title: An epidemiological study of Hirschsprung's disease and additional anomalies.
abstract: 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.
authors.affiliation: Department of Neurology, Gentofte Hospital, University of Copenhagen, Hellerup, Denmark.
authors.forename: M B
authors.initials: MB
authors.lastname: Russell
authors.forename: C A
authors.initials: CA
authors.lastname: Russell
authors.forename: E
authors.initials: E
authors.lastname: Niebuhr
mesh_terms: D000015:Abnormalities, Multiple / Q000453:epidemiology*; D000293:Adolescent; D002648:Child; D003718:Denmark / Q000453:epidemiology; D005260:Female; D006627:Hirschsprung Disease / Q000453:epidemiology*; D006801:Humans; D015994:Incidence; D007223:Infant; D008297:Male