Preface

Hirschsprung's disease is one of the most important and most fascinating diseases in paediatric surgery. Our understanding of Hirschsprung's disease is developing rapidly, not only in relation to its pathophysiology and the development of new surgical techniques, but especially in relation to new genetic findings. A first comprehensive description of the pathophysiology, clinical symptoms, diagnosis and therapy of Hirschsprung's disease was outlined in 1970 by Theodor Ehrenpreis, Professor of...

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Her autopsy demonstrated dilatation of the colon and upper rectum that ceased abruptly 2 inches from the anus. No anal stricture or stenosis was observed 14 . This may have represented an instance of low segment Hirschsprung's disease. While a number of other physicians reported instances of severe constipation and colon dilatation in children that eventually led to their demise, Harald Hirschsprung, a Danish pediatrician from Queen Louise Children's Hospital, Copenhagen, presented the...

Origin and Development of Neural Crest Derived Cells

The NC is located along the entire length of the body axis. Two groups of undifferentiated cells, derived from NCs, colonize the gut wall and migrate in craniocaudal and caudocranial directions. The embryonic NC arises in the neural tube, originating with the central nervous system, but NC cells detach from this tissue via reduction of cell-cell and cell-matrix adhesion. The epitheliomesenchymal transformation allows NC cells to migrate along pathways of defined routes to various tissues, where...

References

Adamson WAD, Aird I (1932) Megacolon evidence in favor of a neurogenic origin. Br J Surg 20 220-223 2. Adson A (1937) Hirschsprung's disease indications for and results obtained by sympathectomy. Surgery 1 859-877 3. Albanese CT, Jennings RW, Smith B, Bratton B, Harrison MR (1999) Perineal one-stage pull-through for Hirschsprung's disease. J Pediatr Surg 34 377-380 4. Aslam A, Spicer RD, Corfield AP (1998) Turnover of radioactive mucin precursors inn the colon of patients with Hirschsprung's...

The Effect of Laminin1 on Enteric Neuronal Development Depends on the Binding of its a1 Chain to LBP110

LBP110 is not an integrin, but is similar to a -amy-loid precursor protein 234 . The domain of laminin that binds to LBP110 contains an (IKVAV) sequence and is located on the laminin a1 chain, near its globular C-terminal end 235-237 . Expression of LBP110 by PC12 cells is down-regulated by transfection of the cells with an antisense amyloid precursor protein cDNA 234, 235 . The ability of NGF to induce neurite extension on a laminin-1 substrate is reduced in such antisense-treated PC12 cells....

Endothelin Signaling Pathway

The endothelins (EDN1, EDN2, and EDN3) are intercellular local messengers that act via the cell surface receptors, EDNRA and EDNRB 45 . EDN is initially produced as an inactive preproendothelin that undergoes two proteolytic steps to produce an active peptide. The first cleavage produces inactive big endothelins, and these are finally cleaved by a specific protease, endothelin-converting enzyme (ECE) to produce biologically active EDN 7, 16, 45 . EDN3 and EDNRB have a role in the migration and...