The Occurence and Survival of Children with Selected Types of
Birth Defects in the Czech Republic in the Period
of 1994-2001. Part 2
Šípek A.1,2, Gregor V.3,4, Horáček J.3,4, Mašátová D.5, Světnicová K.3
1Ústav pro péči o matku a dítě, Praha, ředitel doc. MUDr. J. Feyereisl, CSc. 2Katedra gynekologicko-porodnická, Institut postgraduálního vzdělávání ve zdravotnictví, Praha, vedoucí katedry doc. MUDr. J. Feyereisl, CSc. 3Oddělení lékařské genetiky, Fakultní Thomayerova nemocnice, Praha, ředitel Ing. J. Pubrdle 4Katedra lékařské genetiky, Institut postgraduálního vzdělávání ve zdravotnictví, Praha, ředitel MUDr. A. Malina, PhD., 5Ústav zdravotnických informací a statistiky České republiky, Praha, ředitelka Mgr. V. Mazánková |
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Summary:
Objective: Analysis of prenatal and postnatal incidence of selected types of birth defects in the
Czech Republic in the period of 1994-2001. In the postnatally diagnosed cases the survival in the
course of the fi rst year of life was analyzed. Selected birth defects – omphalocele, gastroschisis,
abdominal wall defects, renal agenesis and hypoplasie, cystic kidney disease, diaphragm hernia.
Type of study: Retrospective analysis from registry of birth defects in the Institute of Health Information
and Statistie of the Czech Republic in the period of 1994-2001.
Method: The authors analyzed frequency of prenatally and postnatally diagnosed cases of 6 selected
types of birth defects in the period of 1994-2001 in the Czech Republic. In cases where the
diagnosis was established after birth, analysis of survival and death rate during the fi rst year of
life was performed for selected birth defects.
Results: In the period of 1994-2001, 745 410 children were born in the Czech Republic. There were
22 711 children with one or more birth defects diagnosed in this cohort during the fi rst year of
life. The mean incidence of all cases in this period of observation was 304.52 per 10 000 live born
children. The percentage of prenatally diagnosed cases, ended for this diagnosis in the above
mentioned period of observation, was 66% in abdominal wall defects, 55% in omphalocele and
77% in gastroschisis. In cases where the diagnosis was established after birth, 93.55% of children
survived the fi rst year of life. In cases of birth cystic kidney 25% were diagnosed prenatally and
ended for renal agenesis and hypoplasie of the kidneys and 27% due to cystic kidney. In cases of
diaphragmatic hernia, 31% of cases were diagnosed an ended prematurely after positive prenatal
diagnosis, and about one third of affected children died during the fi rst year of life.
Conclusion: Incidence of these types of birth defects in newborns in the Czech Republic is presently
lower than in previous years particularly due to successful prenatal diagnostics. Cases
where the diagnosis is established after delivery represent an important part of perinatal, neonatal
and post-neonatal mortality and morbility.
Key words:
birth defect, abdominal wall defect, omphalocele, gastroschisis, cystic kidney defects,
diaphragmatic hernia
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