Lack of intestinal pacemaker (C-KIT-positive) cells in infantile hypertrophic pyloric stenosis.
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.
Yamataka
A
A
Department of Surgery, Dokkyo University School of Medicine, Tochigi, Japan.
Fujiwara
T
T
Kato
Y
Y
Okazaki
T
T
Sunagawa
M
M
Miyano
T
T
D001683:Biological Clocks; D005260:Female; D019084:Fluorescent Antibody Technique, Indirect; D005769:Gastrointestinal Motility; D006801:Humans; D006984:Hypertrophy; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D009197:Myenteric Plexus / Q000503:physiopathology / Q000648:ultrastructure; D019009:Proto-Oncogene Proteins c-kit / Q000032:analysis*; D011519:Proto-Oncogenes / Q000502:physiology*; D011707:Pyloric Stenosis / Q000209:etiology* / Q000378:metabolism / Q000473:pathology; D011708:Pylorus / Q000473:pathology / Q000648:ultrastructure
D019009:Proto-Oncogene Proteins c-kit