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  • 1
    Electronic Resource
    Electronic Resource
    Springer
    Pediatric surgery international 10 (1995), S. 459-464 
    ISSN: 1437-9813
    Keywords: Intestinal neuronal dysplasia ; Hirschsprung's disease ; Acetylcholinesterase staining ; Diagnostic criteria
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract The incidence of isolated intestinal neuronal dysplasia (IND) has varied from 0.3% to 62% of all suction rectal biopsies in different centres. The uncertainty regarding the incidence has resulted from the considerable confusion regarding the essential diagnostic criteria. In an attempt to clarify the diagnostic criteria for IND, we examined biopsy material from the following three groups using acetylcholinesterase (AChE) histochemistry: (1) full-thickness normal colon from 23 controls; (2) suction rectal biopsies from 9 patients who had isolated IND; and (3) full-thickness biopsies from 10 patients with Hirschsprung's disease (HD) who demonstrated IND in the proximal margin of the resected segment. Our data show that hyperganglionosis is the most consistent finding in both IND associated with HD and isolated IND. Other histochemical criteria of IND were dependent upon whether the biopsy was full-thickness or a suction rectal biopsy. Where full-thickness biopsies were available, giant ganglia and ectopic ganglion cells were seen in all cases. Increases in AChE-positive nerve fibres in the mucosa was a frequent finding in patients with IND diagnosed by suction rectal biopsies. We recommend that patients suspected to have IND on suction rectal biopsy should have a full-thickness biopsy for detailed examination of the submucous and myenteric plexuses.
    Type of Medium: Electronic Resource
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  • 2
    ISSN: 1437-9813
    Keywords: Hirschsprung's disease ; Enterocolitis ; Intercellular adhesion molecule-1 (ICAM-1)
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Intercellular adhesion molecule-1 (ICAM-1) is a glycoprotein that is necessary for the transendothelial migration of leucocytes. This study was undertaken to elucidate the role of ICAM-1 in the pathophysiology of Hirschsprung's disease (HD)-related enterocolitis. Ganglionic and aganglionic portions of bowel from 18 patients with HD who did not have clinical or histological evidence of enterocolitis and 5 patients with HD who developed enterocolitis before or after a pull-through operation were stained using monoclonal antibody against ICAM-1. The bowel specimens obtained from 2 children with imperforate anus at the time of colostomy closure and 3 children at the time of bladder augmentation were similarly stained to act as controls. The ganglionic portion of bowel from patients with HD without enterocolitis and controls showed either no ICAM-I staining or occasional staining of the endothelial lining of submucosal vessels with no staining of the glandular crypt epithelium. In contrast, both ganglionic and aganglionic bowel from patients with enterocolitis complicating HD demonstrated strong ICAM-1 staining in the endothelium of submucosal vessels. Strong expression of ICAM-1 in the glandular crypt epithelium was seen in only 2 patients who had developed enterocolitis before pull-through operations. This study illustrates the importance of endothelial cell activation in the pathogenesis of HD-related enterocolitis.
    Type of Medium: Electronic Resource
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