Deficiency of c-kit+ cells in patients with a myopathic form of chronic idiopathic intestinal pseudo-obstruction.
OBJECTIVE
Chronic idiopathic intestinal pseudo-obstruction (CIIP) is a syndrome characterized by a failure of intestinal movement, but the cause of dysmotility remains unknown. Because interstitial cells of Cajal (ICCs) are believed to initiate basic contractile activity of the gastrointestinal tract, there is a possibility that changes in ICCs are involved in the development of CIIP. ICCs express c-kit in mice, and it has been reported that the c-kit+ cells, the location and shape of which resemble those in mice, are detected in the human gastrointestinal muscular layer using immunohistochemistry. In the present study, we counted the number of c-kit+ cells in the affected intestine of two patients with myopathic form of CIIP and compared this number with the number of c-kit+ cells in the normal intestine.


METHODS
The c-kit+ cells in the external muscle layer were detected by immunohistochemistry, and the number of them was counted under the microscope. Mast cells, which are known to express c-kit, were detected by staining with Alcian blue, and the number of them was also counted.


RESULTS
Immunohistochemistry revealed that the distribution pattern of c-kit+ cells resembles that of ICCs in the external muscle layer of normal control subjects. The numbers of c-kit+ cells apart from mast cells in two patients with myopathic form of CIIP decreased to about 3% of those in normal subjects.


CONCLUSIONS
The failure of intestinal movement in patients with CIIP, at least in a subpopulation, might be related to a deficiency of c-kit+ cells, probably ICCs.
Isozaki
K
K
Second Department of Internal Medicine, Osaka University Medical School, Japan.
Hirota
S
S
Miyagawa
J
J
Taniguchi
M
M
Shinomura
Y
Y
Matsuzawa
Y
Y
D000328:Adult; D002452:Cell Count; D002908:Chronic Disease; D005260:Female; D006801:Humans; D007150:Immunohistochemistry; D007418:Intestinal Pseudo-Obstruction / Q000209:etiology / Q000378:metabolism* / Q000473:pathology*; D007421:Intestine, Small / Q000378:metabolism / Q000473:pathology; D008297:Male; D009130:Muscle, Smooth / Q000378:metabolism / Q000473:pathology*; D009197:Myenteric Plexus / Q000378:metabolism / Q000473:pathology; D019009:Proto-Oncogene Proteins c-kit / Q000378:metabolism*
D019009:Proto-Oncogene Proteins c-kit