Publication: Hirschsprung's disease - immunohistochemical findings
Authors
Larsson, L.T.
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Publisher
Murcia : F. Hernández
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DOI
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info:eu-repo/semantics/article
Description
Abstract
Hirschsprung's disease (HSCR) is
characterized by a non-propulsive distal intestinal
segment (usually colon) leading to a functional
obstruction. An absence of ganglia in the affected - - segment explains the synonymous term ccaganglionosis
coli,,. The lack of peristalsis is partly due to a deficient
intestinal smooth muscle relaxation based on an
absence of non-adrenergic, non-cholinergic (NANC)
inhibitory innervation. Morphological studies
using conventional microscopy, imrnunohistochemistry
and immunochemistry against general
neuronal markers and neuropeptides have been used
to characterize the disturbed NANC innervation in
HSCR.
An increased cholinergic and adrenergic innervation
is registered in the aganglionic segment in spite of
the lack of neuronal cell bodies: Neuropeptides
like vasoactive intestinal peptide (VIP), pituitary
adenylate cyclase-activating polypeptide (PACAP),
gastrin-releasing peptide (GRP), calcitonin generelated
peptide (CGRP), substance P (SP), enkephalins
and galanin imrnunoreactive nerve fibres are all
reduced in number i n the aganglionic segment.
In contrast, neuropeptide Y (NPY)-containing nerve
fibres are increased in number in the diseased
segment, probably reflecting the adrenergic hyperinnervation.
General neuronal markers including
chromogranins have been used to map the ne~lron~l
network in the HSCR intestine and also to
investigate the endocrine cell system in the intestinal
mucosa. Nitric oxide is a potent component of the
NANC inhibitory innervation and its synthesizing
enzyme, nitric oxide synthase (NOS). is shown to be
almost absent in the neuronal system in aganglionic
intestine.
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