Evolutionary Patterns with Age of Acetylcholinesterase Activity of Rect al Suction Biopsies in Hirschsprung's Disease

Hirschsprung씨 질환에서 직장 흡인 생검의 Acetylcholinesterase 활성도의 연령에 따른 변화

The acetylcholinesterase(ACHE) activity in 37 biopsies from the patients with Hirschsprung's disease was analyzed for histochemical patterns according to age, and its evolutional behavior was also assessed. The histochemical criterion used for the diagnosis of Hirschsprung's disease was that of Chow, i.e., "the presence of many coarse discrete cholinergic fibers in the muscularis mucosae and in the immediately subjacent submucosa regard less of an infiltration of cholinergic fibers in the lamina propria." The acetylcholinesterase activity in Hirschsprung's disease was further classified into 3 patterns, advocated by de Brito and Maksoud : Pattern I-many thick fibers exclusively in the muscularis mucosae and submucosa(newborn pattern). Pattern II-many thin fibers in the muscularis mucosae and submucosa with a clear infiltration of cholinergic fibers in the lamina propria(classical pattern). Pattern III-an intermediate pattern showing morphological characteristics of the two patterns with predominance of one or the other. Of 37 biopsies, Pattern 1 was seen exclusively in 16 biopsies taken from the neonates. Pattern III was 11 of 37 biopsies and it was mainly seen in children between 2 to 12 months of age. Pattern II was seen in all 3 biopsies of older than 1 year of age and in 6 ones of 3 to 12 months of age. In summary, this study reinforces the impression that there is an evolutional character of the distribution and morphology of the cholinergic ACHE positive fiber with age. In other words, ACHE activity in Hirschsprung's disease appears to evolve with age from the newborn pattern to the intermediate pattern finally to the classical pattern.

HD 환아 26례에서 시행한 37번의 직장흡인생검을 ACHE 조직화학검사한 결과 신생아형으로 판명된 16례 전례가 생후 1달 이내 검사한 경우였다. 혼합형으로 판명된 11례 중 9례가 2개월에서 12개월 사이에 검사한 경우였으며 고전형으로 판명된 9례 중 생후 3개월 부터 12개월 사이에 검사한 경우가 6례 였으며, 나머지 3례는 모두 1년 이후에 검사한 경우였다. 위의 결과로 보아 ACHE 조직화학검사에서 콜린성 신경섬유의 형태와 분포가 시간이 지남에 따라 변화함을 알 수 있다. 다시 말해서 신생아시기에 점막근층 및 이의 직하부 점막하층에 산재한 비교적 굵은 콜린성 신경섬유가 나이가 많아 짐에 따라 점점 가늘어 지면서 고유판과 점막하층에 광범위하게 분포하게 된다. 따라서 연령에 따른 ACHE 조직화학검사양상의 변화를 알고 이를 HD 진단에 응용함으로 Meier-Ruge등의 전통적인 진단기준으로 야기되는 오류를 극복할 수 있으리라 생각된다.