Advances in Pediatric Surgery

"Neural cell adhesion molecule"

Original Articles

[English]

Comparative Nerve Distribution of the Pylorus in Infantile Hypertrophic Pyloric Stenosis: Gang Do Kim, Dae Hyun Joo, Yong Oon Yoo, Sung Hwan Park, Ki Ho Park, Jae Bok Park; J Korean Assoc Pediatr Surg 2002;8(1):23-27. Published online June 30, 2002; DOI: https://doi.org/10.13029/jkaps.2002.8.1.23

Abstract
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Infantile hypertrophic pyloric stenosis (IHPS) a common childhood disorders characterized by nonbilious projectile vomiting, an olive shaped mass in the right upper quadrant of the abdomen and visible gastric peristaltic wave in the upper abdomen. Its etiology and pathogenesis are not clear but abnormal nerve distribution of the pylorus has been postulated^2-6. We performed immunocytochemical staning to the pyloric muscle from 10 IHPS and 3 controls patients, utilizing specific monoclonal antibody to NCAM(neural cell adhesion molecule). In IHPS patients, the number of NCAM protein immunoreactive nerve fibers were less than that in normal subjects. Auerbach myenteric plexuse was well developed and interbundle nerve plexuse was present but nerve fibers supplying individual muscle cells in smooth muscle bundles were poorly developed. These results indicate reduction of innervation in smooth muscles in IHPS patients that possibly contributes to the pathogenesis of IHPS.

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[English]

Expression of Neural Cell Adhesion Molecule (NCAM) and Glial Cell Line-Derived Neurotrophic Factor (GDNF) in Aganglionic Bowel of Hirschsprung's Disease: Oh, Jung Tak , Han, Airi , Son, Suk Woo , Choi, Seung Hoon , Han, Seok Joo , Hwang, Eui Ho , Yang, Woo Ick; J Korean Assoc Pediatr Surg 2001;7(1):15-20.; DOI: https://doi.org/10.13029/jkaps.2001.7.1.15

Abstract
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The pathophysiology of Hirschsprung's disease (HD) is not fully understood, but recent studies have disclosed that neural cell adhesion molecule (NCAM) and glial cell line-derived neurotrophic factor (GDNF) play important roles in the formation of aganglionic bowel of Hirschsprung's disease. To evaluate the roles of NCAM and GDNF in HD, immunohistochemical analysis was performed using formalin-fixed and paraffin-embedded tissue sections. On the basis of the results, we tried to evaluate them as diagnostic markers. The specimens were obtained from 7 patients with HD who underwent modified Duhamel operation. The diagnosis was based on the clinical findings and the absence of ganglion cells in the nerve plexuses by routine microscopy. NCAM immunoreactivity was found in the nerve plexuses and scattered nerve fibers in the smooth muscle layers of ganglionic segments. In aganglionic segments, the number of NCAM positive nerve fibers in the smooth muscle layers was significantly reduced compared with ganglionic segments. In two cases the nerve plexuses in aganglionic segments, NCAM was negligible. The smooth muscle cells showed diffuse immunoreactivity for GDNF and the staining intensity was not different in the aganglionic and ganglionic segments. However, higher expression of GDNF in the nerve plexus of the ganglionic segments was noted comparing to aganglionic segments. These data suggest that both NCAM and GDNF may play important roles in pathogenesis of Hirschsprung's disease and immunohistochemical staining for NCAM can be used as an ancillary diagnostic tool for HD.