I do not intend to give an account of the changing philosophies concerning the aetiology and treatment of the disease which followed upon Hirschsprung's (1888) first presentation of it as an entity in 1886. It required about 60 years of vague or wrong ideas to find the key to our present knowledge. That happened when our attention was diverted from the characteristic megacolon and was focused o...

BACKGROUND & AIMS In Hirschsprung disease (HSCR), neural crest-derived progenitors (NCPs) fail to completely colonize the intestine so that the enteric nervous system (ENS) is absent from distal bowel. Despite removal of the aganglionic region, many HSCR patients suffer from residual intestinal dysmotility. To test the hypothesis that inappropriate lineage segregation of NCPs in proximal gangli...

OBJECTIVE Our aim was to correlate the pathological results and clinical response in patients who underwent botulinum toxin (BT) injection for obstructive symptoms (OS) after a pullthrough operation for Hirschsprung's disease (HD). METHODS Between August 2002 and February 2006, 16 of 107 HD patients (15%) were referred with persistent OS after pull-through (PT) operation in this center. They ...

Hirschsprung disease (HSCR) is a newborn colorectal disease characterized by an absence of ganglia in the distal gut. Hedgehog (Hh) and endothelin signaling serve important roles in gastrointestinal tract formation. Alterations in the signaling pathways disrupt the development of enteric neural crest cells (ENCCs). It is not known whether there is any coordination between these pathways in the ...

INTRODUCTION Hirschsprung's disease, or congenital megacolon,is a malformation characterised by the absence of ganglion cells in the distal bowel. Most often, the aganglionic segment includes the rectosigmoid, but it may extend proximally to variable length. In late years, significant improvements regarding the surgical treatment of Hirschsprung's disease were made, by the introduction of both ...

There is increasing interest in localizing nerves in the intestine, especially specific populations of nerves. At present, the usual histochemical marker for cholinergic nerves in tissue sections is acetylcholinesterase activity. However, such techniques are applicable only to frozen sections and have uncertain specificity. Choline acetyltransferase (ChAT) is also present in cholinergic nerves,...

The cases of three full-term infant girls with both Hirschsprung's disease (HD) and Ondine's curse (OC) are presented, and the relevant literature is reviewed. All three patients required endotracheal intubation and assisted ventilation during the first 24 hours of life because of respiratory distress. The diagnosis of HD was subsequently established at age 13, 16, and 20 days, respectively. Th...

I do not intend to give an account of the changing philosophies concerning the aetiology and treatment of the disease which followed upon Hirschsprung's (1888) first presentation of it as an entity in 1886. It required about 60 years of vague or wrong ideas to find the key to our present knowledge. That happened when our attention was diverted from the characteristic megacolon and was focused o...

OBJECTIVE Adult Hirschsprungs disease is a rare disease and frequently misdiagnosed as the long-standing refractory constipation. Almost all cases have short or ultra-short aganglionic segment of distal rectum. The clinical features are different from those in childhood when the diseased segment is long. Amongst the few successful operations that have been used to treat this condition, internal...

Hirschsprung's disease (HSCR) is characterized by aganglionosis from failure of neural crest cell (NCC) migration to the distal hindgut. Up to 40% of HSCR patients suffer Hirschsprung's-associated enterocolitis (HAEC), with an incidence that is unchanged from the pre-operative to the post-operative state. Recent reports indicate that signaling pathways involved in NCC migration may also be invo...