Abstract
Hypogonadotropic hypogonadism (HH), consequent to congenital or acquired disorders of the hypothalamic-pituitary axis, presents as absent/delayed/arrested sexual maturation and infertility. Optimal management includes: (a) confirmation of the diagnosis and prognosis, (b) timing and choice of therapeutic intervention and (c) consideration of future fertility prospects. Therapy is usually initiated with testosterone to induce development of secondary sexual characteristics, taking the patient (often diagnosed late) through puberty. Monitoring of the impact of the condition on long-term health and psychosocial function is necessary. Treatment is likely to be life-long, requiring regular monitoring for its optimization and avoidance of adverse responses. Induction of spermatogenesis requires either pulsatile gonadotropin releasing hormone (GnRH) or gonadotropin administration. Gonadotropins can be self-administered subcutaneously and are not inferior to the more costly GnRH. 'Reversible genetic hypogonadotropic hypogonadism' is a recently described entity which has implications for the long-term management of patients with HH.
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