Výskyt a přežívání dětí s vybranými typyvrozených vad v České republicev období 1994-20012. část

The Occurence and Survival of Children with Selected Types ofBirth Defects in the Czech Republic in the Periodof 1994-2001. Part 2

Objective:
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 – 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 Informationand Statistie of the Czech Republic in the 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 were22 711 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 66% in abdominal wall defects, 55% in omphalocele and77% in gastroschisis. In cases where the diagnosis was established after birth, 93.55% of childrensurvived the fi rst year of life. In cases of birth cystic kidney 25% were diagnosed prenatally andended for renal agenesis and hypoplasie of the kidneys and 27% due to cystic kidney. In cases ofdiaphragmatic hernia, 31% of cases were diagnosed an ended prematurely after positive prenataldiagnosis, 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 presentlylower than in previous years particularly due to successful prenatal diagnostics. Caseswhere the diagnosis is established after delivery represent an important part of perinatal, neonataland post-neonatal mortality and morbility.

Key words:
birth defect, abdominal wall defect, omphalocele, gastroschisis, cystic kidney defects,diaphragmatic hernia