An epidemiological study of Hirschsprung's disease and additional anomalies
- 21 January 1994
- journal article
- Published by Wiley in Acta Paediatrica
- Vol. 83 (1), 68-71
- https://doi.org/10.1111/j.1651-2227.1994.tb12955.x
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.Keywords
This publication has 20 references indexed in Scilit:
- Hirschsprung disease: a genetic studyClinical Genetics, 2008
- Hirschsprung's disease: Associated abnormalities and demographyJournal of Pediatric Surgery, 1992
- Waardenburg's Syndrome with Hirschsprung's Disease: A Neural Crest DefectPediatric Dermatology, 1986
- Diagnosis and Treatment of Hirschsprungʼs Disease in JapanAnnals of Surgery, 1984
- Anorectal manometry results in defecation disorders.Archives of Disease in Childhood, 1983
- Hirschsprung's disease in a kindred: A possible clue to the genetics of the diseaseJournal of Pediatric Surgery, 1982
- Hirschsprung's disease a survey of the members of the Surgical Section of the American Academy of PediatricsJournal of Pediatric Surgery, 1979
- The association of Waardenburg syndrome and Hirschsprung megacolonAmerican Journal of Medical Genetics, 1979
- Investigation and management of long-standing chronic constipation in childhood.Archives of Disease in Childhood, 1976
- A family study of Hirschsprung's diseaseAnnals of Human Genetics, 1963