This condition is due to the lack of the hypothalamic decapeptide gonadotropin-releasing hormone (GnRH), When associated with midline defects such as anosmia, it is called Kallmann's syndrome. Very low or undetectable serum levels of LH, FSH and T in a prepubertal appearing adult confirms the diagnosis. Treatment for the development of secondary sexual characteristics and maintenance of libido is Depo-Testosterone. For induction of spermatogenesis, the Depo-Testosterone is discontinued. Human chorionic gonadotropin (hCG) 1500 IU 3 times weekly is begun. After 3-6 months of hCG therapy, when serum T,Ievels are in the normal range, human menopausal gonadotropin (hMG) 25-75 IU 3 times weekly is added. Sperm usually begin to appear in the ejaculate 6-18 months after initiation of therapy. Testis size and sperm counts remain lower than normal, but pregnancies occur regularly with sperm densities in the 2-6 million per mL range. Men who fail to respond to gonadotropin replacement may respond to pulsatile administration of GnRH by pump.
