Management should begin with counseling and reassurance. If possible, underlying causes of pubertal delay should be treated. If this is not possible, management is either directed towards puberty induction with short courses of low dose sex steroids in those with reversible delayed puberty, or to long-term sex hormone replacement therapy (Box 5).
Short courses of sex steroids induce puberty which then continues spontaneously, albeit sometimes at a slower rate. This is usually enough to result in the pubertal height spurt, development of secondary sex characteristics, and accretion of bone mass.
Issues to consider around the timing of treatment are: prepubertal growth and any concomitant GH therapy; psychological impact including delayed development of adolescent tasks; and the potential long-term effect of a prolonged delay till puberty induction on bone mass accretion.
The decision to treat or not to treat, and if so when, should be made in conjunction with the young person. Treatment is therefore individualized, taking into account all aspects of the young person's life, including health status and psychological effects, along with intended benefits.
The main error to avoid with treatment is accelerating the bone age faster than the chronological age, and thereby reducing final height. This error can be avoided with short, low-dose courses of sex steroids.
For males older than 14 years, a short course (3-6 months) of low-dose testosterone or androgens will often be enough to induce puberty, initiating changes of the external genitalia (penis and scrotum). Any increase in testicular size during treatment indicates the onset of normal puberty. Occasionally a second course may be required if puberty does not commence following the first course and a period of observation. The common regimes include:
■ testosterone enanthate, 50 mg IM monthly for 4-6 months;
■ oxandrolone, 2.5 mg orally daily for 3-6 months.
Box 5 Treatment of Delayed Puberty
■ Primary objective is disease control
■ Ensure adequate caloric intake and nutrition
■ Counsel and reassure
■ Use short courses of low-dose sex steroids to induce puberty (in consultation with the young person), avoiding acceleration of bone maturation
Those with permanent gonadotrophin deficiency will require long-term hormone replacement therapy starting usually around the average age of puberty. Testosterone enanthate is given IM every four weeks, starting at 50 mg and increasing by 50 mg every six months to a maintenance dose of 200-300 mg 3-4 weekly.
Short courses of treatment for pubertal induction are less commonly given to females, with reassurance and padded bras often being satisfactory unless puberty is markedly delayed. When treatment is considered low-dose androgens, such as oxandrolone orally daily for six months, may initially be used in the presence of short stature and a bone age of less than 11 years (35). Puberty (and feminization) can then be induced with ethinyl oestradiol 2 ^g daily orally (or with a depot estradiol preparation) for six months.
If ongoing hormone replacement is required, the dose of ethinyl oes-tradiol is doubled every 6 to 12 months to 20 ^g, simulating normal pubertal progression. Menarche usually occurs after two to three years of treatment, at which time a progestin, such as medroxyprogesterone acetate (MPA) can be added to cyclical estrogen. For example, give 5-10 mg of MPA daily for the last 5-7 days of a 21-25 day estrogen cycle per month. On attainment of final height, a low-dose estrogen combined oral contraceptive pill is then usually preferred (35).
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