1998 Fiscal Year Final Research Report Summary
A Histopathological Study on the Pathophysiology of Pseudo-Hirschsprung's Disease
| Project/Area Number |
08672062
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| Research Category |
Grant-in-Aid for Scientific Research (C)
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| Allocation Type | Single-year Grants |
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| Section | 一般 |
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| Research Field |
小児外科
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| Research Institution | Hyogo College of Medicine |
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Principal Investigator |
TOYOSAKA Akihiro Hyogo College of Medicine, 1st Dept.of Surgery, Professor, 医学部, 助教授 (20068498)
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| Co-Investigator(Kenkyū-buntansha) |
NAKAI Yoshiyuki Hyogo College of Medicine, 1st Dept.of Surgery, Instructor, 医学部, 講師 (50198024)
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| Project Period (FY) |
1996 – 1998
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| Keywords | pseudo-Hirschsprung's disease / immaturity of ganglia / hypoganglionosis / oligoganglionosis / meconium disease / immature ganglionosis / meconium ileus |
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| 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
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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
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