Variants of normal puberty
These include premature thelarche
and premature adrenarche. Neither condition is associated with pubertal
activation of the hypothalamicâ€“pitu-itaryâ€“gonadal axis.
Â· Isolated premature breast
development occurring in the absence of any other signs of puberty.
Â· Typically, females present in
infancy and usually by 2yrs of age.
Â· Breast development is due to the
action of physiological or mild increases in the amounts of circulating
The clinical course is
characterized by a waxing and waning of breast size, normal growth (height)
rate, and the absence of any further sexual devel-opment. Breast development
may be asymmetrical, and there is usually a resolution of any breast
enlargement by age 4â€“5yrs.
The cause is unknown, but small
increases in basal and stimulated serum FSH levels are usually observed. In
contrast LH levels remain suppressed in the prepubertal range. Ovarian follicle
development is often observed, but no changes in ovarian or uterine size are
seen. Serum oestradiol levels are increased when measured by sensitive assays,
but typically within nor-mal range by standard radioimmunoassay.
The condition is benign. Bone
maturation, age of onset of menarche, and final adult height are not affected.
Management is conservative with re-evaluation of growth and puberty stage at
Â· An intermediate condition between
premature thelarche and central precocious puberty.
Â· It represents a non-progressive
form of early pubertal development.
Patients have evidence of breast
development, increased growth rate, and advanced skeletal maturation on bone
age assessment. There may also be evidence of ovarian enlargement and raised
serum oestradiol levels. For most patients the tempo of progression of pubertal
development will be slow and they will have laboratory findings within normal
range for age. Management is usually conservative with regular re-evaluation of
growth and pubertal status at 3â€“6 monthly intervals. Decisions to treat (as for
central PP) are based on height velocity and final height predictions.
Early onset of pubertal adrenal
androgen secretion is a common variation of normal pubertal development.
Premature adenarche is the result of premature secretion of androgens from the
zona reticualris of the adre-nal gland.
Â·Children typically present with
premature appearance of androgen-dependent s
sexual hair development (axillary hair, pubic hair, or both), acne, and
axillary (body) odour.
Â·Patients may have mild
acceleration in height velocity and slight increase in BA.
Â·Laboratory investigations reveal
an increase in serum DHEAS levels that are appropriate for pubic hair stage
rather for than age.
Â·Serum concentrations of
testosterone and 17-OH progesterone are normal.
When evaluating patients for
premature adrenarche it is important to assess for clinical signs and symptoms
that might indicate another cause of excess androgen production (e.g. adrenal
tumour; congenital adrenal hyperplasia). The later are characterized with signs
of virilization, rapid growth rate, and significantly advanced bone age.
Premature adrenarche is a benign
condition. The timing of onset of true puberty is normal and final adult height
is unaffected. Management is conservative with reassurance after exclusion of
other causes of adrenal androgen excess. Symptomatic treatment may be required
if adrenarche is pronounced, particularly in females who may go on to develop
features of ovarian hyperandrogenism and the polycystic ovarian syndrome.