Paediatrics: Precocious puberty: management

Precocious puberty: management

Diagnosis

The diagnosis is based on demonstrating progressive pubertal develop-ment and increased growth rate, together with laboratory evidence of increased sex steroid production. Distinguishing central and peripheral PP and PP from other normal variants of pubertal development may be diffi-cult (see Table 13.3). In CPP there is evidence of consonance in sequence of pubertal development in keeping with the normal physiological activa-tion of puberty.

Management

The management of precocious puberty is aimed at the following:

·Detection and treatment of underlying pathological causes of PP: this is especially important in males in whom early puberty is invariably due to organic disease.

·Reducing the rate of skeletal maturation, if necessary: accelerated skeletal maturation and growth rate occur and will result in the affected child being tall during childhood relative to peers. However, skeletal maturation exceeds concominant growth and thus growth potential is reduced, growth is complete prematurely, and final adult height is reduced and potentially below the predicted expected familial target height range.

·Reducing and halting, if necessary, the rate of physical pubertal development.

·Addressing potential behavioural and psychological difficulties: sexual and reproductive characteristics advance inappropriately for age, leading to mature appearance. Early menstruation occurs in girls, and spermatogenesis and ejaculation in boys. Sexualized behaviour may occur and interactions with age-peers and adults may be based on assumed, but age-inappropriate, mental and social expectations.

Before therapy is considered, it is essential that an explanation of the physiology and physical consequences of precocious puberty should be discussed with the parents and the child. The decision on therapy should be made jointly with the parents.

Treatment of precocious puberty

Central PP

·Suppression of the hypothalamic–pituitary–gonadal axis with a long-acting GnRH analogue is the only currently effective treatment for central PP. These agents work by providing continuous stimulation of the GnRH receptor on the pituitary gonadotrophes, resulting in down-regulation of the receptor and thus decreased LH and FSH secretion.

·GnRH analogues are administered by either SC or IM injection, monthly (or 3-monthly in depot preparations).

Treatment efficacy should be assessed by monitoring growth rate and pubertal stage. In addition, serum LH and FSH levels (basal and stimulated) should be measured to ensure hypothalamic–pituitary– gonadal axis suppression.