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      Trends in congenital anomalies in Europe from 1980 to 2012

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          Abstract

          Background

          Surveillance of congenital anomalies is important to identify potential teratogens.

          Methods

          This study analysed the prevalence of 61 congenital anomaly subgroups (excluding chromosomal) in 25 population-based EUROCAT registries (1980–2012). Live births, fetal deaths and terminations of pregnancy for fetal anomaly were analysed with multilevel random-effects Poisson regression models.

          Results

          Seventeen anomaly subgroups had statistically significant trends from 2003–2012; 12 increasing and 5 decreasing.

          Conclusions

          The annual increasing prevalence of severe congenital heart defects, single ventricle, atrioventricular septal defects and tetralogy of Fallot of 1.4% (95% CI: 0.7% to 2.0%), 4.6% (1.0% to 8.2%), 3.4% (1.3% to 5.5%) and 4.1% (2.4% to 5.7%) respectively may reflect increases in maternal obesity and diabetes (known risk factors). The increased prevalence of cystic adenomatous malformation of the lung [6.5% (3.5% to 9.4%)] and decreased prevalence of limb reduction defects [-2.8% (-4.2% to -1.5%)] are unexplained. For renal dysplasia and maternal infections, increasing trends may be explained by increased screening, and deceases in patent ductus arteriosus at term and increases in craniosynostosis, by improved follow up period after birth and improved diagnosis. For oesophageal atresia, duodenal atresia/stenosis and ano-rectal atresia/stenosis recent changes in prevalence appeared incidental when compared with larger long term fluctuations. For microcephaly and congenital hydronephrosis trends could not be interpreted due to discrepancies in diagnostic criteria. The trends for club foot and syndactyly disappeared once registries with disparate results were excluded. No decrease in neural tube defects was detected, despite efforts at prevention through folic acid supplementation.

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          Most cited references17

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          Reduction in neural-tube defects after folic acid fortification in Canada.

          In 1998, folic acid fortification of a large variety of cereal products became mandatory in Canada, a country where the prevalence of neural-tube defects was historically higher in the eastern provinces than in the western provinces. We assessed changes in the prevalence of neural-tube defects in Canada before and after food fortification with folic acid was implemented. The study population included live births, stillbirths, and terminations of pregnancies because of fetal anomalies among women residing in seven Canadian provinces from 1993 to 2002. On the basis of published results of testing of red-cell folate levels, the study period was divided into prefortification, partial-fortification, and full-fortification periods. We evaluated the relationship between baseline rates of neural-tube defects in each province and the magnitude of the decrease after fortification was implemented. A total of 2446 subjects with neural-tube defects were recorded among 1.9 million births. The prevalence of neural-tube defects decreased from 1.58 per 1000 births before fortification to 0.86 per 1000 births during the full-fortification period, a 46% reduction (95% confidence interval, 40 to 51). The magnitude of the decrease was proportional to the prefortification baseline rate in each province, and geographical differences almost disappeared after fortification began. The observed reduction in rate was greater for spina bifida (a decrease of 53%) than for anencephaly and encephalocele (decreases of 38% and 31%, respectively). Food fortification with folic acid was associated with a significant reduction in the rate of neural-tube defects in Canada. The decrease was greatest in areas in which the baseline rate was high. Copyright 2007 Massachusetts Medical Society.
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            Reduction of birth prevalence rates of neural tube defects after folic acid fortification in Chile.

            To verify whether the decreasing neural tube defects birth prevalence rates in Chile are due to folic acid fortification or to pre-existing decreasing trends, we performed a population survey using a network of Estudio Colaborativo Latino Americano de Malformaciones Congenitas (ECLAMC, Latin American Collaborative Study of Congenital Malformations) maternity hospitals in Chile, between the years 1982 and 2002. Within each maternity hospital, birth prevalence rates of spina bifida and anencephaly were calculated from two pre-fortification periods (1982-1989 and 1990-2000), and from one fortified period (2001-2002). There was no historical trend for spina bifida birth prevalence rates before folic acid fortification, and there was a 51% (minimum 27%, maximum 66%) decrease in the birth prevalence rates of this anomaly in the fortified period. The relative risks of spina bifida were homogeneous among hospitals in the two period comparisons. There was no historical trend for the birth prevalence of anencephaly comparing the two pre-fortified periods, but the relative risks were heterogeneous among hospitals in this comparison. There was a 42% (minimum 10%, maximum 63%) decrease in the birth prevalence rate of anencephaly in the fortified period as compared with the immediately pre-fortified period, with homogeneous relative risks among hospitals. Within the methodological constraints of this study we conclude that the birth prevalence rates for both spina bifida and anencephaly decreased as a result of folic acid fortification, without interference of decreasing secular trends. Copyright (c) 2005 Wiley-Liss, Inc.
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              Paper 1: The EUROCAT network--organization and processes.

