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Epidemiology of congenital diaphragmatic hernia in Europe: a register-based study

Epidemiology of congenital diaphragmatic hernia in Europe: a register-based study
Epidemiology of congenital diaphragmatic hernia in Europe: a register-based study
Introduction

Published prevalence rates of congenital diaphragmatic hernia (CDH) vary. This study aims to describe the epidemiology of CDH using data from high-quality, population-based registers belonging to the European Surveillance of Congenital Anomalies (EUROCAT).

Methods

Cases of CDH delivered between 1980 and 2009 notified to 31 EUROCAT registers formed the population-based case series. Prevalence over time was estimated using multilevel Poisson regression, and heterogeneity between registers was evaluated from the random component of the intercept.

Results

There were 3373 CDH cases reported among 12?155?491 registered births. Of 3131 singleton cases, 353 (10.4%) were associated with a chromosomal anomaly, genetic syndrome or microdeletion, 784 (28.2%) were associated with other major structural anomalies. The male to female ratio of CDH cases overall was 1:0.69. Total prevalence was 2.3 (95% CI 2.2 to 2.4) per 10?000 births and 1.6 (95% CI 1.6 to 1.7) for isolated CDH cases. There was a small but significant increase (relative risk (per year)=1.01, 95% credible interval 1.00–1.01; p=0.030) in the prevalence of total CDH over time but there was no significant increase for isolated cases (ie, CDH cases that did not occur with any other congenital anomaly). There was significant variation in total and isolated CDH prevalence between registers. The proportion of cases that survived to 1?week was 69.3% (1392 cases) for total CDH cases and 72.7% (1107) for isolated cases.

Conclusions

This large population-based study found an increase in total CDH prevalence over time. CDH prevalence also varied significantly according to geographical location. No significant association was found with maternal age.
maternal age, population based, prevalence, survival, congenital diaphragmatic hernia
1359-2998
F137-F144
McGivern, M.R.
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Best, K.E.
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Rankin, J.
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Wellesley, D.
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Greenlees, R.
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Addor, M.-C.
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Arriola, L.
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de Walle, H.
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Barisic, I.
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Beres, J.
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Bianchi, F.
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Calzolari, E.
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Doray, B.
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Draper, E.S.
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Garne, E.
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Gatt, M.
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Haeusler, M.
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Khoshnood, B.
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Klungsoyr, K.
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Latos-Bielenska, A.
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O'Mahony, M.
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McDonnell, B.
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Mullaney, C.
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Nelen, V.
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Queisser-Luft, A.
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Sipek, A.
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Tucker, D.
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Wertelecki, W.
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Martos, C.
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McGivern, M.R.
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Best, K.E.
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Rankin, J.
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Wellesley, D.
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Greenlees, R.
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Addor, M.-C.
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Arriola, L.
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de Walle, H.
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Barisic, I.
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Beres, J.
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Bianchi, F.
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Calzolari, E.
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Doray, B.
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Garne, E.
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Gatt, M.
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Haeusler, M.
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Khoshnood, B.
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Klungsoyr, K.
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Latos-Bielenska, A.
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O'Mahony, M.
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Braz, P.
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McDonnell, B.
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Mullaney, C.
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Nelen, V.
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Queisser-Luft, A.
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Randrianaivo, H.
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Rissmann, A.
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Rounding, C.
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Sipek, A.
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Thompson, R.
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Tucker, D.
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Wertelecki, W.
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Martos, C.
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McGivern, M.R., Best, K.E., Rankin, J., Wellesley, D., Greenlees, R., Addor, M.-C., Arriola, L., de Walle, H., Barisic, I., Beres, J., Bianchi, F., Calzolari, E., Doray, B., Draper, E.S., Garne, E., Gatt, M., Haeusler, M., Khoshnood, B., Klungsoyr, K., Latos-Bielenska, A., O'Mahony, M., Braz, P., McDonnell, B., Mullaney, C., Nelen, V., Queisser-Luft, A., Randrianaivo, H., Rissmann, A., Rounding, C., Sipek, A., Thompson, R., Tucker, D., Wertelecki, W. and Martos, C. (2015) Epidemiology of congenital diaphragmatic hernia in Europe: a register-based study. Archives of Disease in Childhood - Fetal and Neonatal Edition, 100 (2), F137-F144. (doi:10.1136/archdischild-2014-306174). (PMID:25411443)

Record type: Article

Abstract

Introduction

Published prevalence rates of congenital diaphragmatic hernia (CDH) vary. This study aims to describe the epidemiology of CDH using data from high-quality, population-based registers belonging to the European Surveillance of Congenital Anomalies (EUROCAT).

Methods

Cases of CDH delivered between 1980 and 2009 notified to 31 EUROCAT registers formed the population-based case series. Prevalence over time was estimated using multilevel Poisson regression, and heterogeneity between registers was evaluated from the random component of the intercept.

Results

There were 3373 CDH cases reported among 12?155?491 registered births. Of 3131 singleton cases, 353 (10.4%) were associated with a chromosomal anomaly, genetic syndrome or microdeletion, 784 (28.2%) were associated with other major structural anomalies. The male to female ratio of CDH cases overall was 1:0.69. Total prevalence was 2.3 (95% CI 2.2 to 2.4) per 10?000 births and 1.6 (95% CI 1.6 to 1.7) for isolated CDH cases. There was a small but significant increase (relative risk (per year)=1.01, 95% credible interval 1.00–1.01; p=0.030) in the prevalence of total CDH over time but there was no significant increase for isolated cases (ie, CDH cases that did not occur with any other congenital anomaly). There was significant variation in total and isolated CDH prevalence between registers. The proportion of cases that survived to 1?week was 69.3% (1392 cases) for total CDH cases and 72.7% (1107) for isolated cases.

Conclusions

This large population-based study found an increase in total CDH prevalence over time. CDH prevalence also varied significantly according to geographical location. No significant association was found with maternal age.

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More information

Accepted/In Press date: 19 November 2014
Published date: March 2015
Keywords: maternal age, population based, prevalence, survival, congenital diaphragmatic hernia
Organisations: Human Development & Health

Identifiers

Local EPrints ID: 372607
URI: http://eprints.soton.ac.uk/id/eprint/372607
ISSN: 1359-2998
PURE UUID: 1d33d5e1-660b-4664-b40b-2771e6571107

Catalogue record

Date deposited: 16 Dec 2014 16:45
Last modified: 14 Mar 2024 18:39

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Contributors

Author: M.R. McGivern
Author: K.E. Best
Author: J. Rankin
Author: D. Wellesley
Author: R. Greenlees
Author: M.-C. Addor
Author: L. Arriola
Author: H. de Walle
Author: I. Barisic
Author: J. Beres
Author: F. Bianchi
Author: E. Calzolari
Author: B. Doray
Author: E.S. Draper
Author: E. Garne
Author: M. Gatt
Author: M. Haeusler
Author: B. Khoshnood
Author: K. Klungsoyr
Author: A. Latos-Bielenska
Author: M. O'Mahony
Author: P. Braz
Author: B. McDonnell
Author: C. Mullaney
Author: V. Nelen
Author: A. Queisser-Luft
Author: H. Randrianaivo
Author: A. Rissmann
Author: C. Rounding
Author: A. Sipek
Author: R. Thompson
Author: D. Tucker
Author: W. Wertelecki
Author: C. Martos

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