Amenorrhea related to progestin excess in congenital adrenal hyperplasia

Studies of the cause of amenorrhea in a patient with congenital adrenal hyperplasia (CAH) whose androgen levels were easily controlled suggested that inhibition of menses in CAH is more closely related to overproduction of progestins than to overproduction of androgens. Supporting this concept was the observation in another patient with CAH that menarche was associated with improved control of the plasma progesterone. Subsequent detailed investigation of the diurnal plasma steroid pattern of other treated CAH patients indicates that major intermittenet spisodic bursts of progestin secretion occur in many patients whose plasma androgen levels are well controlled. Estimation of 17-hydroxyprogesterone (17-P) production, by measurement of serum 17-P or urinary y pregnanetriol, provides the best index of biochemical control of the 21-hydroxylase deficiency form of CAH. In patients in whom poorly controlled progestins are possible contributers to abnormal menses, low-dose dexamethasone (about 0.25 mg/m2/day) in 1 or 2 divided doses seems to be the treatment of choice.