Development of interstitial cells of Cajal in a full-term infant without an enteric nervous system

The relationship between the development of the enteric nervous system and interstitial cells of Cajal (ICC) in the human small intestine was investigated in a full-term infant who presented with intestinal pseudo-obstruction. Immunohistochemistry revealed absence of enteric nerves and ganglia but a...

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Veröffentlicht in:Gastroenterology (New York, N.Y. 1943) N.Y. 1943), 2001-02, Vol.120 (2), p.561-567
Hauptverfasser: Huizinga, Jan D., Berezin, Irene, Sircar, Kanishka, Hewlett, Bryan, Donnelly, Graeme, Bercik, Premysl, Ross, Catherine, Algofi, Talal, Fitzgerald, Peter, Der, Tara, Riddell, Robert H., Collins, Stephen M., Jacobson, Kevan
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container_title Gastroenterology (New York, N.Y. 1943)
container_volume 120
creator Huizinga, Jan D.
Berezin, Irene
Sircar, Kanishka
Hewlett, Bryan
Donnelly, Graeme
Bercik, Premysl
Ross, Catherine
Algofi, Talal
Fitzgerald, Peter
Der, Tara
Riddell, Robert H.
Collins, Stephen M.
Jacobson, Kevan
description The relationship between the development of the enteric nervous system and interstitial cells of Cajal (ICC) in the human small intestine was investigated in a full-term infant who presented with intestinal pseudo-obstruction. Immunohistochemistry revealed absence of enteric nerves and ganglia but abundant c-Kit immunoreactivity associated with Auerbach's plexus (ICC-AP). However, c-Kit immunoreactivity associated with the deep muscular plexus (ICC-DMP) and intermuscular ICC was absent. Electron microscopy showed ICC-AP with a normal ultrastructure; ICC-DMP were seen but were severely injured, suggesting degeneration. In vitro recording of intestinal muscle showed slow wave activity as well as response to cholinergic stimulation. Fluoroscopic examination of the small bowel showed a variety of motor patterns, including rhythmic, propagating contractions. In conclusion, total absence of enteric nerves was associated with absence of normal ICC-DMP. However, a normal musculature, including a network of ICC-AP, allowed for generation of rhythmic, propagating contractile activity, suggesting the presence of functional motor activity. GASTROENTEROLOGY 2001;120:561-567
doi_str_mv 10.1053/gast.2001.21200
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Immunohistochemistry revealed absence of enteric nerves and ganglia but abundant c-Kit immunoreactivity associated with Auerbach's plexus (ICC-AP). However, c-Kit immunoreactivity associated with the deep muscular plexus (ICC-DMP) and intermuscular ICC was absent. Electron microscopy showed ICC-AP with a normal ultrastructure; ICC-DMP were seen but were severely injured, suggesting degeneration. In vitro recording of intestinal muscle showed slow wave activity as well as response to cholinergic stimulation. Fluoroscopic examination of the small bowel showed a variety of motor patterns, including rhythmic, propagating contractions. In conclusion, total absence of enteric nerves was associated with absence of normal ICC-DMP. However, a normal musculature, including a network of ICC-AP, allowed for generation of rhythmic, propagating contractile activity, suggesting the presence of functional motor activity. 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subjects Biological and medical sciences
Biological Clocks - physiology
Digestive system
Enteric Nervous System - abnormalities
Enteric Nervous System - ultrastructure
Fatal Outcome
Gastric Emptying - physiology
Gastrointestinal Motility - physiology
Humans
Infant, Newborn
Intestine, Small - cytology
Intestine, Small - embryology
Intestine, Small - innervation
Investigative techniques, diagnostic techniques (general aspects)
Male
Medical sciences
Membrane Potentials - physiology
Microscopy, Electron
Motor Neurons - physiology
Muscle, Smooth - innervation
Muscle, Smooth - physiology
Muscle, Smooth - ultrastructure
Pathology. Cytology. Biochemistry. Spectrometry. Miscellaneous investigative techniques
title Development of interstitial cells of Cajal in a full-term infant without an enteric nervous system
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