Microbiological studies of the enterocolitis of Hirschsprung's disease.

Abstract

The results of a prospective study of 20 cases of newly diagnosed Hirschsprung's disease (nine of whom developed enterocolitis) and 10 normal controls showed no variations in the bacterial flora (including Clostridium difficile) in the stools of the groups studied. Viral studies showed that rotavirus was present in the stools of seven of the nine cases of enterocolitis during the episode. We suggest that Hirschsprung's enterocolitis may have a complex infective aetiology and that rotavirus plays a part.

New insights into the pathogenesis of Hirschsprung’s associated enterocolitis

“…Of note, there were no symptoms of vomiting in these patients which is pathognomonic of rotavirus gastroenteritis. Also, there was no evidence of contact before, during or after admission to the hospital [60]. However, these results have not been replicated.…”

“…However, this has not been proven on subsequent investigations; 50% of patients with HD have C. difficile and there was no variation in incidence between those pre-and postoperative periods [59]. Wilson-Storey et al [60] in 1990 demonstrated a broad spectrum of organisms present in the stools with no significant difference in the clostridium carriage rate between those with HAEC and without enterocolitis or normal controls. Stool samples in our centre reveal a wide range of colonic flora present during episodes of HAEC.…”

“…Rotavirus has been identified in seven of nine patients with enterocolitis [60]. Of note, there were no symptoms of vomiting in these patients which is pathognomonic of rotavirus gastroenteritis.…”

The ITGB2 immunomodulatory gene (CD18), enterocolitis, and Hirschsprung's disease

“…The etiology of HAEC remains obscure, although current hypotheses include possible immune impairment [10,14,29], mucosal ischemia related to the mechanical intestinal wall distention [7], viral opportunistic pathogens such as Rota virus [8], and Clostridium difficile infection. In addition, proximal disorders of enervation [11,12], abnormal mucin production [13,30], increased prostaglandin E 1 activity [31], or enzyme (eg, sucrase-isomatase) deficiency [32] are other reported possibilities.…”

“…Essentially, it results from disruption of normal signaling during development because of several genetic variations (on at least 9 genes), determining its phenotypic expression [5,6]. The etiology of HAEC remains obscure despite considerable research [7][8][9][10][11][12], but current evidence suggests a complex interaction of genetic and environmental factors, with underlying factors suggesting impairment of local defense mechanisms [13] and the immune system [3,14].…”