An epidemiological study of Hirschsprung's disease and additional anomalies |
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| Authors: | Michael Bjø rn Russell,Charlotte Astrid Russell,Erik Niebuhr |
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| Affiliation: | Department of Neurology, Gentofe Hospital, University of Copenhagen, Hellerup. Denmark;Department of Hematology, Rigshospitalet, University of Copenhagen, Copenhagen, Denmark;Institute of Medical Genetics, University of Copenhagen, Copenhagen, Denmark |
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| Abstract: | Russell MB, Russell CA, Niebuhr E. An epidemiological study of Hirschsprung's disease and additional anomalies. Acta Pædiatr 1994;83:68–71. Stockholm. ISSN 0803–5253.
The incidence of Hirschsprung's disease was studied in approximately 1.5 million consecutive live births in Denmark by hospital records. A diagnosis of Hirschsprung's disease required a histologic verified absence of ganglion cells in either biopsy or surgical colonic specimens. The incidence of Hirschsprung's disease was found to be 0.140 per 1000 live births (1:7,165) with a male: female ratio of 4.1:1 in short segment, and 2.4:1 in long segment Hirschsprung's disease (p = 0.36). Maternal age and birth order were unimportant factors. The association of Hirschsprung's disease and Down's syndrome was seen in 9 of the 207 patients and may represent a real association, whereas the association with congenital heart defects seen in 2% (not including patients with Down's syndrome) is more doubtful. A mortality of 16% among the patients with Hirschsprung's disease emphasizes the extreme importance of early diagnosis. |
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| Keywords: | Hirschsprung's disease incidence and additional anomalies |
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