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Hirschsprung's disease in Oman

A Rajab1, N V Freeman, M A Patton

  • 1Department of Child Health, Royal Hospital, Muscat, Oman.

Journal of Pediatric Surgery
|May 1, 1997
PubMed
Summary

The population frequency of Hirschsprung

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Area of Science:

  • Pediatric Surgery
  • Medical Genetics
  • Epidemiology

Background:

  • Hirschsprung's disease (HD) is a congenital condition affecting the large intestine.
  • Understanding the incidence and risk factors of HD is crucial for early diagnosis and management.

Purpose of the Study:

  • To determine the population-based incidence of Hirschsprung's disease in Oman.
  • To investigate regional variations, demographic factors, and associated anomalies in Omani children with HD.

Main Methods:

  • Retrospective analysis of hospital-based data from Oman's sole pediatric surgery unit (1989-1994).
  • Calculation of incidence based on 85 HD cases and 261,000 livebirths.
  • Analysis of presentation age, regional frequency, sex ratio, consanguinity, and co-occurring conditions.

Main Results:

  • The overall population frequency of Hirschsprung's disease in Oman was 1 in 3,070 (0.3/1,000).
  • The North Sharqiya region showed the highest incidence (1 in 1,800).
  • A male to female ratio of 2.9:1 was observed, with a high consanguinity rate (75%) and 11% of cases associated with Down's syndrome.

Conclusions:

  • Hirschsprung's disease incidence in Oman is relatively low but shows regional variations.
  • High consanguinity rates suggest a potential genetic predisposition in the Omani population.
  • Associated malformations, particularly Down's syndrome, are common in Omani children with HD.

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