Treatment algorithm

Please note that formulations/routes and doses may differ between drug names and brands, drug formularies, or locations. Treatment recommendations are specific to patient groups: see disclaimer

ACUTE

constitutional delay

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observation and monitoring

Patients need monitoring to assess for progression of pubertal signs and an increased height velocity.

Treatment is reserved for patients with severe delay or abnormalities of psychosocial adjustment.

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short course of testosterone

Some boys have severe delay or abnormalities of psychosocial adjustment.

These patients may benefit from a short course of depot testosterone. The aim of low-dose testosterone is to activate puberty. Once activated, puberty often progresses spontaneously despite withdrawal of treatment.

Adverse effects associated with testosterone treatment include erythrocytosis, weight gain, and prostate hyperplasia.[1]​ While these are rare, they should be monitored during treatment.

Treatment course: 3 to 6 months.

Primary options

testosterone cypionate: consult specialist for guidance on dose

OR

testosterone enanthate: consult specialist for guidance on dose

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observation and monitoring

Patients need monitoring to assess for progression of pubertal signs and an increased height velocity.

Treatment is reserved for patients with severe delay or abnormalities of psychosocial adjustment.

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short course of estrogen

Some girls have severe delay or abnormalities of psychosocial adjustment and may benefit from a short course of estradiol therapy.

Treatment course: 3 to 6 months.

Primary options

estradiol transdermal: consult specialist for guidance on dose

OR

estradiol: consult specialist for guidance on dose

organic (permanent) cause: boys

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pubertal induction with testosterone or gonadotropin therapy

Pubertal induction is carried out gradually, with a low starting dose of testosterone therapy that is increased until adult levels are reached. This can be achieved with intramuscular testosterone preparations administered every 4 weeks. The dose is gradually increased, as required by the patient, every 6 months once an adult dose is reached. A decreasing interval between injections (2-3 weekly) may be necessary if the patient becomes symptomatic of hypogonadism 2-3 weeks after each injection.

The serum levels of testosterone achieved through the intramuscular route are not physiologic. Some boys report symptoms of irritability, aggression, and hypersexuality in the days following the injection. Other adverse effects associated with testosterone treatment include erythrocytosis, weight gain, and prostate hyperplasia.[1]​ While these are rare, they should be monitored during treatment. Serum total testosterone levels should be checked 1 week after therapy to assess whether the doses are therapeutic. 

In males with gonadotropin deficiency, pubertal induction can alternatively be with gonadotropin therapy.[57] As with testosterone, doses are increased gradually to induce puberty over 1 to 3 years. Combination therapy with recombinant follicle-stimulating hormone (rFSH) and human chorionic gonadotropin (hCG) are most commonly used. Careful monitoring is required to target physiologic serum concentrations of FSH and testosterone, and such regimes should be limited to experienced centers.[58] Consult your local protocols for guidance on dose regimens.

Primary options

testosterone cypionate: consult specialist for guidance on dose

OR

testosterone enanthate: consult specialist for guidance on dose

organic (permanent) cause: girls

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pubertal induction with estrogen

Patients require gradually increasing doses of estrogen treatment, with cyclic progesterone therapy introduced once adequate estrogenization has occurred or with breakthrough uterine bleeding. Puberty should not be induced using oral contraceptive pills or patches because the doses of estrogen are too high and the androgenic progestins impair optimal breast development.

The preferred preparations are either transdermal or oral estradiol in the smallest available dose. Transdermal delivery avoids first-pass effects on the liver.[59]

Primary options

estradiol transdermal: consult specialist for guidance on dose

OR

estradiol: consult specialist for guidance on dose

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cyclic progesterone after breakthrough bleeding or adequate estrogenization

Treatment recommended for ALL patients in selected patient group

Progesterone is added to estrogen therapy after a suitable duration of unopposed estrogen (usually 2-3 years) or if more than one episode of significant breakthrough bleeding occurs, to promote endometrial shedding. Many different formulations are available; consult your local drug formulary for more information.

chronic illness or malnutrition

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treatment of underlying abnormality

Malnutrition, eating disorders, chronic heart disease, moderate to severe asthma, cystic fibrosis, celiac disease, inflammatory bowel disease (Crohn disease and ulcerative colitis), inflammatory disorders (e.g., juvenile idiopathic arthritis), chronic renal failure, any chronic malignancy, and poorly controlled diabetes mellitus can all cause temporary delay in puberty.

