The Occurence and Survival of Children with Selected
Types of Birth Defects in the Czech Republic
in the Period of 1994-2001 (Part 1)
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. 2Institut postgraduálního vzdělávání ve zdravotnictví, Katedra gynekologicko-porodnická, Praha, vedoucí 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 – anencephaly, spina bifi da, encephalocele,
neural tube defects, congenital hydrocephalus, Down syndrome, were investigated.
Type of study: Retrospective analysis from registry of birth defects in the UZIS 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
3,572 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 on the average 72.5% in neural tube and the highest in
anencephaly – more than 96%. In cases of congenital hydrocephalus it was higher than 45% and in
Down syndrome it represented 56.5%. The survival in children with neural tube defects was 82.5% on the average, being obviously zero in anencephaly. The children with congenital hydrocephalus
survive in more than 75% and those with Down syndrome survive in almost 95%.
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 morbidity.
Key words:
birth defect, neural tube defects, anencephaly, spina bifi da, encephalocele, congenital
hydrocephalus, Down syndrome
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