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EUROlinkCAT: Establishing a linked European Cohort of Children with Congenital Anomalies

Periodic Reporting for period 2 - EUROlinkCAT (EUROlinkCAT: Establishing a linked European Cohort of Children with Congenital Anomalies)

Reporting period: 2018-07-01 to 2019-12-31

Issue being addressed
- Over 130000 children born in Europe every year will have a congenital anomaly (CA)
- CAs (birth defects) are a major cause of infant mortality, childhood morbidity and long-term disability
- There is a lack of evidence-based information on the survival, health and educational achievements of children with CAs in Europe

Importance to society
- Investigating the health and educational outcomes of children with CAs over their first 10 years of life will allow parents, health professionals, public health and education authorities to plan for, and make informed decisions about, the treatment, services and education arrangements for these children. This will result in improved care, support and quality of life of these children.
- The project will help to facilitate the development of a reciprocal relationship between families with children with CAs, health and social care professionals and researchers via a multi-national survey and focus groups. These aim to provide the opportunity for public and professional engagement in setting and disseminating relevant research priorities and their outcomes.

- Establish a European network of standardised datasets containing information on the mortality, health, educational achievements and needs of children with CAs born from 1995-2014 up until 10 years of age
- Expand the knowledge on the survival, health, disease determinants, educational achievements and needs of children according to their specific CA
- Investigate socio-economic health inequalities
- Evaluate the costs of hospitalisation during the first five years of life for children with a CA
- Evaluate the accuracy of existing electronic health care databases and make recommendations on improving their use
- To focus on 4 specific CAs: Heart surgery in children, spina bifida, cleft lip, Down syndrome and use a multi-national survey and focus groups to ensure the experiences of parents of children with these congenital anomalies are heard
- Engage with relevant international/national/regional health authorities to ensure that relevant findings are implemented and translated into health policy
- Enable this unique research platform to be available for local research and future European wide analyses beyond the end of the project
EUROlinkCAT has formed a consortium of 22 EUROCAT registries in 14 European countries who, where possible, are linking children with a CA born from 1995-2014 up to age 10 to mortality, hospital discharge, prescription and education databases. Each registry standardises their data and runs centrally provided syntax scripts to create standard summarised tables and analytic results. These results are submitted to a Central Results Repository (CRR), enabling hypotheses on their health and education to be investigated at an EU level by researchers in individual work packages. Focus groups and a multi-national survey will involve these families in setting research priorities and improving dissemination of results.

Building EUROlinkCAT Central Results Repository
All data sources have been identified and an online database provides detailed information on all variables available in these data sources and the corresponding EUROlinkCAT standardised variables. Linkage and standardisation have been performed for 14 registries with mortality data. Linkage with health data has been performed for 7 registries and standardisation is ongoing. Four registries have linked with prescription data.

The mortality protocol and analysis plans have been completed. Relevant registries have obtained ethics approvals to link their data. A systematic review on mortality in children with CAs, registered with PROSPERO (Reg # CRD42017074675), has been submitted for publication. 12 out of 14 registries have submitted their linkage data quality results and 9 have submitted their mortality analysis results to the CRR, with delays being due to data suppression issues. Preliminary analyses on mortality for specific CAs have been performed.

The morbidity protocols covering hospital episode data and prescription data have been completed. Work is progressing on the standardisation of surgery and diagnosis codes recorded during the hospital stays. 6 out of 7 registries have started standardising their morbidity data. The majority are planning to include controls from the background population. Two clinicians have identified surgery codes specific for each anomaly subgroups and for each different national coding system from 5 countries (Wales, Italy, Spain, Denmark, Finland). Work is ongoing on measuring the 30-day fatality rate after cardiac and gastro-intestinal surgery and a coding and analysis plan for these has been completed, along with draft analysis plans for the prescription study and risk factors study.

A questionnaire was circulated to all registries and a report and public summary on educational provision for children with CAs is available online. Registries in Finland, Denmark and the UK have identified sources for linking with educational datasets. Special education needs data for children with CAs from Emilia Romagna (Italy) will be available. The WP5 analysis plan is based on education outcomes of 5 to 16 year old children using data from the National Pupil Database (England and Wales) and data from Finland (national tests only at 16 years old). A systematic review on educational achievements of children with CAs has been registered with PROSPERO (Reg. # CRD42017074675) and initial literature searches identifying over 7000 papers have been completed.

A variety of dissemination activities promoting the project are ongoing. An information pamphlet has been created, translated into 5 languages and distributed. A Parent Area has been created on the website and populated with research summaries. Parents of children with CAs are being consulted in different countries using face-to-face focus groups to ascertain their opinions. A multi-national survey will be translated into native languages and disseminated using social media and parent support group contacts to ensure wide geographic and demographic coverage, to find out about the support and information parents received following their child’s diagnosis."
The project will be the 1st to establish a comprehensive set of independent standardised databases containing consistently coded and verified information on the morbidity, mortality and educational experiences of children with CAs up to age 10 across Europe. It will be an invaluable resource for research to be conducted to improve the survival and morbidity of these children and will also establish a platform for continued data collection and collaboration across Europe.

The project will demonstrate that by setting up the correct infrastructure, protocols and coding guidelines, data and expertise can be truly shared and analysed across Europe obtaining enough information on often rare diseases to inform and optimise personalised care and treatment decisions for these children.

The proposed work will be the first to exploit the enormous potential of electronic health records in a standardised manner across Europe. Not only will the study provide guidance on research for CAs, but it will also provide a template for how to establish other European cohorts such that routine electronic data can be used for both research and surveillance.
Group Photo EUROlinkCAT researchers
Project Logo/Header
Map of participating Sites and countries