We report the descriptive epidemiology of holoprosencephaly and arhinencephaly using data from the Metropolitan Atlanta Congenital Defects Program, a population-based birth defects surveillance system with multiple sources of ascertainment.
From 1968-1992, we ascertained 63 cases of holoprosencephaly and arhinencephaly from approximately 734,000 births, for a birth prevalence of 0.86 per 10,000.
Thirteen case infants with holoprosencephaly and four case infants with arhinencephaly were categorized as having syndromes.
Of the case infants with non-syndromic holoprosencephaly, 55% had malformations not attributable to the underlying brain defect.
The rate of holoprosencephaly and arhinencephaly increased from 0.58 per 10,000 during 1968-1972 to 1.2 per 10,000 during 1988-1992 (P for trend=0.016).
Rates were higher for females than for males (risk ratio=1.45,95% C.I. 0.88-2.41) and higher for nonwhites than for whites (risk ratio=1.74,95% C.I. 1.06-2.86).
There was a U-shaped distribution of risk associated with maternal age with a slightly increased risk for younger women (risk ratio for maternal age<20 years, compared with age 25-29 years=1.68,95% C.I. 0.77-3.62) and older women (risk ratio for maternal age>34 years, compared with age 25-29 years=2.30,95% C.I. 0.93-5.7), but this was not statistically significant.
The increased risk in the older age group could be largely explained by the presence of cases with autosomal trisomies. (...)
Mots-clés Pascal : Arhinencéphalie, Epidémiologie, Etats Unis, Amérique du Nord, Amérique, Nourrisson, Homme, Système nerveux pathologie, Système nerveux central pathologie, Encéphale pathologie, Maladie congénitale, Malformation, Holoprosencéphalie
Mots-clés Pascal anglais : Arhinencephaly, Epidemiology, United States, North America, America, Infant, Human, Nervous system diseases, Central nervous system disease, Cerebral disorder, Congenital disease, Malformation, Holoprosencephaly
Notice produite par :
Inist-CNRS - Institut de l'Information Scientifique et Technique
Cote : 97-0126657
Code Inist : 002B17D. Création : 21/05/1997.