Severe gastrointestinal dysmotility in a patient with congenital myopathy: causal relationship to decrease of interstitial cells of Cajal.
Interstitial cells of Cajal (ICC) are known to be essential regulators of gastrointestinal (GI) motility. Here, we report the clinical course and abnormalities of intestinal ICC distribution in a 5-year-old patient with congenital fiber type disproportion myopathy who demonstrated long-term GI dismotility. Full thickness biopsies of the small intestine and colon showed a normal enteric muscle layer and myenteric plexus. However, the density of ICC was strikingly decreased around the myenteric plexus compared to that in autopsied cases without GI tract disease. These findings suggest that a decline in ICC may contribute to disturbed GI motility in our patient with congenital myopathy.
Kubota
Masaya
M
Department of Pediatrics, University of Tokyo, 7-3-1 Hongo, Bunkyo-ku, Tokyo 113-8655, Japan. mkmegped@opal.plala.or.jp
Kanda
Etsuko
E
Ida
Kohmei
K
Sakakihara
Yoichi
Y
Hayashi
Masaharu
M
D002675:Child, Preschool; D003106:Colon / Q000294:innervation / Q000473:pathology / Q000503:physiopathology; D005260:Female; D005769:Gastrointestinal Motility; D006801:Humans; D007421:Intestine, Small / Q000294:innervation / Q000473:pathology / Q000503:physiopathology; D009135:Muscular Diseases / Q000473:pathology* / Q000503:physiopathology*; D009197:Myenteric Plexus / Q000473:pathology* / Q000503:physiopathology*; D012720:Severity of Illness Index