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Evolution with age of the acetylcholinesterase activity in rectal suction biopsy in Hirschsprung's disease
Insights
Hirschsprung
Area of Science:
- Gastroenterology
- Pediatric Surgery
- Histopathology
Background:
- Hirschsprung's disease is a congenital disorder characterized by the absence of ganglion cells in the distal bowel.
- Acetylcholinesterase (AChE) activity is a key histochemical marker used in diagnosing Hirschsprung's disease.
- Understanding the age-related patterns of AChE activity can aid in diagnosis and assessing disease progression.
Purpose of the Study:
- To analyze the histochemical patterns of acetylcholinesterase (AChE) activity in Hirschsprung's disease based on patient age.
- To evaluate the evolutional nature of AChE activity in Hirschsprung's disease from infancy to older childhood.
Main Methods:
- Retrospective analysis of rectal suction biopsies from 230 children (0-16 years) with Hirschsprung's disease.
- Histochemical staining for AChE activity to identify distinct patterns.
- Prospective study of 15 children with serial biopsies to observe AChE activity evolution.
Main Results:
- Three distinct AChE histochemical patterns were identified: Newborn (Pattern I), Classical (Pattern II), and Intermediate (Pattern III).
- Pattern I (newborn) characterized by absent lamina propria AChE, predominant in infants <3 months.
- Pattern II (classical) with lamina propria infiltration, seen in children >1 year, showing evolution from Pattern I.
Conclusions:
- AChE histochemical patterns in Hirschsprung's disease exhibit a clear age-dependent evolution.
- The observed progression from newborn to classical patterns aids in understanding the disease's developmental trajectory.
- These findings support the use of AChE histochemistry for diagnosing Hirschsprung's disease and monitoring its evolution in children.
Abstract:
The acetylcholinesterase (AChE) activity in Hirschsprung's disease was analyzed for histochemical patterns according to age. Its evolutional nature was also assessed. Two hundred thirty children (0 to 16 years of age) with acute or chronic obstipation were submitted to rectal suction biopsy for AChE histochemical staining. One hundred nineteen of them showed abnormal AChE activity. Retrospective analysis of those cases permitted us to establish three histochemical patterns: Pattern I, thick nerve trunks present only in the submucosa and, characteristically, absence of AChE activity in the lamina propria. This pattern was predominant in newborn up to 3 months of age and was designated the newborn pattern. Pattern II, thin nerve fibers in the muscularis mucosae and submucosa with a clear infiltration in the lamina propria. This pattern was mainly seen in children older than 1 year of age and was called classical pattern. Pattern III, an intermediate pattern showing morphologic characteristics of the two previous patterns with predominance of one or another, according to the age. A prospective study was also made in 15 children with Hirschsprung's disease who were submitted to suction rectal biopsies at different ages. In this study, a clear evolutional character of the AChE activity was observed from the newborn pattern to the intermediate and finally to the classical pattern.