The Occurence and Survival of Children with SelectedTypes of Birth Defects in the Czech Republicin the Period of 1994-2001 (Part 1)

Authors: A. Šípek 1,2;  V. Gregor 3,4;  J. Horáček 3,4;  D. Mašátová 5;  K. Světnicová 3
Authors‘ workplace: Ústav 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 Thomaye 1
Published in: Čes. Gynek.2004, , č. 1 s. 59-65


Analysis of prenatal and postnatal incidence of selected types of birth defects in theCzech Republic in the period of 1994-2001. In the postnatally diagnosed cases the survival in thecourse 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 inthe period of 1994-2001.Method: The authors analyzed frequency of prenatally and postnatally diagnosed cases of 6 selectedtypes of birth defects in the period of 1994-2001 in the Czech Republic. In cases where thediagnosis was established after birth, analysis of survival and death rate during the fi rst year oflife was performed for selected birth defects.Results: In the period of 1994-2001, 745,410 children were born in the Czech Republic. There were3,572 children with one or more birth defects diagnosed in this cohort during the fi rst year oflife. The mean incidence of all cases in this period of observation was 304.52 per 10,000 live bornchildren. The percentage of prenatally diagnosed cases, ended for this diagnosis in the abovementioned period of observation, was on the average 72.5% in neural tube and the highest inanencephaly – more than 96%. In cases of congenital hydrocephalus it was higher than 45% and inDown 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 hydrocephalussurvive 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 presentlylower than in previous years particularly due to successful prenatal diagnostics. Cases,where the diagnosis is established after delivery, represent an important part of perinatal, neonataland post-neonatal mortality and morbidity.

Key words:
birth defect, neural tube defects, anencephaly, spina bifi da, encephalocele, congenitalhydrocephalus, Down syndrome

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Paediatric gynaecology Gynaecology and obstetrics Reproduction medicine

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Czech Gynaecology

2004 Issue 1

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