Distribution Of Connexin 43 And Interstitial Cells Of Cajal In Hirschsprung's Disease Bowels

Background and ObjectiveHirschsprung's disease is a gastrointestinal disease,which is pathologically characterized as the de ficiency of ganglion cells in colon or intestinum rectum. The affection intestines are short of motion and then cause obstruction. HD is a common cause of a disease low-set bowel obstruction in neonatal period.Its etiopathogenisis has not been elucidated yet.Most researchers presume it as a result of heredity and envirment.Interstitial cells of Cajal (ICC) are widely distributed throughout the gastrointestinal tract, which are believed to have a crucial role in gastrointestinal tissues by generating and propagating electrical slow waves to gastrointestinal muscles and/or mediating signals from the enteric nervous system. Recent studies have indicated that the abnormal distribution, the loss or decrease of ICC and the changes of their ultrastructure might play an important role in several disorders of human gastrointestinal motility. Recent studies have indicated that the loss or decrease of ICC and the changes of their ultrastructure might influence the neurotransmission and the function of smooth mucle cell (SMC), and resulted in the dysfunction of human gastrointestinal motility.Gap junction(GJ) is a special membrane structure between two cells,which is arranged by connexins. GJ transmit the intercellular substances and the electrical signals,of which Connexin43 is main of human.The loss or decrease of Cx43 may result in multiple diseases.ObjectiveTo investigate the distribution of Connexin 43(Cx43) and interstitial cells of Cajal (ICC)in Hirschsprung's disease (HD)bowels and to explore its role in the pathogenesis of HD.Methods42 cases (male 33,female 9)aging from 2 month to 10 years of HD were included in this study using immunohistochemistry method.All cases were sporadic,Of which, common type was in 30 case,short segment type was in 12 case.ONE with Ileal atresia.All cases were diagnosed as HD using histopathological method. The other 5 tissues from patiets(aging from 1 month to 8 years, male 4,female1)with intussusception were used as controls.ResultsThere was no immunoreactivity of Cx43 and ICC in the muscle layers of aganglionic segment in HD,which had significant difference of Cx43 and ICC expressions in ganglionic segment of HD and normal bowels . Moderate immunostainning of Cx43 and ICC were observed at the migratory segment from patients with HD,which had significant difference of Cx43 expression in ganglionic and aganglionic segment of HD.Moderate to strong immunostainning of Cx43 and ICC was observed at the circular muscle and the region between the circular and longitudinal layer in ganglionic segment from patients with HD and in normal bowels.There was no significant difference of Cx43 and ICC expression in ganglionic segment of HD and normal bowels. ConclusionThe lack expression of Cx43 and ICC in the aganglionic bowel of HD and the destruction of the gap junctionin dicate that the impaired intercellular substance exchange .Intercellular communication between cells might partly be responsible for the motility dysfunction in HD.