Deficiency of c-kit+ cells in patients with a myopathic form of chronic idiopathic intestinal pseudo-obstruction

Am J Gastroenterol. 1997 Feb;92(2):332-4.


Objectives: 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.

Publication types

  • Case Reports
  • Comparative Study
  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Adult
  • Cell Count
  • Chronic Disease
  • Female
  • Humans
  • Immunohistochemistry
  • Intestinal Pseudo-Obstruction / etiology
  • Intestinal Pseudo-Obstruction / metabolism*
  • Intestinal Pseudo-Obstruction / pathology*
  • Intestine, Small / metabolism
  • Intestine, Small / pathology
  • Male
  • Muscle, Smooth / metabolism
  • Muscle, Smooth / pathology*
  • Myenteric Plexus / metabolism
  • Myenteric Plexus / pathology
  • Proto-Oncogene Proteins c-kit / metabolism*


  • Proto-Oncogene Proteins c-kit