Distribution of mast cells and its role in different phenotypes of Hirschsprung's disease

Abstract

Objective To explore the role of mast cells (MCs) in the pathogenesis of Hirschsprung's disease (HD) through examining the distribution of MC in different phenotypes of HD.Methods Twenty three hospitalized HD patients between March 2010 and September 2012 were recruited and another 13 undergoing colostomy for congenital anorectal malformations selected as control group.Based on medical history,they were divided into common type (n =12),long segment type (n=6) and total colon type groups (n =5).The MCs in colonic wall were stained by toluidine blue and immunofluorescence.And their numbers of MCs were counted microscopically in mucosal,submucosal and muscular layers.Results The numbers of MCs in mucosa (34.5 ± 7.2,42.6 ± 11.1,54.0± 4.6) and submucosa (21.8 ± 6.1,27.0 ± 6.5,37.2 ± 12.9) of aganglionic segments from common type,long-segment type and total colon type groups were all higher than those in mucosa (24.3 ± 8.3,24.9 ± 8.9,25.4 ±9.5,25.7 ± 11.1)and submucosa(13.8 ± 5.3,14.9 ± 6.9,13.6 ± 6.6,13.7 ± 6.2)from ganglionic segments and control group(P＜0.05).Through pairwise comparisons among aganglionic segments from common type,lon,segment type and total colon type groups,the numbers of MCs were different in both mucosa and submucosa of all groups(P＜0.05).Marked degranulation occurred.Conclusions The distributions of MCs vary in different phenotypes of HD.Thus MCs may play an important role in the pathogenesis of HD. Key words: Hirschsprung's disease; Mast cell; Pathogenesis