Abstract

Abstract Three siblings, 2 females and 1 male, had eunuchoidism with selective insufficiency of pituitary gonadotropin. A fourth sibling, a male, probably was affected, but death precluded definitive study. A maternal half sister was normal. There were no distinctive somatic features except absence of secondary sex characteristics and relatively long extremities in proportino to height. No other associated congenital defects were observed. Chromosome constitutions were normal for their phenotypes. Consanguineous parental mating between second cousins once removed was probably causative. Evidence for autosomal recessive inheritance is presented.

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