2. Puber- marriageable age
Pubertus – adulthood
The period of transition between sexual
immaturity and maturity
Puberty is first phase of adolescence.
How puberty occurs ?
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3. Fetal and InfancyFetal and Infancy
During the latter half of fetal life, the
hypothalamus pituitary ovarian axis is
functional completely.
FSH levels are suppressed from 20 weeks
gestation by the production of estrogen by
the placenta and by the fetus itself.
At birth, the fetus is separated from its
placenta and therefore the major source of
estrogen is removed.
10/04/13 3
4. Hypoestrogenic
state of the fetus
FSH level rises and
remains elevated for
6-10 months
After birth
But FSH is suppressed by Central
inhibition of production of GnRH
Controlled by gene
in the GnRH cell
nucleus in the
hypothalamus
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5. FSH pulses are
undetectable -8-9 yrs
1-2 yrs – spike of
FSH increases in
frequency
Childhood
4-5 yrs – frequency of the FSH
pulses increases in day light hours
Fully functional
production of GnRH
with N adult frequency,
amplitude and pulse s
5-10yrs ovulatory
menstrual cycle
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6. Pituitary glands
All activities increases
At puberty – increase secretion of releasing
factors by the hypothalamus
Manifested by sudden spurt in height,
enlargement of thyroid, adrenal cortex
activity, skin pigmentation
Cyclical production of gonadotrophin
and estrogen in amount10/04/13 6
9. STAGES OF PUBERTYSTAGES OF PUBERTY
Growth spurt
Breast development (thelarche)
Pubic hair growth (Adrenarche)
Menstruation (menarche)
Axillary hair growth
70% of girls, variation often occur in
Tanner
Definite signs of puberty are usually
present by the age 9 or 10 years
10/04/13 9
10. Growth spurtGrowth spurt
It begins around the age of 11yrs in girls
6 to 10cms per year for around 2 years
Effect of estrogen – fusion of end plate of
the femur and growth ceases by the age
of 15 yrs
10/04/13 10
11. Tanner staging of breastTanner staging of breast
development –development – Marshall and Tanner (1969)Marshall and Tanner (1969)
Elevation of papilla
Elevation of papilla & breast
on a small mount, increased
in areola
Further enlargement
Secondary mound of areola
and papilla
Recession of areola to
contour of breast
prepubertal
9-13 yrs
10-14 yrs
11-15 yrs
12-17 yrs
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13. MenarcheMenarche
occurs at any between 9 to 17 yrs
In India -13.5 yrs
Age of menarche varies
family
Race
Social class
Family size,
birth order
Environment
Diet
General health
10/04/13 13
15. Axillary hairAxillary hair
Appears later
During the 2yrs before the menarche the
genital tract develops
Menstrual phase itself often preceded by
mucoid vaginal discharge
10/04/13 15
16. Other Changes during pubertyOther Changes during puberty
Apart from development of secondary
sexual characters and growth spurt
other changes are
Gonads
Sex organs
Pelvis
Skin changes
Psychological changes
Hormonal
10/04/13 16
19. Precocious pubertyPrecocious puberty
Tanner stage 2 of
breast development
prior the age of 8
yrs in white and 7
yrs in black
Elevation of papilla &
breast on a small
mount, increased in
areola
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23. CompleteComplete
Central isosexual pubertyCentral isosexual puberty
Systemic estrogen effect
True or gonadotrophin dependent
90%
Cyclic release of gonadotrophin
Classification: Idiopathic or organic brain
disease
Idiopathic :
Most common
70%
Underlying etiology unknown
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24. Growth spurt is rapid with short duration
Rate of progression vary
General health is not impaired
USG- functional follicular ovarian cyst
Incidence of POF and infertility is not
increased
Other causes are to be excluded before
diagnosis – MRI, CT scan, etc
30% cases may have organic brain disease
10/04/13 24
27. Ovarian tumourOvarian tumour
It is common cause for PPP
Granulosa theca cell tumour – benign,
estrogen secreting, confined to one
ovary,
Palpable rectal abdominal examination or
USG
Treatment : unilateral
salpingoopherectomy
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28. Mc Cune Albright syndromeMc Cune Albright syndrome
Rare – girls
Triad
1. Precocious puberty
2. multiple area of fibrous dysplasia of bone
3. café au lait spots of the skin
facial asymmetry or skeletal deformities
X-ray shows dysplastic lesions
Fluctuation of estrogen levels and low
gonadotrophin- independent of GnRH
stimulation
10/04/13 28
29. Café au lait skin pigmentationCafé au lait skin pigmentation
10/04/13 29
31. X ray showing dysplastic lesionX ray showing dysplastic lesion
Single view of the left hand demonstrates
multiple large expansile "bubbly" lytic
lesions with sharp transition zones and
without an associated periosteal reaction (
arrows). The lesions are located in the
phalanges, carpels, metacarpals, distal ulna
and radial bones. The cortex is very thin in
many areas overlying the expansile lytic
lesion, making it difficult to determine if a
fracture has occurred
10/04/13 31
35. Incomplete precocious pubertyIncomplete precocious puberty
No systemic estrogen effect
One pubertal change is clinically apparent
Absence of superficial cell desquamated
from vaginal mucosa or bone age
10/04/13 35
36. Premature thelarche – development ofPremature thelarche – development of
breast < 8yrs in white and <7yrs in blackbreast < 8yrs in white and <7yrs in black
10/04/13 36
This is a bilateral enlargement of breasts in 1-2 yr olds that is
common. There are no other signs of puberty development
and the growth is normal. As long as the vulva, labia, vagina
are normal infantile, and there is no pubic hair, then nothing is
done.
