BACKGROUND/PURPOSE The purpose of this study was to review the authors' 25-year experience with redo pull-through procedures for Hirschsprung's disease including surgical technique and long-term outcome. METHODS From 1974 to now, over 325 patients with Hirschsprung's disease have been treated at C.S. Mott Children's Hospital. This includes 30 patients referred after an unsuccessful pull-throu...

The excision of the aganglionic segment of the recto-sigmoid colon proposed by Swenson is the logical outcome of the new conception of the aetiology of Hirschsprung's disease and I do not think it necessary to discuss it here any further. The operation which I shall describe constitutes but a technical variance of Swenson's operation and is not a new method; it has also some other applications ...

The aim is to present optimal ways of early diagnosis and tactics Hirschsprung's disease (HD) surgical correction in newborns young children based on literature data our own experience. Materials methods. Clinical observations optimization the treatment HD 58 infants over past 9 years have been carried out. All patients underwent a complex general physical examinations, including collection ana...

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...

Hirschsprung’s disease is a neuronal dysplasia of the hindgut, characterised by a loss of neurones, which aVects about 1 in 5000 live births. Genetic factors have been implicated in the aetiology of this disease in about 20% of cases and a dominant pattern of inheritance has been revealed in several families. 3 The pathogenesis of the aganglionosis is often attributed to a failure of migration ...

BACKGROUND & AIMS Hirschsprung disease (HSCR) is caused by failure of cells derived from the neural crest (NC) to colonize the distal bowel in early embryogenesis, resulting in absence of the enteric nervous system (ENS) and failure of intestinal transit postnatally. Treatment is by distal bowel resection, but neural cell replacement may be an alternative. We tested whether aneuronal (aganglion...

Single nucleotide polymorphisms (SNPs) of the coding regions of RET are associated with Hirschsprung’s disease (HSCR, aganglionic megacolon,). These SNPs, individually or combined, may act as a low penetrance susceptibility locus or/and be in linkage disequilibrium (LD) with another susceptibility locus located in RET regulatory regions. Because two RET promoter SNPs have been found associated ...

The terminal colon is aganglionic in mice lacking endothelin-3 or its receptor, endothelin B. To analyze the effects of endothelin-3/endothelin B on the differentiation of enteric neurons, E11-13 mouse gut was dissociated, and positive and negative immunoselection with antibodies to p75(NTR )were used to isolate neural crest- and non-crest-derived cells. mRNA encoding endothelin B was present i...

BACKGROUND Hirschsprung's disease (HSCR) is one the most common congenital intestinal disease. It leads to aganglionic megacolon in the early childhood. Several susceptibility genes have been identified : RET protooncogene and its ligand, glial cell derived neutrophic factor (GDNF), Sox 10, Endothelin-3 (EDN3) and its receptor B (EDNRB). EDNRB mutations are found in 5% of familial or sporadic H...

Background Rectal biopsy and its histopathological study with hematoxylin and eosin (HE) is the gold standard for Hirschsprung's disease (HD) diagnosis. However, there are some limitations in the diagnosis of ganglion cells in HE approach. Recently, it was reported that the utility of Calretinin is a reliable ancillary immunohistochemistry (IHC) test for HD diagn...