Colon aganglionosis

Careskey JM, Weber TR, Grosfeld JL Total colonic aganglionosis. Analysis of 16 cases. Am JSurg 1982 143 160-168. 

PK1 has recently been detected in the mucosa/mesenchyme of mouse embryonic gut. It modulates both proliferation and differentiation of enteric neural crest cells (NCC) during enteric nervous system development, which eventually contribute to the formation of the myenteric and submucosal enteric plexus of the bowel. Hence, PK1 may be useful to patients exhibiting congenital colonic aganglionosis also known as Hirschprung disease (Ngan et al., 2007, 2008). 

Hahn H, Hoepner F, Kalle T, et al. (1997) Appendicitis in childhood (German). Radiologe 37 454-458 Hajivassiliou CA (2003) Intestinal obstruction in neonatal/ pediatric surgery. Semin Pediatr Surg 12 241-253 Hayakawa K, Hamanaka Y, Suzuki M et al (2003) Radiological findings in total colon aganglionosis and allied disorders. Radiat Med 21 128-134... 

Solari V, Piotrowska AP, Puri P (2003) Histopathological differences between recto-sigmoid Hirschsprung s disease and total colonic aganglionosis. Pediatr Surg Int 19 349-354... 

Ultra Short Segment Hirschsprung s Disease and Total Colonic Aganglionosis 206 Imaging in Functional Constipation 207... 

Overall there is an incidence of approximately 1 in 5000 to 1 in 8000 live births. Occurrence is usually sporadic but is reported to be familial in 4%. There is an increased incidence in children with Down syndrome. Presentation is most common in the full term infant during the first 6 weeks of life (70%-80%) and is extremely rare in premature infants. In classic Hirschsprung s disease and in ultra-short segment disease there is a male-to-female ratio of 3 1 and in total colonic aganglionosis the ratio is 1.8 1. 

Note Constipation may be defined as the passage of excessively dry stools, infrequent stools, or stools of insufficient size. Constipation is a symptom and not a disease. It may be of brief duration (e.g., when one s living habits or diet change abruptly), or it may be a hfe-long problem, as occurs in congenital aganglionosis of the colon (Hirschsprung s disease). 

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