Pituitary-gonadal function in three relatives presenting with Kallmann's syndrome

The assumption that abnormalities exist in both the pituitary and gonads was tested in 3 familial cases of Kallmann's syndrome, 2 brothers, aged 16 and 17 years, and their cousin, aged 26 years. Serial serum and urinary LH and FSH titers were measured after subcutaneous administration of 100 micrograms LRH and in the 2 brothers after a second test using 400 micrograms LRH. Serum testosterone and estradiol were estimated before and 60 and 90 min after LRH and also before and on the 3rd and 4th day after daily administration of 5,000 IU of hCG for 3 days. Prolactin was also measured in blood samples of all LRH tests and found normal in 2 cases and increased in 1. Tomograms of the pituitary fossa were normal in this patient. With all procedures used, a distinct stimulatory effect on the pituitary and the testes failed to appear in the patients of the present study.