PMID- 8801317
OWN - NLM
STAT- MEDLINE
DCOM- 19961002
LR  - 20190710
IS  - 0022-3468 (Print)
IS  - 0022-3468 (Linking)
VI  - 31
IP  - 4
DP  - 1996 Apr
TI  - Is intestinal neuronal dysplasia a disorder of the neuromuscular junction?
PG  - 575-9
AB  - Intestinal neuronal dysplasia (IND) is a malformation of the enteric plexus and 
      clinically resembles Hirschsprung's disease. The pathogenesis of IND is not 
      known. There is no information available regarding the nature of the 
      neuromuscular junction in this disease. The authors examined 
      immunohistochemically full-thickness biopsy specimens from 14 patients (aged 3 
      weeks to 26 months) with IND and 10 age-matched controls, using monoclonal 
      antibodies to growth associated protein-43 (GAP-43), synaptophysin, and 
      neural-cell adhesion molecule (NCAM) as neuromuscular junction markers. In the 
      normal bowel, GAP-43, synaptophysin, and NCAM immunoreactivity was abundant in 
      the submucous and myenteric plexuses as well as in the muscularis mucosae and 
      circular and longitudinal muscle layers. However, of the biopsy specimens from 
      patients with IND, five had absence of GAP-43, synaptophysin, and NCAM 
      immunoreactivity in muscularis muscosae and the circular and longitudinal muscle 
      layers; five had no immunoreactivity in the longitudinal muscle; and four had no 
      immunoreactivity in the muscularis mucosae. The submucous and myenteric plexuses 
      of all patients with IND displayed strong immunoreactivity for GAP-43, 
      synaptophysin, and NCAM. The findings demonstrate that patients with IND have 
      defective innervation of the neuromuscular junction of the affected bowel.
FAU - Kobayashi, H
AU  - Kobayashi H
AD  - Children's Research Centre, Our Lady's Hospital for Sick Children, Dublin, 
      Ireland.
FAU - Hirakawa, H
AU  - Hirakawa H
FAU - Puri, P
AU  - Puri P
LA  - eng
PT  - Journal Article
PL  - United States
TA  - J Pediatr Surg
JT  - Journal of pediatric surgery
JID - 0052631
RN  - 0 (Biomarkers)
RN  - 0 (GAP-43 Protein)
RN  - 0 (Membrane Glycoproteins)
RN  - 0 (Nerve Tissue Proteins)
RN  - 0 (Neural Cell Adhesion Molecules)
RN  - 0 (Synaptophysin)
SB  - IM
MH  - Biomarkers/analysis
MH  - Biopsy
MH  - Child, Preschool
MH  - Female
MH  - GAP-43 Protein
MH  - Hirschsprung Disease/*pathology/surgery
MH  - Humans
MH  - Immunoenzyme Techniques
MH  - Infant
MH  - Infant, Newborn
MH  - Male
MH  - Membrane Glycoproteins/analysis
MH  - Myenteric Plexus/*pathology/surgery
MH  - Nerve Tissue Proteins/analysis
MH  - Neural Cell Adhesion Molecules/analysis
MH  - Neuromuscular Junction/*pathology
MH  - Submucous Plexus/*pathology/surgery
MH  - Synaptophysin/analysis
EDAT- 1996/04/01 00:00
MHDA- 1996/04/01 00:01
CRDT- 1996/04/01 00:00
PHST- 1996/04/01 00:00 [pubmed]
PHST- 1996/04/01 00:01 [medline]
PHST- 1996/04/01 00:00 [entrez]
AID - S0022-3468(96)90500-X [pii]
AID - 10.1016/s0022-3468(96)90500-x [doi]
PST - ppublish
SO  - J Pediatr Surg. 1996 Apr;31(4):575-9. doi: 10.1016/s0022-3468(96)90500-x.