title: Neuronal immaturity in normoganglionic colon from cases of Hirschsprung disease, anorectal malformation, and idiopathic constipation.
abstract: OBJECTIVE
  Immaturity of neurons in normoganglionic colon in Hirschsprung disease (HD), anorectal malformation (AM), idiopathic constipation (IC), and normal controls (C) was assessed using polysialyated neural cell adhesion molecule.
  
  
  METHODS
  Polysialyated neural cell adhesion molecule immunoreactivity in 3 sections of normoganglionic colon from HD (n = 48), AM (n = 25), IC (n = 36), and C (n = 18) were scored semiquantitatively according to age; 1 day to 11 months (G1), 1 to 4 years (G2), and 5 years and older (G3).
  
  
  RESULTS
  Neurons in all specimens appeared mature irrespective of age on hematoxylin-eosin stain. Polysialyated neural cell adhesion molecule was positive (immaturity) in all specimens during G1 (1.34 in HD, 1.60 in AM, 0.89 in IC, and 1.59 in C) and decreased significantly with age in C (0.34* for G2, 0.25* for G3; *P < .01), decreased after 4 years old in IC (0.93 for G2, 0.10(#) for G3; (#)P < .05), decreased gradually in AM (1.10 for G2, 0.75( section sign) for G3; ( section sign)P < .05), but remained strongly positive in HD (1.34 for G1, 1.26 for G2, and 1.21 for G3; P = not significant), which after 4 years was significantly higher than C (P < .05).
  
  
  CONCLUSIONS
  Postoperative colonic dysmotility may be because of persistence of immature neurons in HD and impaired maturation of neurons in AM and IC.
authors.affiliation: Department of Pediatric General and Urogenital Surgery, Juntendo University School of Medicine, Tokyo 113-8421, Japan. katsuo@juntendo.ac.jp
authors.forename: Katsumi
authors.initials: K
authors.lastname: Miyahara
authors.forename: Yoshifumi
authors.initials: Y
authors.lastname: Kato
authors.forename: Tatsunori
authors.initials: T
authors.lastname: Seki
authors.forename: Atsushi
authors.initials: A
authors.lastname: Arakawa
authors.forename: Geoffrey J
authors.initials: GJ
authors.lastname: Lane
authors.forename: Atsuyuki
authors.initials: A
authors.lastname: Yamataka
mesh_terms: D017677:Age Distribution; D001003:Anal Canal / Q000002:abnormalities* / Q000378:metabolism* / Q000473:pathology; D002648:Child; D002675:Child, Preschool; D003106:Colon / Q000294:innervation* / Q000473:pathology; D003248:Constipation / Q000473:pathology*; D017615:Enteric Nervous System / Q000002:abnormalities / Q000473:pathology; D005769:Gastrointestinal Motility; D006627:Hirschsprung Disease / Q000473:pathology*; D006801:Humans; D007150:Immunohistochemistry; D007223:Infant; D007231:Infant, Newborn; D009130:Muscle, Smooth / Q000294:innervation / Q000473:pathology; D039842:Neural Cell Adhesion Molecule L1 / Q000276:immunology / Q000378:metabolism*; D055495:Neurogenesis / Q000502:physiology*; D009474:Neurons / Q000276:immunology / Q000378:metabolism* / Q000473:pathology*; D012007:Rectum / Q000002:abnormalities* / Q000378:metabolism / Q000473:pathology; D012794:Sialic Acids / Q000276:immunology / Q000378:metabolism*
chemical_list: D039842:Neural Cell Adhesion Molecule L1; D012794:Sialic Acids; C103469:polysialyl neural cell adhesion molecule