Attenuated nitrergic inhibitory neurotransmission to interstitialcells of Cajal in the lower esophageal sphincter with esophagealachalasia in children

• Published in: Pediatrics international 2002-04, Vol.44 (2), p.145-148
• Main Authors: Watanabe, Yoshio, Ando, Hisami, Seo, Takahiko, Katsuno, Shinsuke, Marui, Yuji, Ono, Yasuyuki, Torihashi, Shigeko
• Format: Article
• Language: English
• ISSN: 1328-8067
• DOI: 10.1046/j.1442-200x.2002.01518.x

Background: Esophageal achalasia (EA) is a raredisease in children, the etiology and pathogenesis of which remaincontroversial. Previous studies have suggested that a specific classof interstitial cells of Cajal (ICC) act as mediators in nitrergicinhibitory neurotransmission in the lower esophageal sphincter (LES).The aim of this investigation is to clarify the status of ICC andnitrergic inhibitory neurons in the LES of EA using immunohistochemistry.Methods: Specimens were obtained from two patientswith EA (aged 6 and 10 years) and two patients with esophageal carcinoma(aged 56 and 63years) not involving the lower esophagusas controls. Immunohistochemistry was used to study the distributionof ICC and nitrergic inhibitory neuron.Results: The LES contains the c-kit positiveICC in the muscle layers, which form close relationships with nitricoxide synthase (NOS)-containing nerve fibers in the controls. Thedistribution of ICC was almost the same between samples with EAand controls. However, such nerve fibers were absent in EA witha longer duration of the symptoms, but were reduced in a shorterduration. Conclusions: Decreased nitrergic inhibitory neurotransmissionto ICC in LES is a possible cause of sphincter achalasia in pediatricpatients with EA. The decrease in NOS-positive neurons of patientswith achalasia may be gradual, which may account for the long durationof symptoms prior to treatments. Further advancement of esophagealmotility damage was suspected in pediatric EA.