Hirschsprung's disease prevalence in Europe: A register based study.

Détails

ID Serval
serval:BIB_D10115E7488E
Type
Article: article d'un périodique ou d'un magazine.
Collection
Publications
Institution
Titre
Hirschsprung's disease prevalence in Europe: A register based study.
Périodique
Birth Defects Research. Part A, Clinical and Molecular Teratology
Auteur⸱e⸱s
Best K.E., Addor M.C., Arriola L., Balku E., Barisic I., Bianchi F., Calzolari E., Curran R., Doray B., Draper E., Garne E., Gatt M., Haeusler M., Bergman J., Khoshnood B., Klungsoyr K., Martos C., Materna-Kiryluk A., Matias Dias C., McDonnell B., Mullaney C., Nelen V., O'Mahony M., Queisser-Luft A., Randrianaivo H., Rissmann A., Rounding C., Sipek A., Thompson R., Tucker D., Wellesley D., Zymak-Zakutnia N., Rankin J.
ISSN
1542-0760 (Electronic)
ISSN-L
1542-0752
Statut éditorial
Publié
Date de publication
2014
Peer-reviewed
Oui
Volume
100
Numéro
9
Pages
695-702
Langue
anglais
Notes
Publication types: Journal Article Publication Status: ppublish
Résumé
BACKGROUND: Hirschsprung's disease is a congenital gut motility disorder, characterised by the absence of the enteric ganglion cells along the distal gut. The aim of this study was to describe the epidemiology of Hirschsprung's disease, including additional congenital anomalies, total prevalence, trends, and association with maternal age.
METHODS: Cases of Hirschsprung's disease delivered during 1980 to 2009 notified to 31 European Surveillance of Congenital Anomaly registers formed the population-based case-series. Prevalence rates and 95% confidence intervals were calculated as the number of cases per 10,000 births. Multilevel Poisson regression was performed to investigate trends in prevalence, geographical variation and the association with maternal age.
RESULTS: There were 1,322 cases of Hirschsprung's disease among 12,146,210 births. The total prevalence was 1.09 (95% confidence interval, 1.03-1.15) per 10,000 births and there was a small but significant increase in prevalence over time (relative risk = 1.01; 95% credible interval, 1.00-1.02; p = 0.004). There was evidence of geographical heterogeneity in prevalence (p < 0.001). Excluding 146 (11.0%) cases with chromosomal anomalies or genetic syndromes, there were 1,176 cases (prevalence = 0.97; 95% confidence interval, 0.91-1.03 per 10,000 births), of which 137 (11.6%) had major structural anomalies. There was no evidence of a significant increased risk of Hirschsprung's disease in cases born to women aged ≥35 years compared with those aged 25 to 29 (relative risk = 1.09; 95% credible interval, 0.91-1.31; p = 0.355).
CONCLUSION: This large population-based study found evidence of a small increasing trend in Hirschsprung's disease and differences in prevalence by geographic location. There was also no evidence of an association with maternal age. Birth Defects Research (Part A), 100:695-702, 2014. © 2014 Wiley Periodicals, Inc.
Pubmed
Web of science
Création de la notice
30/10/2014 17:32
Dernière modification de la notice
20/08/2019 15:51
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