Hirschsprung’s disease: Is there a relationship between mast cells and nerve fibers?

Abstract

AIM:To define the topography of mast cells and their numbers in cases of Hirschsprung's disease (HD) and non-HD, assess neural hypertrophy using imaging software and to study the relationship between mast cells and nerve fibers. METHODS:HE stained sections of 32 cases of chronic constipation in the age group of 0-14 years were reviewed for ganglion cells. AChE staining was performed on frozen sections of colonic and rectal biopsies. Based on their findings cases were divided into HD and non-HD and mast cells stained by toluidine blue were evaluated. Image analysis by computerized software was applied to S-100 stained sections for assessment of neural hypertrophy. RESULTS:Difference between number of mast cells in HD group (mean = 36.44) and in non-HD group (mean = 14.79) was statistically significant. Image analysis morphometry on S-100 stained sections served as a useful adjunct. The difference between number, size, and perimeter of the nerve fibers between HD and non-HD group was statistically significant. CONCLUSION:Mast cells are significantly increased in HD and their base line values are much higher in Indian children than that reported in Western literature. Their role in HD needs further research. Morphometry of S-100 stained nerve fibers is a useful adjunct to conventional methods for diagnosis of HD.

Colonic mast cells in controls and slow transit constipation patients

“…However, MC have been less extensively studied in patients complaining of constipation. Apart from sporadic observations of an increased number of colonic mucosal MC in constipation‐predominant IBS patients, 38 the only available studies in constipated patients were obtained in chagasic patients 10–12 and in those with Hirschsprung’s disease 13–16 . Concerning Chagas’ disease, the increase of MC found in these subjects has been put in relation to the increased fibrosis found in the colon of these patients, 11 whereas in Hirschsprung’s disease this increase has been hypothesised as a possible mechanism of ENS reparation 15 …”

“…However, MC could also play some role in other pathological conditions characterised by constipation as expression of an abnormal motility of the colon. For instance, it has been shown that patients with megacolon because of Chagas’s disease display an increased number of MC in the colon 10–12 and the same is true for patients with Hirschsprung’s disease 13–16 …”

Image Analysis Tools for Evaluation of Microscopic Views of Immunohistochemically Stained Specimen in Medical Research–a Review

“…Yadav et al [19] report assessment of neural hypertrophy using Scion image analysis software. Images of relevant areas were analyzed and the size and perimeter of the thickest nerve fiber in the submucosa were measured, the number of nerve fibers was counted.…”

The role of mast cells in pediatric gastrointestinal disease

“…Despite extensive research, the pathogenesis of the disease is not fully understood. There have been reports suggesting that MCs may play a role in the development of HD [58,60-62].…”