History and Funding
The official population-based registration of congenital anomalies in former Czechoslovakia started in 1964. After the dissolution of Czechoslovakia in 1993, the registration in Czech Republic continues as the National Registry of Congenital Anomalies of the Czech Republic. The registry is a part of the National Registry of Reproduction Health which is run by the Institute of Health Informatics and Statistics of the Czech Republic (UZIC CR, http://www.uzic.cz/). The registration process itself is compulsory by the national law and financed by the Government. The registry joined EUROCAT in 2009.
Population Coverage
The registry is population-based. Currently the Czech Republic is consisting of thirteen regions and the capital Prague (with regional status). The regions can be further divided into 76 districts in total. The population of the Czech Republic is approximately 10 693 939 inhabitants (January 1st, 2020 ). The annual number of live births is approximately 110 000 births (112 231 live births in 2019).
Sources of Ascertainment
The Registry has multiple sources, any physician who diagnoses the anomaly for the first time (prenatally or postnatally) should submit the case report (notification). From 2016, the case reports are submitted electronically into the central database of the Institute of Health Information and Statistics of the Czech Republic. Most of the reports are submitted from delivery units, paediatric and neonatology department, departments of medical genetics (including cytogenetic and molecular-genetic laboratories), departments of ultrasound diagnostics, from children's surgery departments (cardio-surgery, plastic-surgery) etc. The registry receives notifications about prenatally diagnosed cases (including elective terminations of pregnancy and fetal deaths) and postnatally diagnosed cases (including stillbirths). The cases of spontaneous abortions are not reported.
All diagnoses from the XVII chapter of the ICD-10 (group Q00-Q99) are reported. We do not receive the verbal description or photographs of the reported anomalies, nor do we receive the complete lists of pathologic results of cytogenetic and/or molecular genetic examinations. From 2016, the cases may be coded also by the OMIM, ORPHAnet or SSIEM codes. Currently, there is no age limit for reporting, during 1994-2015 time period the age limit was up to 15 years of age.
Termination of Pregnancy
Termination of pregnancy is legal in the Czech Republic. After the 12th week of pregnancy, the termination is allowed only in selected situations (defined by national law, including confirmed genetic syndromes and/or severe structural anomalies of the fetus). After the 24th week of gestation, the pregnancy can be terminated only in rare situations defined by the law (lethal anomalies of the fetus, severe risk for maternal health) .
Stillbirth Definition and Early Foetal Deaths
Stillbirth definition: no signs of life and birthweight above 500g. If the birthweight cannot be determined, the gestational age should be 22 weeks or over (if the gestational age cannot be determined then the birth length should be 25 cm or over).
Early foetal deaths / spontaneous abortions definition: no signs of life and birth weight less than 500g and/or gestational age less than 22 weeks.
Exposure Data Availability
Information about medication in pregnancy and maternal diseases is collected in the National Registry of Congenital Anomalies. Partial information about exposure is also available for the control (all newborns are registered in the National Registry of Mothers and Newborns).
Denominators and Controls Information
Detailed demographical data (including annual births in the area of the Czech Republic are collected and reported by the Statistical Office (CSU, https://www.czso.cz).
Registry Description References
Sipek A, Gregor V, Horacek J, Sipek A Jr. National Registry of Congenital Anomalies of the Czech Republic: commemorating 50 years of the official registration. Cent Eur J Public Health.- 2014; 22(4):287-8. doi:10.21101/cejph.a4201.
Sipek A, Gregor V, Horacek J, Mazankova V, Langhammer P, Sipek A Jr. [History and present of registration of congenital anomalies in the Czech Republic], Cas Lek Cesk, 2009;148(10):505-9. In Czech.
Ethics & Consent
The reporting process is compulsory by the Czech national law and does not require informed consent. Datasets issued for scientific/surveillance purposes are strictly anonymous, the follow-up of selected cases is impossible. Ethical committee approval is obtained for several specific studies and/or grant proposals.
Address for Further Information
Antonin Sipek, MD, PhD, Program Director
National Registry of Congenital Anomalies of the Czech Republic
Department of the Medical Genetics
Thomayer University Hospital
Videnska 800
140 59 Prague 4
CZECH REPUBLIC
Tel: +420 261 083 636
Email: registrvvv@vrozene-vady.cz
Web: http://www.vrozene-vady.cz/
Antonin Sipek Jr. MD, PhD
Institute of Biology and Medical Genetics, 1st Faculty of Medicine of the Charles University and General University Hospital, Prague
Email: antonin.sipek@lf1.cuni.cz
Vladimir Gregor, MD
Department of the Medical Genetics, Thomayer University Hospital, Prague
Email: vladimir.gregor@ftn.cz
Updated 16.02.2023