[Survival in children with birth defects during first year of their life].

OBJECTIVE

To analyze infant mortality in children with selected types of birth defects during a first year of their life.

DESIGN

A retrospective study with an analysis of prenatal and postnatal occurrence of birth defects in the Czech Republic during 1994 - 2006.

SETTING

Department of Medical Genetics, Thomayer's University Hospital, Prague. Chair of Medical Genetics, Postgraduate Medical Institute, Prague.

METHODS

Data were collected from national registers (Institute of Health Information and Statistics) and particular departments of medical genetics. A study of frequency of 14 selected birth defects was performed along with an analysis of survival and mortality in children with birth defects during a first year of their life.

RESULTS

In 1994 - 2006, totally 1 132 567 children were born in the Czech Republic, out of which more than 42 000 with a birth defect. A mean incidence was 339 per 10 000 live births with a maximum of 41458 per 10 000 in 2003. In correctible defects, first year survival was lowest in congenital hydrocephalus (72%) and neural tube defects (anencephaly excluded) (71%). In congenital defects of gastro-intestinal tract and in abdominal wall defects survival was between 82-91%.

CONCLUSION

Birth defects present an important contribution to an infant mortality and morbidity in the first year of life. In a comparison of 1980 - 1993 and 1994 - 2006 periods, a significant increase of prenatal diagnostics efficiency was recorded.