              The European Surveillance of Congenital Anomalies (EUROCAT) is a network of population-based congenital anomaly registries in Europe, funded by the European Union, which has been in operation for more than 30 years. It currently surveys more than 1.7 million births per year, including 31% of births in the European Union, and includes almost all population-based European congenital anomaly registries as its members. EUROCAT member registries collect data, ascertained from multiple sources, on all major structural congenital and chromosomal anomalies. EUROCAT surveillance relates to three areas: prevalence, primary prevention, and prenatal screening. This article describes the history of EUROCAT and gives an overview of the current methodology and work of EUROCAT covering the database content and management, coding and classification of anomalies, core surveillance, prevalence tables, statistical monitoring. The monitoring of new developments in prenatal diagnosis, medication during pregnancy, use of folic acid, and investigation of clusters and exposures are overseen by working groups responsible for organizing research and producing regular reports. The EUROCAT Web site includes current data on prevalence rates and prenatal detection rates-an example of information useful to clinicians, public health service managers, and patients. Copyright © 2011 Wiley-Liss, Inc.
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                Author and article information

                Contributors
                Role: ConceptualizationRole: Formal analysisRole: MethodologyRole: Writing – original draftRole: Writing – review & editing
                Role: Formal analysisRole: Writing – review & editing
                Role: Data curationRole: Project administrationRole: Writing – review & editing
                Role: ConceptualizationRole: Writing – review & editing
                Role: Data curationRole: Writing – review & editing
                Role: Data curationRole: Writing – review & editing
                Role: Data curationRole: Writing – review & editing
                Role: Data curationRole: Writing – review & editing
                Role: Data curationRole: Writing – review & editing
                Role: Data curationRole: Writing – review & editing
                Role: Data curationRole: Writing – review & editing
                Role: Data curationRole: Writing – review & editing
                Role: Data curationRole: Writing – review & editing
                Role: Data curationRole: Writing – review & editing
                Role: Data curationRole: Writing – review & editing
                Role: Data curationRole: Writing – review & editing
                Role: Data curationRole: Writing – review & editing
                Role: Data curationRole: Writing – review & editing
                Role: Data curationRole: Writing – review & editing
                Role: Data curationRole: Writing – review & editing
                Role: Data curationRole: Writing – review & editing
                Role: Data curationRole: Writing – review & editing
                Role: Data curationRole: Writing – review & editing
                Role: Data curationRole: Writing – review & editing
                Role: Data curationRole: Writing – review & editing
                Role: Data curationRole: Writing – review & editing
                Role: Data curationRole: Writing – review & editing
                Role: Data curationRole: Writing – review & editing
                Role: Data curationRole: Writing – review & editing
                Role: ConceptualizationRole: Writing – review & editing
                Role: Editor
                Journal
                PLoS One
                PLoS ONE
                plos
                plosone
                PLoS ONE
                Public Library of Science (San Francisco, CA USA )
                1932-6203
                5 April 2018
                2018
                : 13
                : 4
                : e0194986
                Affiliations
                [1 ] Wolfson Institute of Preventive Medicine, Queen Mary University of London, London, United Kingdom
                [2 ] Faculty Life & Health Sciences, University of Ulster, Newtownabbey, United Kingdom
                [3 ] Department of Woman-Mother-Child, University Hospital Center CHUV, Lausanne, Switzerland
                [4 ] Public Health Division of, Biodonostia Research Institute, San Sebastián, Spain
                [5 ] Department of Medical Genetics and Reproductive Health,Children’s Hospital Zagreb, Medical School University of Zagreb, Zagreb, Croatia
                [6 ] Department of Genetics, University Medical Center Groningen, University of Groningen, Groningen, the Netherlands
                [7 ] National Public Health and Medical Officer Service, Hungarian Congenital Abnormality Registry, Budapest, Hungary
                [8 ] Centro de Estudos e registo de A C, Lisbon, Portugal
                [9 ] Department of Health Sciences, University of Leicester, Leicester, United Kingdom
                [10 ] Paediatric department, Hospital Lillebaelt, Kolding, Denmark
                [11 ] Directorate for Health Information and Research, Guardamangia, Malta
                [12 ] Paris Registry of Congenital Anomalies, Inserm UMR 1153, Obstetrical, Perinatal and Pediatric Epidemiology Research Team, Center for Epidemiology and Statistics Sorbonne Paris Cité, Paris Descartes University, Paris, France
                [13 ] Department of Global Public Health and Primary Care, University of Bergen, Bergen, Norway
                [14 ] Division for mental and physical health, Norwegian Institute of Public Health, Bergen, Norway
                [15 ] Department of Public Health, Health Service Executive, Kilkenny, Ireland
                [16 ] Department of Public Health, Health Service Executive, Dublin, Ireland
                [17 ] Provincial Institute for Hygiene, Antwerp, Belgium
                [18 ] IMER Registry, Center for Clinical and Epidemiological Research, University of Ferrara, Ferrara, Italy
                [19 ] Azienda Ospedaliero- Universitaria di Ferrara, Ferrara, Italy
                [20 ] Department of Public Health, Health Service Executive, Cork, Ireland
                [21 ] CNR Institute of Clinical Physiology, Pisa, Italy
                [22 ] Center for child and adolescence medicine, University Medical Center of the Johannes Gutenberg University, Mainz, Germany
                [23 ] Registre des Malformations Congenitales de la Reunion, St Pierre, Ile de la Reunion, France
                [24 ] Institute of Health & Society, Newcastle University, Newcastle upon Tyne, United Kingdom
                [25 ] Malformation Monitoring Centre Saxony-Anhalt, Otto-von-Guericke University Magdeburg, Magdeburg, Germany
                [26 ] National Perinatal and Epidemiology Unit, University of Oxford, Oxford, United Kingdom
                [27 ] Public Health Wales, Swansea, United Kingdom
                [28 ] Center for Human Genetics,Institut de Pathologie at de Genetique, Charleroi, Belgium
                [29 ] University of Southampton and Wessex Clinical Genetics Service, Southampton, United Kingdom
                Universidad Miguel Hernandez de Elche, SPAIN
                Author notes

