History and Funding
The Central East Registry began in 1973 within the Rhone-Alps area - the Auvergne region was added in 1983, the Jura area in 1985, the Cote d'Or & Nievre in 1989 and Saone-et-Loire in 1990. The Registry joined EUROCAT as an associate member in 1999, and sent data back-dated to 1980. In 1998 the Registry was split up and the Auvergne region became financially independent under the responsibility of Christine Francannet.
The collaboration between Auvergne and the rest of the Central East France Registry is maintained and common results are to be published. At the start of 2007, the Registry was renamed REMERA and limited to Rhone-Alpes region.
Since January 2007, the financial support is provided only by public founds: by the Rhône Alpes Region authority, the French Institute of disease Monitoring (InVS), the French Health Products Safety Agency (AFSSAPS), the National Institute of Health and Medical Research (INSERM), and the Conseil Général of Isère.
Population Coverage
The Registry is population-based II where all mothers delivering within defined geographic area, irrespective of place of residence. Approximately 1.5% of non-resident mothers deliver within the registry area. Mothers living in the defined area who are later transferred outside the area for delivery because of prenatal diagnosis of malformation made in the defined area are included as well.
The Registry covers the Rhone-Alps region, with approximately 57 500 births annually which represents about 7% of all births in France. The geographic region changed over time as follows: Rhone-Alps was included from 1976 onwards, Auvergne from 1983 to 1999, Jura from 1985 onwards, Cote d'Or and Nievre from 1989 onwards and Saone-et-Loire from 1990 onwards. From 2007, the Registry is limited to a part of Rhone-Alpes region: Rhône, Loire, Isère, Savoie.
Sources of Ascertainment
Notification to the Registry is voluntary. Reports are collected from multiple sources: maternity unit records, paediatric records, cytogenetic laboratory, pathology laboratory, child health services, specialised departments for medical genetics, paediatric surgeons, birth notifications. We have difficulties accessing the paediatric cardiology department of one hospital in our region. Consequently there will be some cases that are not reported.
Active registration is performed by four data collectors, assigned specifically to about 35 sources each. Confirmatory sources: every year a list of abnormal karyotypes is sent by cytogenetic laboratories, and copies of pathology reports are systematically sent to the Registry if malformations are present. At least, more than 145 sources collaborate with the registry. Infants up to the age of one are registered, as well as fetuses delivered after medical abortion.
A cut-off for notification is applied: for children born in year x, notifications are taken into account until March x+2. There is no follow-up procedure. Children are notified when noticed by the persons in charge of data collection in hospitals. The following specific anomalies are excluded: balanced chromosomal anomalies, pyloric stenosis, metabolic disorders, minor malformations (small angiomas or naevi, hip subdislocation, small foot deformities, ill-defined facial anomalies, inguinal and umbilical hernias). Aggregated data is transmitted to EUROCAT.
Maximum Age at Diagnosis
Up to 1 year of age.
Terminations of Pregnancy for Fetal Anomaly (TOPFA)
Termination of pregnancy is legal and there is no upper gestational age limit for any cause whatsoever. In the case of termination after diagnosis of a congenital anomaly, agreement is sought from a multidisciplinary committee. Terminations for fetal malformation have been registered since 1985. Notification of terminations of pregnancy is provided by: obstetric units, cytogenetic laboratories and pathology reports.
Stillbirth and Early Fetal Deaths
Currently still births and early fetal death/spontaneous abortions are registred from 20 weeks gestation onward. Before 1997, stillbirths were registered at 28 weeks or more after the last menstrual period. Stillbirth and infant death certificates are not routinely available as a source. Autopsy rates for spontaneous abortions are not registered, stillbirths 90%, induced abortions 90% and early neonatal deaths (0-7 days) 80%. These numbers are only available to the registry as autopsy rates for malformed infants/fetuses. Most autopsies of stillborn babies are performed by a fetal pathologist.
Exposure Data Availability
Information on maternal and paternal occupation, drug use, diseases etc is collected by interviews of the mothers of the malformed infants. This is not transmitted to Central Registry. No controls are interviewed.
Denominators and Controls Information
Birth statistics are provided by the National Institute of Statistics. The same population definition is used for the birth statistics except for induced abortions. Some background information is also available from the general population statistics. No information on controls is collected.
Registry Description References
See the description in following link: http://www.invs.sante.fr/beh/2008/28_29/beh_28_29_2008.pdf
Ethics & Consent
The registry does require ethics committee approval in order to collect and store data and this approval comes from CNIL (Commission Nationale Informatique et Liberte) + CCTIRS (Comite Consultatif du Traitement de l’Information Recherche en Sante). Approval has to be checked or renewed when there is a change in the database.
National legislation requires informed consent in order to register a baby with a congenital anomaly. Parents have to ask for removal of the child from the Register (opt-out).
Address for Further Information
Emmanuelle Amar (leader of REMERA registry from 2007)
Registre des Malformations en Rhône Alpes
Faculté de médecine Laennec 7-9 rue Guillaume Paradin
69372 Lyon, France
Tel: +33 4 78771058
Fax: +33 4 78771088
Email: emmanuelle.amar@remera.fr