (1996)

    • Related Report
      1996 Annual Research Report
  • [Publications] 豊坂昭弘: "ヒルシユスプルング病類縁疾患-特に壁内神経細胞減少症について-" 小児外科. 28(9). 1037-1047 (1996)

    • Related Report
      1996 Annual Research Report
  • [Publications] 豊坂昭弘: "腸壁内神経細胞未熟症とmeconium disease" Hirschsprung病類縁疾患 (永井書店). 67-80 (1996)

    • Related Report
      1996 Annual Research Report
  • [Publications] 岡本英三: "ヒルシユスプルング病類縁疾患-定義・名称・分類-" 小児外科. 28(9). 1017-1022 (1996)

    • Related Report
      1996 Annual Research Report

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Published: 1996-04-01   Modified: 2016-04-21  

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