Background and Methods Persons with birth defects are at highrisk for death during the perinatal period and infancy. Lessis known about the later survival or reproduction of such persons.We studied a cohort that comprised 8192 women and adolescentgirls with registered birth defects and 451,241 women and adolescentgirls with no birth defects, all of whom were born in Norwayfrom 1967 through 1982. The rate of survival was determinedthrough 1992, and the rate of childbearing was determined throughOctober 1997. We also estimated the risk of birth defects inthe children of these subjects.
Results Among the subjects with birth defects, 80 percent survivedto 15 years of age, as compared with 98 percent of those withno birth defects. Among the surviving subjects, 53 percent ofthose with birth defects gave birth to at least one infant bythe age of 30 years, as compared with 67 percent of those withno birth defects. The subjects with birth defects were one thirdless likely to give birth by the age of 30 than those with nobirth defects. The children of the subjects with birth defectshad a significantly higher risk of birth defects than the childrenof those with no birth defects (relative risk, 1.6; 95 percentconfidence interval, 1.3 to 2.1). This increased risk was confinedentirely to the specific defect carried by the mother, withthe relative risk of recurrence varying from 5.5 to 82 accordingto the defect. In contrast, there was no increase in the riskof having an infant with a different type of defect.
Conclusions Women and girls with birth defects have decreasedsurvival as compared with those with no birth defects, especiallyin the first years of life, and are less likely to have children.In addition, they have an increased risk of having childrenwith the same defect.
Source Information
From the Section for Medical Statistics and the Medical Birth Registry of Norway, University of Bergen, Bergen, Norway (R.S., R.T.L.); and the National Institute of Environmental Health Sciences, Research Triangle Park, N.C. (A.J.W.).
Address reprint requests to Dr. Skjærven at the Section for Medical Statistics, University of Bergen, Armauer Hansens Bldg., 5021 Bergen, Norway, or at rolv.skjaerven{at}smis.uib.no.
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