[Hirschsprung's disease: neurocristopathy of migration and cell differentiation].
Hirschsprung disease or aganglionosis coli, is a predominantly pediatric condition, in which there is an innervation defect of the colon, manifested by chronic intermittent constipation alternating with explosive evacuations. The involved colonic segment is unable to relax and acts as an area of obstruction, which causes the proximal segment to dilate enormously. The diagnosis requires participation from several specialties, including pediatric surgery, gastroenterology, radiology and anatomic pathology. In recent years, significant progress in relevant knowledge of this area has been obtained, in particular about defective migration of the neural crest-derived colonic ganglion cell precursors, as well as the genetic aspects of the disease. This article reviews the diagnostic anatomic pathology and the most relevant points about the biology of Hirschsprung disease.
Reyes Múgica
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Departamentos de Patología y Pediatría, Esquela de Medicina, Universidad Yale New Haven, CT 06520-8023, USA. Miguel.Reyes@Yale.Edu
D001706:Biopsy; D002454:Cell Differentiation; D003106:Colon / Q000473:pathology; D006627:Hirschsprung Disease / Q000175:diagnosis / Q000235:genetics / Q000473:pathology; D006801:Humans; D009432:Neural Crest / Q000473:pathology
Enfermedad de Hirschsprung: neurocristopatía de la migración y la diferenciación celular.