Objective: A review of the premature ovarian failure syndrome. Design: Review article. Setting: Department of Obstetrics and Gynaecology, Charles University, Prague, Czech Republic. Summary: The incidence of premature ovarian failure (POF) is reported to vary between 0.9% and 3%. Most often, the syndrome is manifested by amenorrhoea followed by typical postmenopausal symptoms caused by oestrogen deficiency. The only diagnostic criteria of the syndrome are FSH levels > 40 IU/L obtained twice at least one month apart in women below 40 years of age. The cause of the disease remains obscure in most women. The most often identifiable cause is an autoimmune disorder of the follicular apparatus with a genetic cause established less frequently. All POF women should be examined for other possible autoimmune diseases and women below 30 years of age should have their karyotype identified. Despite the possibility of preserved follicular activity, the probability of pregnancy is very low in those women. Induction of ovulation is virtually ineffective. The diagnosis of POF is a clear indication for long-term oestrogen-progesta- gen replacement therapy even in the absence of symptoms of oestrogen deficiency.

        Key words: premature ovarian failure, fragile X syndrome, autoimmune disease