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Morphometric evaluation of PGP9.5 and NCAM expressing nerve fibers in colonic muscle of patients with Hirschsprung's disease

 Jung Tak Oh  ;  Ai Ri Han  ;  Woo Ick Yang  ;  Seok Joo Han  ;  Seung Hoon Choi  ;  Eui Ho Hwang 
 YONSEI MEDICAL JOURNAL, Vol.43(1) : 31-36, 2002 
Journal Title
Issue Date
Hirschsprung's disease ; enteric nervous system ; immunohistochemistry ; morphometry ; PGP9.5 ; NCAM ; NGFR
A quantitative assessment of the density of the protein gene product 9.5 (PGP9.5), the neural cell adhesion molecule (NCAM), and the low-affinity nerve growth factor receptor (NGFR) expressing nerve fibers in the circular muscle layer in the colon was carried out by morphometric analyses from 13 patients with Hirschsprung's disease (HD). The difference in the nerve fiber density between the ganglionic and aganglionic segments was compared by calculating the ratio of the sum of the areas occupied by positively stained nerve fibers per unit area of the muscle after immunohistochemical staining on paraffin embedded tissue sections using computer software. There was an obvious difference in the density of the PGP9.5 stained nerve fibers between the ganglionic (0.0380 ± 0.0171) and aganglionic segments (0.0143 ± 0.01661). The NCAM-positive nerve fibers were fewer in number than those of both the PGP9.5-positive fibers and NCAM-positive fibers, which were also markedly lower in number in the aganglionic segment (0.0066 ± 0.0076) than in the ganglionic segment (0.0230 ± 0.0195). Immunostaining for low-affinity NGFR revealed much fainter staining in the ganglionic and aganglionic segment without a statistically significant difference in their density. Considering the fact that PGP9.5 is a very sensitive marker for nerve fibers, the results of this study reaffirm the innervation failure of the proper muscle in HD. The decreased NCAM expression level in the aganglionic segment appears to be caused not by the selective down-regulation of NCAM expression among the nerve fibers but by a markedly reduced number of nerve fibers.
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1. College of Medicine (의과대학) > Dept. of Surgery (외과학교실) > 1. Journal Papers
Yonsei Authors
Oh, Jung Tak(오정탁)
Choi, Seung Hoon(최승훈)
Han, Seok Joo(한석주) ORCID logo https://orcid.org/0000-0001-5224-1437
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