J Korean Assoc Pediatr Surg.  2013 Dec;19(2):122-129. 10.13029/jkaps.2013.19.2.122.

Prevalence and Significance of Immature Ganglion Cell in Hirschsprung's Disease

• Yang HB
• Kim HY
• Kim SH
• Jung SE
• Park KW

• KMID: 1961502
• DOI: http://doi.org/10.13029/jkaps.2013.19.2.122

Abstract

Immature ganglion cell (IGC) is known for its relationship with intestinal motility and its impact on postoperative functional outcomes of Hirschsprung's disease (HD). There are few studies on the relationship between intestinal dysmotility and IGC in HD patients. 67 patients pathologically diagnosed with HD and who received definitive operation in Seoul National University Children's Hospital from 2010 to 2011 were included. 10 patients were excluded due to inadequate immunohistochemical staining results. The proximal end of resected ganglionic segment was evaluated with immunohistochemistry examination with MAP-2, a marker of ganglionic cells and bcl-2, a marker of IGCs The median age at operation was 155 (15-4678) day-old. 55 (96.5%) patients positive for bcl-2, were regarded as having IGC, and 2 (3.5%) patients positive for MAP-2 but negative for bcl-2, were regarded as having only mature ganglion cells. In the bcl-2 positive group, there were 7 patients (12.7%) with constipation, 15 patients (27.3%) with soiling, 3 patients (5.5%) with perianal excoriation and 6 patients (10.9%) with medication use. In bcl-2 negative group, intestinal dysmotility was not seen. There was no statistical significance in the two groups. Considering that HD is diagnosed at a young age, the rate of IGC present is very high and it might be inappropriate to relate IGC to functional outcome at young ages.

Keyword

Hirschsprung's disease; Immature ganglion cell; Prevalence; bcl-2

MeSH Terms

Constipation
Ganglion Cysts*
Gastrointestinal Motility
Hirschsprung Disease*
Humans
Immunohistochemistry
Prevalence*
Seoul
Soil
Soil

Figure

• 

  Fig. 1 H&E and Immunohistochemical Staining of Ganglion Cell. A: Ganglion Cell in H&E, B: Mature Ganglion Cell in MAP-2, C: Immature Ganglion Cell in bcl-2

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