PUBERTY AND ITS DISORDERS.
Puberty
marks the change from childhood to adolescence.It is associated with the development of breast, secondary sexual characteristics and the onset of
menstruation. At the same time, there is a period of accelerated growth. The
gynaecologists are consulted when these changes are delayed. The pediatrician
will take care of the cause of precocious puberty.
Causes
of precocious puberty
1.
Idiopathic
2.
Mc cune Albright syndrome :
A)
Café – au-lait spots.
B)
Polyostitic fibrous dysplesis
3 Tummors of the adrenal or ovary, producing
tummors
4 Cerebral tumours
5 Ingestion of exogenous estrogens
The age at which the changes
take place is variable, but it is abnormal for there to be no sign of 2o that at the age of 14years.
Physiology
of puberty
The trigger for the changes to start
is an increasing frequency and amplitude of pulse of genaclotropin release. The
ovaries are the stimulate to produce oestrogen, the acts on the breast tissue
to stimulate growth. This usually begins at 9yrs and takes about 5yrs. There is
evidence to suggest that it begins earlier, respiration in African- American
girl’s, prompting a re-assessment of the age at is precocious puberty should be
uninvestigated.
Public hair growth is stimulated by androgens
released by the every and adrenal gland. Breast and public hair duet. Is
described in 5 stages it the classification by marshal and tenure.
|
Stage
|
Breast
|
Public
Hair
|
|
I
|
Preadolescent
elevation of papilla only
|
No
public hair
|
|
2
|
Breast
bred-elevation of breast and papilla small mound; enlargement of are her
diameter
|
Sparse
growth of long downy hair along the labia .
|
|
3
|
Further
enlargement, but no separation of the contuses
|
Hair
is coarser, darker to more coiled, over mores.
|
|
4
|
Projection
of the aerial and papilla to form 2 mounds above the level of the
breast.
|
Adult
type hair but no spread to thighs.
|
|
5
|
Motive,
areola recessed to the general contour of the breast
|
Adult,
the horizontal upper border and spread to the thighs.
|
Growth
charts indicate the range of normal ages at is see changes are attained. In
most girls, breast duet start 6/4 growth of public hair. Even before these
changes are abvoius, there is an acceleration of growth is frequently
accompanied by a rapid in the size (growth spurt). The peak height velenity of
appnox 8cm/yrs occurs just before the inset of menses. ( on avg around the age
of 12yrs). Oestrogen primates costive of the epiphyses. So final height is
attained about 2yrs after menarche.
Menarche
occurs 2.3+ 1yrs after the onset
of breast devt. The avg age of menarch has declined and is now 12.8yrs in while
girls and 12.16yrs in African-Americans.
The
factors involved include: Improved nutrition and genetic inflverces; daughters
of ten undergo menarche at a similar age to hair motheslvitially menstrual
cycles are usually anovuletony and irregular for several years.
Delayed puberty
Most
referrals to gynecologists are because of concerns about delay in the onset of
puberty. In other to determine the likely cause, it is ist unipt to determine
and puberty itself is delayed.
A
detailed hx should be taken, asking about.
General
health.
Age
at is breast and public hair diet started if the girl has had a growth spur or
still appears to be growing. Any chronic illness may lead to constitutional
delay in puberty. For ovulation to occur, a person should weigh up to 45kg the
25% of body fat.
For
menstruation to occur a person should have up to 16% of body fat. Teenage girl
may be reluctant to answer question and the mother gives much of the hx, but it
is impt to address the girl, rather than talking to the mother
Exam
should include accurate measurement of height, together the assessment of the
stage of breast and public hair diet. This should be plotted on growth charts.
Exam
should be sensitively performed.
Ask
the girl if she wants the mother to be present, as some fed more embarrassed
the mother there, and only exposed one part of the body at a time.
An
internal exam is not required; inspection of the external genitalia is all that
is required (esp. the presence of USS, is can take care of further assessment).
Investigation
usually includes
Measurement
of genadotrophins
Measurement
of Oestrogens
Kanyotyping
USS
of pelvis, to confirm presence of uterus and ovary.
X-ray
to determine bone age.
Additional
becalm dashes to asses thyroid probation and 17x-hydrom xylems is also
important.
