Acetylcholinesterase in Hirschsprung's disease

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dc.contributor.authorMoore S.W.
dc.contributor.authorJohnson G.
dc.date.accessioned2011-05-15T16:03:45Z
dc.date.available2011-05-15T16:03:45Z
dc.date.issued2005
dc.description.abstractThe association between the congenital absence of colonic ganglion cells and an increased acetylcholinesterase (AChE) expression in the affected tissue is of diagnostic importance in Hirschsprung's disease (HSCR). Investigation of AChE's function in development may also help unravel some of the complex pathophysiology in HSCR. Normal nerves do not stain for AChE, but increased AChE expression is associated with the hypertrophied extrinsic nerve fibres of the aganglionic segment in HSCR. Although a high degree of histochemical diagnostic accuracy exists, results are not always uniform, and false positives and false negatives are reported. False negative results are primarily related to age, and an absence of AChE reaction does not exclude HSCR in neonates within the first 3 weeks after birth. AChE staining results may lack uniformity, resulting in a number of technical modifications that have been made to improve standardization of AChE staining. At least two distinct histological patterns are described, types A and B. The interpretation of increased AChE staining patterns in ganglionated bowel at the time of surgical pull-through remains a problem in patients with HSCR. The development of rapid staining techniques has helped to identify normal ganglionated bowel with greater certainty. The presence of fine AChE neurofibrils in the ganglionated segment has contributed to the debate surrounding intestinal neuronal dysplasia. Quantitative assay of cholinesterase activity confirms the pattern of histochemical staining. AChE is particularly increased in relation to butrylcholinesterase, with one molecular form, the G4 tetrameric form, predominating. It is likely that the raised levels of AChE in aganglionic tissue are the transcriptional consequence of the abnormalities in signalling molecules that characterize HSCR. Evidence suggests that this AChE is functioning in a nonenzymatic capacity to promote cell adhesion and differentiation and that the hypertrophied nerves and neurofibrils may be the result of this increased AChE expression. © Springer-Verlag 2005.
dc.description.versionReview
dc.identifier.citationPediatric Surgery International
dc.identifier.citation21
dc.identifier.citation4
dc.identifier.issn1790358
dc.identifier.other10.1007/s00383-005-1383-z
dc.identifier.urihttp://hdl.handle.net/10019.1/12771
dc.subjectacetylcholinesterase
dc.subjectcholinesterase
dc.subjectcell adhesion
dc.subjectcell differentiation
dc.subjectdiagnostic accuracy
dc.subjectdiagnostic test
dc.subjectenzyme activity
dc.subjectHirschsprung disease
dc.subjecthistochemistry
dc.subjecthuman
dc.subjectintestinal neuronal dysplasia
dc.subjectnerve cell
dc.subjectnerve fiber
dc.subjectneurofilament
dc.subjectnonhuman
dc.subjectpriority journal
dc.subjectprotein expression
dc.subjectprotein function
dc.subjectquantitative assay
dc.subjectreview
dc.subjectstaining
dc.subjectAcetylcholinesterase
dc.subjectFetus
dc.subjectGene Expression
dc.subjectHirschsprung Disease
dc.subjectHumans
dc.subjectImmunohistochemistry
dc.subjectMale
dc.subjectNeurofibrils
dc.titleAcetylcholinesterase in Hirschsprung's disease
dc.typeReview
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