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Hirschsprung's disease prevalence in Europe: a register based study
Kate E Best1, Marie-Claude Addor, Larraitz Arriola
1Institute of Health & Society, Newcastle University, Newcastle upon Tyne, England, United Kingdom.
Summary
Hirschsprung
Area of Science:
- Pediatric Surgery
- Clinical Epidemiology
- Congenital Disorders
Background:
- Hirschsprung's disease is a congenital gut motility disorder.
- Characterized by the absence of enteric ganglion cells in the distal gut.
Purpose of the Study:
- To describe the epidemiology of Hirschsprung's disease.
- Investigate prevalence, trends, and association with maternal age.
Main Methods:
- Population-based case-series of 1,322 cases from 31 European registers (1980-2009).
- Prevalence calculated per 10,000 births.
- Multilevel Poisson regression used to analyze trends, geographical variation, and maternal age association.
Main Results:
- Total prevalence of 1.09 per 10,000 births, with a significant increasing trend over time.
- Significant geographical heterogeneity in prevalence observed.
- No significant association found between Hirschsprung's disease and maternal age ≥35 years.
Conclusions:
- A small increasing trend in Hirschsprung's disease prevalence noted.
- Geographical variations in prevalence exist.
- No association found with advanced maternal age.