                Competing Interests: The authors have declared that no competing interests exist.

                Author information
                http://orcid.org/0000-0002-7164-612X
                Article
                PONE-D-17-33015
                10.1371/journal.pone.0194986
                5886482
                29621304
                7f9554fa-16a9-4aef-bbff-361759e62698
                © 2018 Morris et al

                This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.

                History
                : 10 September 2017
                : 14 March 2018
                Page count
                Figures: 10, Tables: 2, Pages: 18
                Funding
                Funded by: European Union Framework for Health Programme
                Award Recipient :
                This work was supported by the European Union in the framework of the Health Programme (2008-2013) to all authors. The funders had no role in study design, data collection and analysis, decision to publish, or preparation of the manuscript.
                Categories
                Research Article
                Medicine and Health Sciences
                Congenital Disorders
                Congenital Anomalies
                Medicine and Health Sciences
                Women's Health
                Maternal Health
                Birth
                Medicine and Health Sciences
                Women's Health
                Obstetrics and Gynecology
                Birth
                People and Places
                Geographical Locations
                Europe
                Medicine and Health Sciences
                Congenital Disorders
                Birth Defects
                Microcephaly
                Biology and Life Sciences
                Developmental Biology
                Morphogenesis
                Birth Defects
                Microcephaly
                Medicine and Health Sciences
                Diagnostic Medicine
                Signs and Symptoms
                Stenosis
                Medicine and Health Sciences
                Pathology and Laboratory Medicine
                Signs and Symptoms
                Stenosis
                Biology and Life Sciences
                Anatomy
                Cardiovascular Anatomy
                Heart
                Cardiac Ventricles
                Medicine and Health Sciences
                Anatomy
                Cardiovascular Anatomy
                Heart
                Cardiac Ventricles
                Medicine and Health Sciences
                Congenital Disorders
                Birth Defects
                Congenital Heart Defects
                Ventricular Septal Defects
                Biology and Life Sciences
                Developmental Biology
                Morphogenesis
                Birth Defects
                Congenital Heart Defects
                Ventricular Septal Defects
                Medicine and Health Sciences
                Cardiology
                Congenital Heart Defects
                Ventricular Septal Defects
                Medicine and Health Sciences
                Congenital Disorders
                Birth Defects
                Limb Reduction Defects
                Biology and Life Sciences
                Developmental Biology
                Morphogenesis
                Birth Defects
                Limb Reduction Defects
                Custom metadata
                Data Are available from the EUROCAT website http://www.eurocat-network.eu/accessprevalencedata/prevalencetables and selecting the tables required.

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