Topography and morphometry of intestinal mast cells in children with Hirschsprung's disease.

Abstract

Mast cells (MC) are source of many biological active compounds like cytokines, arachidonic acid derivates, proteoglicanes, prostaglandins, proteases, free oxygen radials, NGF, PAF and many more. The role of MC in pathogenesis of Hirschsprung's disease (HD) is not clear. Substances produced by MC may exert an important effect on embryology, growth, differentiation and regeneration of intestinal nervous system. Additionally, MC products modulate inflammation processes thus influencing on the clinical course of HD. Present study was established to evaluate the morphologic MC examination as a support of making diagnosis in HD. The MC topography and morphometry were evaluated in specimens collected from aganglionic colon of patients with diagnosed HD. The results were compared with corresponding data from normally innervated colon of patients suffering from constipation, and normal colon of children not presenting defecation problems. MC were visualized using indirect immunohistochemical method LSAB with mouse antibody against human tryptase. The MC visualized in submucosa and muscular layer in Hirschsprung's disease were significantly larger in comparison with control group (p<0.05). Also the number of MC/mm 2 in mucosa and lamina propria in HD was significantly elevated (p<0.05). However, the MC density in submucosa was also higher but it was not high statistically significant. In muscular layer and in serosa density of MC/mm 2 was not statistically significant. In the intestinal wall MC in aganglionic segment in Hirschsprung's disease are significantly activated comparing with normally innervated colon segments taken from the patients from other groups. This may confirm the role of MC both in pathogenesis of HD and in the reparation processes of bowel nervous system.

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

“…The HD cases showed a transmural distribution of these cells as described in the previous two studies by Kobayashi et al [5] and Demirbilek et al [6] . However, in a recent study Hermanowicz et al [7] found them to be increased in the mucosa and lamina propria but the increase in mast cells in the submucosa, muscularis propria and serosa was not statistically significantly changed. The mast cells were characteristically distributed around the nerves and blood vessels in addition to being randomly scattered.…”

“…There are only a couple of studies on mast cells in HD in the literature [5][6][7] . There is however no study from India on mast cells in children with HD or constipation.…”

Expression of phenotypic markers of mast cells, macrophages and dendritic cells in gallbladder mucosa with calculous cholecystitis

“…In gallbladders with acute cholecystitis, mean expression of tryptase was slightly higher than that in the CCC group, but the difference was insignificant. The IHC reaction for the marker was well visible and provided the typical "fried eggs" pattern of MCs [18]. No correlation was detected between number of MCs and inflammatory activity in the gallbladder.…”

“…TNF-α mRNA and protein were expressed constitutively in isolated human intestinal MCs [16]. Attempts were made to describe involvement of MCs both in physiology and in several inflammatory diseases and non-inflammatory diseases of the alimentary tract in humans [12,17,18], including pathology of gallbladder [19][20][21][22]. Higher numbers of MCs were detected in chronic exacerbated and chronic sclerotic cholangitis [21], and in Hirschsprung's disease [18], as compared with those in controls.…”

Mast Cells and Intestinal Motility Disorders (Mastocytic Enteritis/Colitis)

“…Since the control group (patients with non-obstructed colorectal cancers) did not have documented constipation, this question also remains unanswered. Increased MC has been reported in motility disorders such as Hirschsprung's disease and Chagas disease [26,27].…”