Phenotypic alteration of interstitial cells of Cajal in idiopathic sigmoid megacolon.
BACKGROUND
Interstitial cells of Cajal (ICCs) are detected as a pacemaker of gastrointestinal movement and express c-kit and CD34. Recently, ICCs have implicated pathogenesis in several human diseases presenting gastrointestinal motor dysfunction. This study was performed to clarify the role of ICCs in idiopathic sigmoid megacolon using histological and immunohistochemical examinations.


METHODS
Four adult patients with idiopathic sigmoid megacolon and 11 controls were studied. Histology and immunocytochemistry using NSE, S100, c-kit, and CD34 were performed in conjunction with quantitative analysis using the public domain NIH image program.


RESULTS
Little histological change in neuromuscular structures in megacolon was observed. Immunohistochemistry demonstrated remarkable decrease of c-kit expressing ICCs without reduction of CD34 expression in the similar interstitial cell population. This observation was further supported by quantitative assessment using public domain NIH image program.


CONCLUSIONS
A specific downregulation of c-kit in ICCs may be a cause of idiopathic sigmoid megacolon in adults.
Adachi
Yasushi
Y
Internal Medicine, Sapporo Shirakabadai Hospital, 2-18 Higashi Tsukisamu, Sapporo, 062-0052, Japan.
Ishii
Yoshifumi
Y
Yoshimoto
Mitsuru
M
Yoshida
Yukinari
Y
Endo
Takao
T
Yamamoto
Hiroyuki
H
Akashi
Hirofumi
H
Imai
Kohzoh
K
Shinomura
Yasuhisa
Y
Kato
Yasuo
Y
D000368:Aged; D018952:Antigens, CD34 / Q000096:biosynthesis*; D003113:Colonoscopy; D003937:Diagnosis, Differential; D017615:Enteric Nervous System / Q000378:metabolism* / Q000473:pathology; D005260:Female; D006801:Humans; D007150:Immunohistochemistry; D008297:Male; D008531:Megacolon / Q000378:metabolism* / Q000473:pathology; D008875:Middle Aged; D010641:Phenotype; D019009:Proto-Oncogene Proteins c-kit / Q000096:biosynthesis*; D012810:Sigmoid Diseases / Q000378:metabolism* / Q000473:pathology
D018952:Antigens, CD34; D019009:Proto-Oncogene Proteins c-kit