Abstract
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 occurence 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.