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Lack of intestinal pacemaker (C-KIT-positive) cells in infantile hypertrophic pyloric stenosis
A Yamataka1, T Fujiwara, Y Kato
1Department of Surgery, Dokkyo University School of Medicine, Tochigi, Japan.
Insights
Infantile hypertrophic pyloric stenosis (IHPS) may stem from a lack of C-KIT positive cells, crucial for gut motility. Reduced C-KIT expression in pyloric muscles suggests a potential cause for this condition.
Area of Science:
- Gastroenterology
- Developmental Biology
- Cell Biology
Background:
- The exact cause of infantile hypertrophic pyloric stenosis (IHPS) remains unclear.
- The protoncogene c-kit and its protein product (C-KIT) are vital for autonomic gut motility and intestinal pacemaker activity.
- C-KIT positive cells are identified as key players in mammalian gut motility regulation.
Purpose of the Study:
- To investigate the presence and distribution of C-KIT positive cells in the pyloric muscles of infants with and without IHPS.
- To determine if alterations in C-KIT expression correlate with the pathogenesis of IHPS.
Main Methods:
- Immunohistochemical techniques using antihuman C-KIT sera were employed.
- Pyloric muscle tissues from 23 infants (16 with IHPS, 7 controls) were analyzed.
- The presence and location of C-KIT immunoreactive (IR+) cells were examined.
Main Results:
- Control infants exhibited numerous C-KIT-IR+ cells within the muscle layers and around the myenteric plexuses.
- Infants with IHPS showed a significant reduction or complete absence of C-KIT-IR+ cells.
- No C-KIT-IR+ cells were detected around the myenteric plexuses in IHPS patients.
Conclusions:
- A deficiency in C-KIT expression, indicative of impaired intestinal pacemaker activity, is strongly associated with the hypertrophic pyloric smooth muscles in IHPS.
- These findings suggest that reduced c-kit expression is a significant factor in the development of IHPS.
Abstract:
The pathogenesis of infantile hypertrophic pyloric stenosis (IHPS) is not well understood. Recent studies have shown that the protonocogene c-kit is essential for the development or maintenance of autonomic gut motility, and also show that the c-kit gene protein product (C-KIT) positive cells in the mammalian gut are responsible for intestinal pacemaker activity. This study examines cells in the pyloric muscles of 23 patients (16 with IHPS, 7 controls) for the presence of the C-KIT (C-KIT+), using immunohistochemical techniques with antihuman C-KIT sera. In the controls, many C-KIT immunoreactive (IR+) cells were observed in the muscle layers. The myenteric plexuses were demarcated by a moderate number of C-KIT-IR+ cells. However, in the IHPS patients, C-KIT-IR were either absent or significantly reduced. No C-KIT-IR+ cells were found around the myenteric plexuses. These findings suggest that a lack of c-kit expression (as an indicator of intestinal pacemaker activity) in the hypertrophic pyloric smooth muscles may be an important factor in the pathogenesis of IHPS.