Abstract
Distributions of the incidence of anencephaly in Japan were obtained using nationwide data on fetal and postnatal deaths of individuals with anencephaly during 1969-71. Higher incidences of anencephaly occurred in the urbanized and industrialized areas and lower values in the comparatively sparsely populated highlands. Population size and variance of incidence show a negative association. The distribution of the incidence seems to be binomial, and expected incidences based on binomial distribution indicated good agreement with weighted mean incidences in populations with different sizes. The rate of first cousin marriages among the parents of anencephalic patients is 1.86%, a value not significantly different from the value of general population, 2.13% The inheritance of anencephaly apparently is not related to a specific number of recessive genes.

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