Treatment of the underlying cause may resolve the pubertal delay, with subsequent spontaneous pubertal progression.

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short course of testosterone

Some boys have abnormalities of psychosocial adjustment and may benefit from a short (3- to 6-month) course of depot testosterone. The aim of low-dose testosterone is to activate puberty. Once activated, puberty progresses spontaneously despite withdrawal of treatment.

Adverse effects associated with testosterone treatment include erythrocytosis, weight gain, and prostate hyperplasia.[1]​ While these are rare, they should be monitored during treatment.

Treatment course: 3 to 6 months.

Primary options

testosterone cypionate: consult specialist for guidance on dose

OR

testosterone enanthate: consult specialist for guidance on dose

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treatment of underlying abnormality

Malnutrition, eating disorders, chronic heart disease, moderate to severe asthma, cystic fibrosis, celiac disease, inflammatory bowel disease (Crohn disease and ulcerative colitis), inflammatory disorders (e.g., juvenile idiopathic arthritis), chronic renal failure, any chronic malignancy, and poorly controlled diabetes mellitus can all cause temporary delay in puberty.

Treatment of the underlying cause may resolve the pubertal delay with subsequent spontaneous pubertal progression.

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short course of estrogen

Some girls have abnormalities of psychosocial adjustment and may benefit from a short course of estradiol therapy.

Treatment course: 3 to 6 months.

Primary options

estradiol transdermal: consult specialist for guidance on dose

OR

estradiol: consult specialist for guidance on dose

ONGOING

persistent hypogonadism postpuberty

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testosterone supplementation

Those with confirmed hypogonadism postpuberty need lifelong testosterone supplementation. Options include intramuscular, oral, topical, or transdermal testosterone. Many formulations are available; consult your local drug formulary for more information.

Local skin irritation is the main adverse effect of transdermal preparations and is more frequent with the patches compared with the gel preparation. Showering and swimming should be avoided for 4 hours after gel application. Some patients who do not tolerate these preparations and have severe local skin irritation may be considered for intramuscular testosterone.

Adverse effects associated with testosterone treatment include erythrocytosis, weight gain, and prostate hyperplasia.[1]​ While these are rare, they should be monitored during treatment.

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testicular implants

Treatment recommended for SOME patients in selected patient group

Males with anorchidism can be counseled for testicular prosthesis. This does not restore fertility and is done for cosmetic and/or psychological concerns. The procedure is done in the latter stages of puberty, when scrotal growth is optimum, so that the procedure does not need to be revised.

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ovarian hormone supplementation

All women with confirmed hypogonadism postpuberty need lifelong hormone-replacement therapy (HRT) after induction of puberty. HRT should continue until about 50 years of age. This can be achieved with transdermal estradiol along with micronized progesterone, or a combination of transdermal estradiol and other progesterone preparations. Another option is to replace transdermal estrogen with oral estradiol. Many formulations are available; consult your local drug formulary for more information.

Inadequate estrogen treatment or a lack of estrogen treatment is associated with an increased risk of long-term complications such as osteoporosis.

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breast implants

Treatment recommended for SOME patients in selected patient group

Girls who do not get adequate breast development with estrogen treatment may require breast implants. The procedure is offered for cosmetic and psychological reasons and done at the end of or latter stages of puberty.

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Please note that formulations/routes and doses may differ between drug names and brands, drug formularies, or locations. Treatment recommendations are specific to patient groups. See disclaimer

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