The enteric nervous system (ENS) develops from neural crest cells (NCCs) that enter the foregut and hindgut to become enteric neural-crest-derived cells (ENCCs). When these cells of neural crest origin fail to colonize the terminal hindgut, this aganglionic region becomes non-functional and results in a condition in humans known as Hirschsprung's disease (HSCR). One of the genes associated with...

BACKGROUND The primary pathology of Hirschsprung's disease (HD) is a congenital absence of ganglion cells in the caudal most gut. The spastic aganglionic bowel is often innervated by a network of hypertrophied nerve fibres. Recently, mutations of SOX10 have been identified in patients with HD but only in those with Waardenburg-Shah syndrome. AIMS To understand the molecular basis for the path...

Spencer NJ, Bayguinov P, Hennig GW, Park KJ, Lee H-T, Sanders KM, Smith TK. Activation of neural circuitry and Ca waves in longitudinal and circular muscle during CMMCs and the consequences of rectal aganglionosis in mice. Am J Physiol Gastrointest Liver Physiol 292: G546–G555, 2007. First published October 12, 2006; doi:10.1152/ajpgi.00352.2006.—In mammals that develop rectal aganglionosis, th...

In mammals that develop rectal aganglionosis, the aganglionic segment still exhibits spontaneous phasic contractions that contribute to dysmotility and pseudoobstruction in this region. However, almost nothing is known about the mechanisms that generate these myogenic contractions or the effects of aganglionosis on the generation of Ca(2+) waves that underlie contractions of the longitudinal mu...

OBJECTIVE Successful results after one-stage trans-anal pull-through (OSTAPT) operation for Hirschsprung's disease (HD) depend on accurate identification of the aganglionic segment in intra-operative frozen section (FS). Misinterpretation of the findings of the rectal biopsy is an anxiety-evoking pitfall for the surgeon. This study aims to describe our experiences in comparing results of FS and...

we report the case of a 54-year-old iranian man who presented with melena from three months prior to admission. esophagogastroduodenoscopy and colonoscopy were normal. double balloon enteroscopy showed a pedunculated tumor located in the jejunum. he underwent laparotomy and resection of a 9.5 cm tumor from the distal jejunum. histopathologic evaluation confirmed the diagnosis of an inflammatory...

the prevalence of various intestinal helminthiasis have been determined by the examination of 8109 inhabitants of 20 villages located in the mountainous and coastal regions of the bandar abbas and minab areas in southern iran. a portion of people infected with each parasite were treated with the appropriate drug i and the worms expelled were collected, identified and counted. the results indica...

background: acute obstruction of the colon continues to be a lethal disease with little improvement in the mortality rate over the past 50 years. so, this study was undertaken to define the causes of colon obstruction and their probable changes after years. â  methods: from 1979 to 2004, all records of patients with definite diagnosis of intestinal obstruction at all hospitals affiliated to shi...

BACKGROUND Hirschsprung's disease is one of the commonest causes of intestinal obstruction in neonates because of gut motility disorder. It is characterized as a complex genetic heterogenous disorder with variable inheritance. Hirschsprung's disease occurs as an isolated phenotype in majority (70 %) of cases. In other cases it may be associated with syndromes (such as Down's syndrome, Waardenbu...

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...