PMID- 12898161
OWN - NLM
STAT- MEDLINE
DCOM- 20040206
LR  - 20181113
IS  - 0179-0358 (Print)
IS  - 0179-0358 (Linking)
VI  - 19
IP  - 5
DP  - 2003 Jul
TI  - Abnormal vasculature in intestinal neuronal dysplasia.
PG  - 345-8
AB  - Intestinal neuronal dysplasia (IND) is an intestinal motility disorder, which 
      clinically resembles Hirschsprung's disease (HD). Adventitial fibromuscular 
      dysplasia (AFMD) consists of proliferation of smooth muscle cells and collagen 
      fibers in the adventitia of blood vessels. The purpose of this study was to 
      investigate vascular abnormalities in large bowel biopsies from patients with 
      isolated HD, IND associated with HD and isolated IND. Large bowel biopsies from 
      patients presenting with isolated HD ( n=23), IND associated with HD ( n=11), 
      isolated IND ( n=16) and normal bowel as controls ( n=6) were investigated using 
      acetylcholinesterase (AChE) histochemistry, van Gieson staining and alpha-smooth 
      muscle actin (alpha-SMA) immunohistochemistry. Increased AChE activity around 
      submucosal vessels was found in 9/16 (56%) cases with isolated IND, 3/11 (27%) 
      cases of IND associated with HD, 5/23 (21%) isolated HD cases and 0/6 controls. 
      AFMD was found in 10/16 (62%) of the isolated IND cases, 4/11 (362) of the cases 
      with IND associated with HD and 4/23 (17%) cases of HD without IND using van 
      Gieson staining. None of the control specimens revealed AFMD. Increased alpha-SMA 
      immunoreactivity filaments were demonstrated in the submucosal vessel wall in 
      9/16 (56%) of isolated IND and 2/11(18%) of IND associated with HD cases. Normal 
      alpha-SMA immunoreactivity around submucosal vessels was seen in isolated HD and 
      controls. Abnormal submucosal vasculature is a common histological finding in 
      isolated IND and IND associated with HD and may be a useful additional diagnostic 
      feature in these patients.
FAU - Rolle, Udo
AU  - Rolle U
AD  - Children's Research Centre, Our Lady's Hospital for Sick Children, University 
      College, Dublin 12, Ireland.
FAU - Piotrowska, Anna Piaseczna
AU  - Piotrowska AP
FAU - Puri, Prem
AU  - Puri P
LA  - eng
PT  - Journal Article
DEP - 20030724
PL  - Germany
TA  - Pediatr Surg Int
JT  - Pediatric surgery international
JID - 8609169
SB  - IM
MH  - Adolescent
MH  - Biopsy
MH  - Blood Vessels/abnormalities/pathology
MH  - Child
MH  - Child, Preschool
MH  - Colon/*blood supply/*pathology
MH  - Fibromuscular Dysplasia/complications/*pathology
MH  - Hirschsprung Disease/complications/*pathology
MH  - Humans
MH  - Infant
MH  - Intestinal Diseases/complications/*pathology
EDAT- 2003/08/05 05:00
MHDA- 2004/02/10 05:00
CRDT- 2003/08/05 05:00
PHST- 2002/12/10 00:00 [accepted]
PHST- 2003/08/05 05:00 [pubmed]
PHST- 2004/02/10 05:00 [medline]
PHST- 2003/08/05 05:00 [entrez]
AID - 10.1007/s00383-003-1008-3 [doi]
PST - ppublish
SO  - Pediatr Surg Int. 2003 Jul;19(5):345-8. doi: 10.1007/s00383-003-1008-3. Epub 2003 
      Jul 24.