The 24-hr urinary excretions of follicle-stimulating hormone (FSH) and luteinizing hormone (LH) were measured in seven males with untreated congenital adrenal hyperplasia. The patients aged 1–10 years had urinary FSH concentrations of 4.1–6.7 IU/day (normal = less than 2.5 IU/day). Elevated levels of 13.6 IU/day were found in the 81-year-old patient. During suppressive steroid therapy, the FSH excretion fell to the normal range in the one so studied; therapy was reduced in two patients and their FSH excretion rose by 20–30% in a 4-month period. The FSH production rate was 41.5 and 40.2 IU/day in the 1-and 6-year-old patients before therapy (these levels being at the upper limit of the normal range for adults). After 2.5 years of the FSH production rate in one of them fell to 10.3 IU/day.The excretion of LH was elevated for age and was 5.9 and 11.8 IU/day in the 1− and 6-year-old untreated patients (normal therapy, range = less than 3 IU/day). After suppressive therapy for 2.5 years, the LH excretion in the 1-year old fell only to 4.7 IU/day. In two patients, therapy was reduced for 4 months and the LH excretion actually fell from 5.1 to 2.9 and 10.0 to 7.4 IU/day. Similarly, the 81-year-old untreated adult male showed an LH excretion of 16.4 IU/day, which is lower than the normal adult range of 22.3–45.1 IU/day. The LH production rates in the 1− and 6-year-old patients before therapy were 166 and 141 IU/day, respectively both being lower than the range of 247.7–464.5 IU/day found in normal adult males.SpeculationThe increased FSH production and excretion could be due to hypothalamic-pituitary stimulation by increased production of androgens or their precursors or from increased estrogens from the adrenal glands, or even to abnormal metabolites such as 17-hydroxy progesterone.