The choice of therapy for GnRH insensitivity depends upon the patient's age and desire to achieve one or more of the following goals: * Induction of puberty and/or maintenance of sexual maturation * Induction or restoration of fertility Puberty induction and sexual maturation Girls and women — Exogenous estrogens are used to start secondary sexual development in prepubertal girls and to build and sustain normal bone and muscle mass. Initiation of treatment are based upon the patient's bone age, current height percentiles, psychosexual needs, and predicted adult height. The shorter the predicted adult height, the later puberty should be induced. Inappropriate use of estrogens may result in rapid osseous maturation with resulting short stature and irregular menstrual bleeding. Initiation of puberty can begin with any type or route of exogenous estrogen, oral or transdermal. Initiation of puberty with transdermal 17-beta estradiol, starting with low doses of approximately 0.08 to 0.12 mcg estradiol per kg/day body weight, is successful and commonly prescribed by pediatricians. The dose is then gradually increased over several years. Initial therapy consist of unopposed estrogen alone to maximize breast growth, achieve appropriate skeletal maturation, and to induce uterine and endometrial proliferation. A progestin eventually needs to be added to prevent endometrial hyperplasia, but adding it prematurely or administering combinations of estrogens and progestins (eg, birth control pills) before completion of breast development should be avoided because it is likely to reduce ultimate breast size. Once pubertal induction is completed, estrogen and progestin therapy are continued indefinitely. Doses and principles of therapy are similar to those for women with primary ovarian insufficiency. Boys and men — In boys, puberty can be induced with testosterone, exogenous<unk> s, or pulsatile<unk><unk>  hormone (GnRH) therapy. The latter two options also induce spermatogenesis, which is not necessary for this age group. Testosterone therapy is suggested for pubertal induction in boys. The goals of therapy are to: * Induce virilization * Promote optimal skeletal maturation (with bone age monitoring) * Maximize adult height * Promote psychosexual development * Build and sustain normal bone and muscle mass Oral testosterone preparations should not be used, because of hepatic toxicity. The choices for testosterone replacement include intramuscular injections of long-acting testosterone preparations or topical gels/solutions/patches. Serum testosterone levels should be monitored and dose adjusted. Whichever form of testosterone replacement is chosen, providing psychological support is important because the patient will have a variety of new and often confusing symptoms, much like an adolescent undergoing puberty but more difficult because it will likely be at a later age. Testosterone therapy should be initiated at a low dose and gradually increased to an adult dose over a few years. Once pubertal induction is completed, testosterone therapy is continued indefinitely.
Gonadotropin-releasing hormone insensitivity
Follicle-stimulating hormone insensitivity
Mild androgen insensitivity syndrome
Partial androgen insensitivity syndrome
Kallmann syndrome
Complete androgen insensitivity syndrome
Adrenocorticotropic hormone deficiency
Androgen insensitivity syndrome