Absent
breast and public hair
When both breasts and public hair
are absent (hypogonadotroplic
hypogenadisn)
-
majority of girl the low
genaelotrophuins have constitution delay
in puberty. It may be 2 to chronic illnesses e.g lystic fibrosis
-
improvement in the underlying
condition usually result in catch up growth. Girls the anorexia-nervosa have
levels of gonaolotrophines x if the problem starts at a young age they will
have absent or poorly devt sexual extensities. A similar e.g. is seen in
athletic girls (gymnastics) who have low body weight and low body fat. This can
lead to “ female Athletic trial”, with Disordered eating
amenonhoea
osteopena
and the risk of stress fracture more often seen in boys the delayed puberty.
May also be associated the anosnuia due to hypoplasia of tehe olfactory lobes,
when it is known as Kallman syndrome. Brain unaging (CT scan, MRI) will be
recesany to establish this diagnosis.
Aquired
deficiency may follow damage to the hypothalamus and pituritany as a result of
trauma, trumeur. Infiltration of the organs can also occur in haemochromatosis
is may be 2 to transfusions for SCD or thalessaemia and to wislson’s dz.
In
all these conditions USS will confirm the presence of an immature uterus and
small, inactive ovaries. The bone age will help to differed the cases of
constitutional delay as it will be believed chronological and height age.
TX
may be required if there are no sign of spontaneous onset of puberty although most girls the
constitutional delay will proceed to
normal if left untreated. Pulsatile gonadotroplins have been used but are very difficult to
sustain as they require a substances injection attached to a portable pump for
several months. The more widely used approach is to give low doses of ethanol
estracliol 1-2mg / day for 3-6months. Frequently, spontaneous sexual maturation occurs. If not, the dose is
gradually the ever several years.
Hypergonadotrophic hypogonadism
The gonadotrophin level is devated,
yet no 2 sexual maturation takes place. It occurs when there is failure of
gonadal devt. The normal release of gonadotrophins occurs, but there is no
response five gonads, hence the normal feedback mechemism is inlibited. The
commenest cause is turner’s syndrome (45x0) or other genetic problems. Other
causes in which damage to the ovaries by radiation, surgery, chemotherapy, or
infection.
Galactosaemia
is also associated the ovarian failure and its right presents a challenge, oral
oestrogen and progesterone contain lactose. Antouinrmure ovarian failure may be
associated the other autoinmure disorders, e.g. Adelison’s dz, vitilgo,
thyroiditis.
One
of the less conmmon causes of congenital adrenal hyperplasia with deficiency of
17& – hyroxylase. This engyme is required to produce both oestrogen and
testosterone. So virilization does not
occur at birth, but there is also a failure of devt of 2o sexual
xtenstics.
TX
includes the gradually using levels of oestrogen replacement, combined the
progesterone to induce
withdrawal
bleed, once doses stimulate the devt. Of the endometrium.
Scenario:
full breast and public hair devt the out menses
1.
Anatomical
causes: If puberty has progressed normally the out
menstruation, the commonest cause is anatomical. Its uncommon for girls the an
imperforate clinic. The mostly present as emergencies; the cyclical abdominal
pain, possibly the a palpable admass.
The blockage prevents flow
of menstrual blood and there
usually a tense blue bulge seen at the introits.
USS may show a distended
vagina containing blood and normal fining ovaries when there is a imperforate
hymen, TX is a straight forward perforation of the hymen, to allow for the
outflow of the blood.
TX of a thunder and possibly
higher septum is more complex and best death the in a tertiary health centre as
injudicious excision can result in stricture formation (referral is difficult
to treat) and causes problems in intercourse.
The commonest cause is
Mullenian agenesis.
2. Hyperprolactinaemia:
Often a cause of 2 amenonhoea; may result in 1 amenonhoea; there might be no
golactorrhoea ( The situation in 13% of
cases). A high prolaction level should
prompt investigation for pituitary adenoma.
TX
is the same as in the older females, with dopamine agonists, e.g. cabegoline
the will result in the onset of menstruation.
3. Congental adrenal hyperplasia
(CAH); Menarche is often delayelin this condition, and
when menstruation starts, it may be artic. Poor control of the condition, often
due to poor compliance to tx, may be the cause.
The ovaries also have a polycystic
appearance on seen. Fertility rates in these women are poor for a no of
reasons.
a.
Infrequent ovulation.
b.
Difficulties in achieving penetrative
sex.
c.
Failure to form relationship
Normal breasts but agent or
scanty public hair development.
This is the classic presentation of Androgen
insensitivity Syndrome. The kanyotype will be xy.
Public hair fails to grow because of end organ
insensitivity to androgens but breast devt. Occurs due to peripheral conversion
of androgen to oestrogen. No hormonal TX is required.
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July 08, 2015
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