The loss of calretinin expression indicates aganglionosis in Hirschsprung's disease.
نویسندگان
چکیده
BACKGROUND Hirschsprung's disease (HD) is a congenital disorder characterised by the absence of ganglion cells in the large bowel, leading to functional obstruction and colonic dilatation proximal to the affected segment. A subclass of nerve cell bodies in both submucosa and myenteric ganglia of the human gastrointestinal tract were found to show immunopositivity for calretinin, a calcium binding protein, which plays an important role in the organisation and functioning of the central nervous system. AIM To investigate calretinin immunostaining in ganglionic and aganglionic HD colon specimens, and compare it with staining for S100, neurone specific enolase, and c-kit. METHODS Ten large bowel, full thickness specimens from patients with classic rectosigmoid HD were selected from the pathology repository. In total, 54 paraffin wax blocks-24 from the ganglionic zone, 17 from the aganglionic zone, and 13 from the transitional zone-were processed. RESULTS Calretinin was not expressed in aganglionic segments of HD and associated nerve fibres, whereas in ganglionic HD segments and in normal colon both ganglion cells and nerve fibres were immunopositive. In addition, c-kit showed an altered distribution in the interstitial cells of Cajal. The transitional zone showed a broad spectrum of histomorphological and immunohistochemical patterns of both calretinin and c-kit expression. CONCLUSION The absence of calretinin expression may serve as a diagnostic aid in identifying aganglionic segments in HD.
منابع مشابه
Diagnostic Value of Calretinin and S100 Immunohistochemistry in Hirschsprung's Disease
Background Rectal biopsy and its histopathological study with hematoxylin and eosin (HE) is the gold standard for Hirschsprung's disease (HD) diagnosis. However, there are some limitations in the diagnosis of ganglion cells in HE approach. Recently, it was reported that the utility of Calretinin is a reliable ancillary immunohistochemistry (IHC) test for HD diagn...
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BACKGROUND Hirschsprung's disease (HD) is a congenital intestinal motility disorder with absence of ganglion cells in the colonic wall. Diagnosis of the disease is mainly based on the identification of the lack of ganglion cells in the pathology sections of the colon which is very difficult and time consuming and also needs several serial cut sections. There are many proposed markers in this fi...
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Segmental aganglionosis Hirschsprung's is an extremely rare condition. Described as a segment of normally ganglionated bowel surrounded proximally and distally by aganglionosis, zonal aganglionosis is of interest because it may cause confusion in interpreting surgical margins. Diagnosis of segmental lesions in Hirschsprung's disease may be missed as it is rarely suspected at initial surgery. We...
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ورودعنوان ژورنال:
- Journal of clinical pathology
دوره 57 7 شماره
صفحات -
تاریخ انتشار 2004