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A~ 7~ ~Acetylcholinesterase
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Abstract=
Evolutionary Patterns with Age of Acetylcholinesterase Activity of Rectal Suction Biopsies in Hirschsprung's Disease
Woo Hyun Park, M.D., Soon Ok Choi, M.D., Sang Pya Kim, M.D.,I) Eun Soak Chang, M.D.I), Tae Won Paik, M.D2)
Division of Pediatric Surgery, Departments of Surgery, Pathology'), Pediatrics'), Institute for Medical Science Keimyung University Dongsan Medical Center, Taegu, Korea
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 regardless 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 infiltraiton 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 excusively 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 sum- mary, 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 pc.ttern finally to the classical pattern.
Index Words: Acetylcholinesterase; Rectal suction biopsy; Hirschsprung's disease
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Fig. 1. Normal ACHE activity. There is no evidence of cholinergic fibers in the mucosa and submuco- sa. ACHE, counterstained with hematoxylin( x 100).
Fig. 2. Pattern I (newborn pattern). Thick ACHE positive cholinergic fibers distribute diffusely in the muscularis mucosae and submucosa, but the lamina propria is clear. ACHE, counterstained with hematoxylin( x 100).
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Fig. 3. Pattern IJJ (intermediate pattern). Thick and thin ACHE positive cholinergic fibers distribute in the muscularis mucosae and submucosa. Also noted is the presence of thin ACHE activity in the lamina propria. ACHE, counterstained with hema- toxylin( x 100).
Fig. 4. Pattern II (classical pattern). Thin ACHE positive fibers distribute diffusely In the muscularis mucosae, submucosa, and in the lami- na propria. ACHE, counterstained with hematox- ylin( x 100).
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Table 1. Analysis of Histochemical Patterns at the Time of Biopsies in 26 Cases of Hirsch·
sprung's Disease Age at the ti· Number of bio·
me of biopsies 1-30 days 2-6 months 7-12 months )1 year
Total
psies(N=37) 19 11 4 3 37 Male: Female = 20 : 6
* : negative ACHE pattern
ACHE pattern
I III II - *
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.!i!.Table 2. Evolution of Histochemical Patterns in 10 Cases of Hirschsprung's Disease
Case Name 1st Bx/Age 2nd Bx/Age Diagnosis
1 P1H 1/4 d Il/5 mo short segment HD
2 LHY 1/4 d Il/5 mo long segment HD
3 KJY 111/6 d IlI/5 rna short segment HD
4 PJ 1/7 d III /30 d. II /7 mo* short segment HD
5 C1J 1/7 d IIl/5 rna short segment HD
6 YHW 1/9 d JIl/8 rna short segment HD
7 YKY 1/10 d Il/4 mo short segment HD
8 KSH 1/12 d III/7 rno TCA
9 KBK 1/14 d I1I/5 rna V short segment HD
10 KJM Neg./21 d JIl/7 rna TeA
Abbreviations: HD, Hirschsprung's disease; TCA, total colonic aganglionosis ; Bx, biopsy
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