Delayed puberty: management
· Children with CDGP may be treated
with a short course of sex steroid therapy to promote physical development and
growth (see Constitutional delay of growth and puberty).
· Children with permanent
gonadotrophin deficiency or gonadal failure requiring complete induction of
puberty and thus long-term treatment can have puberty induced with gradually
increasing doses of sex steroids over a period of 2–3yrs.
· Boys:
testosterone esters by IM
injection. Incremental increases in dose,
starting from 50mg every 4–6wks to 250mg every 3–4wks.
· Girls:
ethinylestradiol, oral. Increasing
doses every 6mths, starting from
2micrograms/day increasing to 5–20micrograms/day. A progesterone (e.g. norethisterone
or levonorgestrel given on days 14 to 21 of the cycle only) should be added
when the dose of ethinylestradiol is 10–15micrograms/day or when vaginal
bleeding or spotting is first observed.
· The aim of long-term sex steroid
therapy is the maintenance of secondary sexual features, libido, and
menstruation in females. There are also positive benefits in terms of bone
mineralization and cardiovascular health.
Note. In males, testosterone therapy
does not promote testicular growth and
testicular size remains prepubertal unless spontaneous puberty occurs.
This is the most common cause of
delayed puberty. Usually observed in boys, this condition reflects a delay in
the timing mechanisms that regulate the onset of puberty. There is often a
family history of delayed puberty in parents or siblings.
· Children presenting with CDGP are
invariably healthy.
· Onset and progress through puberty
will occur normally with time.
· Children achieve a final adult
height in keeping with their predicted familial target range.
It is likely that most children
with CDGP are not referred for medical attention, as they and their parents
will not perceive that there is a prob-lem. However, for many others, concerns
about the lack of physical devel-opment and the lack of anticipated adolescent
growth spurt would be a source of much anxiety and psychological stress.
There is often evidence of delayed
or slow growth in childhood, which is most pronounced in the peripubertal years
due to lack of anticipated growth spurt. Children will also have evidence of
delayed skeletal matura-tion on bone age assessment.
No specific therapy is required.
For many children and families, explana-tion of the benign nature of the
condition and reassurance that puberty will occur normally is sufficient.
However, children who are experienc-ing signifi cant social or psychological
diffi culties may request treatment. In this situation, low dose sex steroids
may be used (e.g. boys: testosterone, 50mg IM monthly for 4–6mths). This
approach will:
·induce sexual development;
·promote an increase in growth
rate;
·stimulate activation of the
hypothalamic–pituitary–gonadal axis.
Thus puberty may continue once the
administration of sex steroids has been stopped.
Any decision regarding whether
therapy is required or not must include the views of the child and their
parents, who should be part of the deci-sion process.
This indicates impaired
gonadotrophin release from the pituitary gland. Congenital and acquired causes
are recognized. The condition is characterized by low or undetectable
gonadotrophin lev-els either under basal or stimulated (GnRH test) conditions.
Congenital causes of HH may be
characterized by micropenis and unde-scended testis at birth in boys, whereas
in girls physical signs are absent.
A genetic disorder characterized
by the association of HH and anosmia (absent sense of smell). This arises due
to a defect in the co-migration of GnRH releasing neurons and olfactory neurons
that occurs during early foetal development. X-linked, autosomal dominant, and
autosomal reces-sive modes of inheritance are recognized. The X-linked form of
KS results from a mutation in the KAL
gene (encoding the glycoprotein, anosmin-1). It is also characterized by a
range of clinical features including synkinesia (mirror-image movements), renal
agensis, and visual problems as well as craniofacial anomalies, although their
expression is highly variable.
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