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PUBERTYPUBERTY
10/04/13 1
Arshiya Sultana
Lecturer, Dept. of Obstetrics &
Gynaecology
NIUM, Bangalore, Karnataka.
Puber- marriageable age
Pubertus – adulthood
 The period of transition between sexual
immaturity and maturity
Puberty is first phase of adolescence.
How puberty occurs ?
10/04/13 2
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
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
10/04/13 4
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
10/04/13 5
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
10/04/13 7
10/04/13 8
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
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
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
10/04/13 11
10/04/13 12
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
10/04/13 14
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
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
FactorsFactors
Geographical
Genetic
Body weight
Health
Socioeconomic
Family background
10/04/13 17
PubertyPuberty
Precocious
puberty
Delayed
puberty
10/04/13 18
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
10/04/13 19
10/04/13 20
PrecociousPrecocious PubertyPuberty
Isosexual Heterosexual
Complete Incomplete
Central
Peripheral
Combined
Premature
thelarche
Premature
adrenarche
10/04/13 21
PrecociousPrecocious PubertyPuberty
Isosexual
Complete
Central
10/04/13 22
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
10/04/13 23
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
PrecociousPrecocious PubertyPuberty
Isosexual
Complete
Central Peripheral
10/04/13 25
Peripheral precocious pubertyPeripheral precocious puberty
 Pseudoprecocious puberty
 Gonadotrophin independent
Classification:
Ovarian tumour
Adrenal tumour – estrogen secreting - rare
Iatrogenic- exogenous administration of sex
steroids
Primary hypothyroidism
MC Cune Albright syndrome
Ovarian cyst- estrogen secreting can cause
PPP
10/04/13 26
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
10/04/13 27
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
Café au lait skin pigmentationCafé au lait skin pigmentation
10/04/13 29
Facial asymmetryFacial asymmetry
10/04/13 30
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
PrecociousPrecocious PubertyPuberty
Isosexual
Complete
Central Peripheral
combined
10/04/13 32
CombinedCombined
CAH
Virilizing adrenal tumours
10/04/13 33
PrecociousPrecocious PubertyPuberty
Isosexual
Complete Incomplete
Central
Peripheral
Combined
Premature
thelarche
Premature
adrenarche
10/04/13 34
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
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. 
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
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
10/04/13 39
Premature AdrenarchePremature Adrenarche
 Adrenal androgen increases
 increase 17 hydroxyprogesterone –
ACTH stimulation
Shows 21 hydroxylase deficiency
10/04/13 40
DiagnosisDiagnosis
To distinguished heterosexual and
isosexual puberty- History
Physical examination –identify
Tanner staging
Height
Incomplete precocious puberty-serial
observation for at least 6 months
10/04/13 41
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
10/04/13 43
Diagnosis contdDiagnosis contd
To distinguish incomplete (PrematureTo distinguish incomplete (Premature
thelarche) from complete precociousthelarche) from complete precocious
pubertypuberty
Serum estradiol
Prolactin
LH
GnRH stimulation test
10/04/13 44
10/04/13 45
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
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
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
TreatmentTreatment
Incomplete forms – self limiting
Hypothyroid –thyroid replacement
therapy
Iatrogenic
Ovarian and adrenal tumours – removed
10/04/13 49
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
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
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
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.
10/04/13 53
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
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
CausesCauses
10/04/13 56
Hypergonadotrophic
hypogonadism
Hypogonadotrophic
hypogonadism
Anatomic
causes
• Gonadal dysgenesis
• Pure gonadal
dysgenesis (46xx,
46xy)
• Ovarian failure
• constitutional delay
• chronic illness
• Malnutrition
• primary
hypothyoidism
• isolated
gonadotrophin
deficiency
• Intracranial lesions
• Pure gonadal
dysgenesis (46xx,
46xy)
• Ovarian failure
• mullerian
• imperforate
hymen
• transerve
vaginal septum
DiagnosisDiagnosis
Thorough history
Previous illness
Physical examination:
Height and weight
 secondary sexual characters
 growth pattern
10/04/13 57
heightheight
Short stature (<147 cm) –
chronic illness
 turner syndrome
Hypothalamic or pituitary lesions
 hypothyroidism
 laurence moon biedl syndrome
10/04/13 58
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
InvestigationsInvestigations
 Physical examination
 karyotyping
 FSH level – Increased in ovarian failure
Decreased in hypopituitarism
Thyroid and prolactin
Ultrasound
X ray pituitary
MRI
CT scan
Laparoscopy
10/04/13 60
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
 combined estrogen and progestin
sequential regimen is started
 cases of hypergonadotrophic
hypogonadism should have
chromosomal study to exclude
intersexuality.
10/04/13 62
10/04/13 63

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Puberty dr-arshiyasultana-110312042731-phpapp02

  • 1. PUBERTYPUBERTY 10/04/13 1 Arshiya Sultana Lecturer, Dept. of Obstetrics & Gynaecology NIUM, Bangalore, Karnataka.
  • 2. Puber- marriageable age Pubertus – adulthood  The period of transition between sexual immaturity and maturity Puberty is first phase of adolescence. How puberty occurs ? 10/04/13 2
  • 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 10/04/13 4
  • 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 10/04/13 5
  • 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 10/04/13 11
  • 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 10/04/13 19
  • 21. PrecociousPrecocious PubertyPuberty Isosexual Heterosexual Complete Incomplete Central Peripheral Combined Premature thelarche Premature adrenarche 10/04/13 21
  • 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 10/04/13 23
  • 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
  • 26. Peripheral precocious pubertyPeripheral precocious puberty  Pseudoprecocious puberty  Gonadotrophin independent Classification: Ovarian tumour Adrenal tumour – estrogen secreting - rare Iatrogenic- exogenous administration of sex steroids Primary hypothyroidism MC Cune Albright syndrome Ovarian cyst- estrogen secreting can cause PPP 10/04/13 26
  • 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 10/04/13 27
  • 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
  • 40. Premature AdrenarchePremature Adrenarche  Adrenal androgen increases  increase 17 hydroxyprogesterone – ACTH stimulation Shows 21 hydroxylase deficiency 10/04/13 40
  • 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 10/04/13 41
  • 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
  • 44. To distinguish incomplete (PrematureTo distinguish incomplete (Premature thelarche) from complete precociousthelarche) from complete precocious pubertypuberty Serum estradiol Prolactin LH GnRH stimulation test 10/04/13 44
  • 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. 10/04/13 53
  • 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
  • 56. CausesCauses 10/04/13 56 Hypergonadotrophic hypogonadism Hypogonadotrophic hypogonadism Anatomic causes • Gonadal dysgenesis • Pure gonadal dysgenesis (46xx, 46xy) • Ovarian failure • constitutional delay • chronic illness • Malnutrition • primary hypothyoidism • isolated gonadotrophin deficiency • Intracranial lesions • Pure gonadal dysgenesis (46xx, 46xy) • Ovarian failure • mullerian • imperforate hymen • transerve vaginal septum
  • 57. DiagnosisDiagnosis Thorough history Previous illness Physical examination: Height and weight  secondary sexual characters  growth pattern 10/04/13 57
  • 58. heightheight Short stature (<147 cm) – chronic illness  turner syndrome Hypothalamic or pituitary lesions  hypothyroidism  laurence moon biedl syndrome 10/04/13 58
  • 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 10/04/13 60
  • 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