37. 10/04/13 37
Benign and needs no therapy
Commonly occurs between 1and 4 yrs of
age.
No progression
1/3th regression
1/10 progression
Estradiol level < 20 ng/ml
GnRH stimulation: FSH increases and LH
no response
38. Appearance of pubic hair <8
yrs
No other pubertal changes
No evidence of systemic
estrogen
Other androgen mediated
clinical findings- axillary hair
growth, oily skin, and acne
One half children have
organic brain disease.
10/04/13 38
Premature AdrenarchePremature Adrenarche
41. DiagnosisDiagnosis
To distinguished heterosexual and
isosexual puberty- History
Physical examination –identify
Tanner staging
Height
Incomplete precocious puberty-serial
observation for at least 6 months
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42. Diagnosis contdDiagnosis contd
Thyroid dysfunction can be evaluated by
thyroid profile.
Serum HCG concentrations are elevated
in the presence of trophoblastic disease.
Iatrogenic sources of estrogen – medical
history
Mc Cune Albright – clinical features
10/04/13 42
46. Incomplete precocious puberty -Incomplete precocious puberty -
Premature adrenarchePremature adrenarche
Cranial CT scan,
17 alpha hydroxyprogesterone level at
baseline and following intravenous ACTH
stimulation
10/04/13 46
47. To distinguish Peripheral PP fromTo distinguish Peripheral PP from
central precocious pubercentral precocious pubertyty
GnRH stimulation test - In PPP no
change in gonadotrophin levels whereas
True PP FSH increases more than LH
advanced bone age in both
Increase in ovarian volume and uterine
size in TPP
10/04/13 47
48. A rectal abdominal examination and pelvic
USG – identify ovarian tumours and
ovarian cysts.
Adrenal tumours –adrenal sonograms
CNS diseases is confirmed with the use of
neurologic and ophthalmologic
examination, skull x – ray, EEG and CT
cranial scan or MRI study of the brain.
10/04/13 48
49. TreatmentTreatment
Incomplete forms – self limiting
Hypothyroid –thyroid replacement
therapy
Iatrogenic
Ovarian and adrenal tumours – removed
10/04/13 49
50. Mc Cune Albright syndrome-
Testolactone – total daily oral dose of 20
mg/kg body in four divided doses-
over a 3 weeks interval the total daily
dose is increased to 40 mg/kg body wt
Continue till the sign regress
Side effects: diarrhoea,
abdominalcramping
10/04/13 50
51. Idiopathic – GnRH analogs are reported
as being sucessful in the treatment of IPP
and central nervous system .
Therapy – early – increase the height
Long acting GnRH agonist
Deslorelin 4-8 ug/kg
Leuprolide acetate 20-60ug/kg
Buserelin 20-30 ug/kg
Leuprolide acetate IM 60ug/kg every 4
weeks
Buserelin 1200-1800 ug/kg intranasally
10/04/13 51
Once
daily SC
injection
52. GnRH agonist are not useful in PPP
Side effects:
allergic reactions
allergy symptoms of lungs with intranasal
GnRH should be continued TPP till the
mean age of pubertal development.
10/04/13 52
53. Precocious puberty can be differentiated from
premature adrenarche by the concomitant
appearance of pubic hair with breast
development in girls and with testicular
enlargement in boys.
Other differential diagnoses include virilization
caused by congenital adrenal hyperplasia and an
adrenocortical or gonadal tumor. In premature
adrenarche, serum concentrations of
dehydroepiandrosterone,
dehydroepiandrosterone sulfate (DHEAS),
androstenedione, and testosterone and urinary
17-ketosteroids are usually increased for
chronological age and in the range of those found
in early puberty.
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54. The bone age is usually within 2 standard
deviations of the chronological age.
Moderately elevated levels of serum
androgen other than DHEAS, bone age
advancement, or signs of atypical
premature pubarche (such as cystic acne or
symptoms of systemic virilization) indicate
the need for a corticotropin test to rule
out late-onset congenital adrenal
hyperplasia.
Marked elevation of serum androgen levels
and advanced bone age suggest the
possibility of an adrenocortical or gonadal
tumor.
10/04/13 54
55. Delayed pubertyDelayed puberty
breast tissue and/or pubic hair have not
appeared by 13-14 yrs of age
Or menarche appears as late as 16 yrs
The normal upper age limit of menarche
is 15 yrs.
15% cases – constitutional delay –
PCOD, cryptamenorrhoea.
10/04/13 55
59. Weight:Weight:
Underweight :
1. malnutrition
2. malabsorption syndrome
3. aneroxia nervosa
4. Excessive dieting
5. other psychiatric diseases
Normal weight or obese :
1. constitutional delay,
2. XY gonadal dysgenesis
3. Kallman syndrome
4. pituitary tumours, PCOS,
5. Adrenal abnormalities and other causes of
secondary amenorrhoea.
10/04/13 59
60. InvestigationsInvestigations
Physical examination
karyotyping
FSH level – Increased in ovarian failure
Decreased in hypopituitarism
Thyroid and prolactin
Ultrasound
X ray pituitary
MRI
CT scan
Laparoscopy
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61. TreatmentTreatment
Treatment is directed according to the
etiology
Assurance, improvement of general
health and treatment of any illness may
be of help in non endocrinal causes
cases with hypogonadism may be treated
with cyclic estrogen
Unopposed estrogen 0.3 mg (conjugated
estrogen) daily is given for first 6 months
10/04/13 61
62. combined estrogen and progestin
sequential regimen is started
cases of hypergonadotrophic
hypogonadism should have
chromosomal study to exclude
intersexuality.
10/04/13 62