Gonadal function in galactosemics and in galactose-intoxicated animals.

Gonadal dysfunction, specifically hypergonadotropic hypogonadism, in female galactosemics is an almost universal finding. Minimal transferase activity may modulate ovarian function and the time of menopause. In contrast, male galactosemics have a relatively low risk of gonadal dysfunction. Animal models have variously suggested prenatal and postnatal insults which may play a role in the gonadal pathology. Several candidate toxic states may be involved. Current dietary restrictions are inadequate to prevent ovarian failure. Until such time as the pathophysiology is better understood, therapy will remain palliative and supportive. Exogenous estrogen and progesterone have roles in assisting pubertal changes and in prevention of the sequelae of a post-menopausal state.