GONADAL-FUNCTION IN GALACTOSEMICS AND IN GALACTOSE-INTOXICATED ANIMALS

Gonadal dysfunction, specifically hypergonadotropic hypogonadism, in f emale galactosemics is an almost universal finding. Minimal transferas e activity may modulate ovarian function and the time of menopause. In contrast, male galactosemics have a relatively low risk of gonadal dy sfunction. Animal models have variously suggested prenatal and postnat al insults which may play a role in the gonadal pathology. Several can didate toxic states may be involved. Current dietary restrictions are inadequate to prevent ovarian failure. Until such time as the pathophy siology is better understood, therapy will remain palliative and suppo rtive. Exogenous estrogen and progesterone have roles in assisting pub ertal changes and in prevention of the sequelae of a post-menopausal s tate.