PUBERTY

Arshiya Sultana Lecturer, Dept. of Obstetrics & Gynaecology NIUM, Bangalore, 5/25/12 1 Karnataka. 1

How puberty occurs ?

Puber- marriageable age adulthood

Pubertus

The period of transition between sexual immaturity and maturity

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Fetal and Infancy

During

the latter half of fetal life, the hypothalamus pituitary ovarian axis is functional completely. levels are suppressed from 20 weeks gestation by the production of estrogen by the placenta and by the fetus itself. birth, the fetus is separated from its placenta and therefore the major source of estrogen is3 5/25/12
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FSH

At

After birth Hypoestrogenic state of the fetus FSH level rises and remains elevated for 610 months But FSH is suppressed by Central inhibition of 5/25/12 production of GnRH

Controlled by gene in the GnRH cell nucleus in the hypothalamus

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Childhood 5-10yrs ovulatory menstrual cycle Fully functional production of GnRH with N adult frequency, amplitude and pulse s

FSH pulses are undetectable -8-9 yrs 1-2 yrs spike of FSH increases in frequency

4-5 yrs frequency of the FSH pulses increases in day 5/25/12 light hours

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At puberty increase secretion of releasing factors by the hypothalamus Pituitary glands All activities increases Manifested by sudden spurt in height, enlargement of thyroid, adrenal cortex activity, skin pigmentation Cyclical production of 5/25/12 gonadotrophin and estrogen in
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STAGES OF PUBERTY
Growth Breast Pubic

spurt

development (thelarche) (menarche)

hair growth (Adrenarche) hair growth

Menstruation Axillary 70%

of girls, variation often occur in Tanner signs of puberty are 5/25/12 usually present by the age 9 or
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Definite

Growth spurt

It begins around the age of 11yrs in girls to 10cms per year for around 2 years of estrogen fusion of end plate of the femur and growth ceases by the age of 15 yrs
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Effect

Tanner staging of breast development Marshall and

Tanner (1969)
prepubert al 9-13 yrs 10-14 yrs 11-15 yrs Elevation of papilla

Elevation of papilla & breast on a small mount, increased in Further areola enlargement Secondary mound of areola and papilla Recession of areola to contour 11 11

12-17 yrs
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Menarche

occurs at any between 9 to 17 yrs India -13.5 yrs of menarche varies

In

Age

family class size,

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Race Social Family birth

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Axillary hair
Appears During

later

the 2yrs before the menarche the genital tract develops phase itself often preceded by mucoid vaginal discharge

Menstrual

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Other Changes during puberty

Apart

from development of secondary sexual characters and growth spurt other changes are organs changes changes
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Gonads Sex

Pelvis Skin

Psychological
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Factors
Geographical Genetic Body

weight

Health Socioeconomic Family

background

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Puberty

Precocious puberty

Delayed puberty

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Precocious 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, 5/25/12 increased in

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Precocious Puberty
Isosexual Heterosexu al Incomplete Premature thelarche Premature adrenarche

Complete

Central

Combined Peripheral

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Precocious Puberty
Isosexual

Complete

Central
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Complete Central isosexual puberty

Systemic True 90% Cyclic

estrogen effect

or gonadotrophin dependent release of gonadotrophin

Classification:

Idiopathic or organic brain disease common

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Idiopathic :
Most
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Growth Rate

spurt is rapid with short duration of progression vary health is not impaired

General

USG- functional follicular ovarian cyst of POF and infertility is not increased causes are to be excluded before diagnosis MRI, CT scan, 5/25/12 etc

Incidence Other

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Precocious Puberty
Isosexual

Complete

Central
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Peripheral
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Peripheral precocious puberty

Pseudoprecocious puberty Gonadotrophin independent tumour

Classification:
Ovarian Adrenal

tumour estrogen secreting - rare exogenous administration of sex steroids

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IatrogenicPrimary

Ovarian tumour
It

is common cause for PPP

Granulosa

theca cell tumour benign, estrogen secreting, confined to one ovary, rectal abdominal examination or USG : unilateral salpingoopherectomy
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Palpable

Treatment

Mc Cune Albright syndrome

Rare

girls

Triad
1. 2. 3. .

Precocious puberty multiple area of fibrous dysplasia of bone caf au lait spots of the skin facial asymmetry or skeletal deformities
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. X-ray

Caf au lait skin pigmentation

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Facial asymmetry

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X 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

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Precocious Puberty
Isosexual

Complete combined Central

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Peripheral
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Combined
CAH Virilizing

adrenal tumours

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Precocious Puberty
Isosexual Incomplete Premature thelarche Premature adrenarche

Complete

Central

Combined Peripheral

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Incomplete precocious puberty

No

systemic estrogen effect

One

pubertal change is clinically apparent of superficial cell desquamated from vaginal mucosa or bone age

Absence

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Premature thelarche development of breast < 8yrs in white and <7yrs in black

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 5/25/12 is done.

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Benign

and needs no therapy

Commonly No

occurs between 1and 4 yrs of age. progression regression level < 20 ng/ml progression

1/3th 1/10

Estradiol GnRH

stimulation: FSH increases 5/25/12 37 and LH no response 37

Premature Adrenarche
Appearance

of

pubic hair <8 yrs

No No

other pubertal changes evidence of systemic estrogen androgen mediated clinical findingsaxillary 5/25/12 hair growth, oily

Other

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Premature Adrenarche

Adrenal androgen increases

increase 17 hydroxyprogesterone ACTH stimulation 21 hydroxylase deficiency

Shows

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Diagnosis
To

distinguished heterosexual and isosexual puberty- History examination identify staging

Physical Tanner Height

Incomplete

precocious pubertyserial observation for at least 6 months

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Diagnosis contd
Thyroid Serum

dysfunction can be evaluated by thyroid profile. HCG concentrations are elevated in the presence of trophoblastic disease. sources of estrogen medical history Cune Albright clinical features
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Iatrogenic Mc

Diagnosis contd

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To distinguish incomplete (Premature thelarche) from complete precocious puberty

Serum

estradiol

Prolactin LH GnRH

stimulation test

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Incomplete precocious puberty Premature adrenarche

Cranial 17

CT scan,

alpha hydroxyprogesterone level at baseline and following intravenous ACTH stimulation

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To distinguish Peripheral PP from central precocious puberty

GnRH stimulation test - In PPP no change in gonadotrophin levels whereas True PP FSH increases more than LH advanced bone age in both in ovarian volume and uterine size in TPP
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Increase

rectal abdominal examination and pelvic USG identify ovarian tumours and ovarian cysts. tumours adrenal sonograms

Adrenal 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.
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Treatment
Incomplete

forms self limiting

Hypothyroid Iatrogenic Ovarian

thyroid replacement therapy and adrenal tumours removed

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Mc Cune Albright syndromeTestolactone

total daily oral dose of 20 mg/kg body in four divided dosesa 3 weeks interval the total daily dose is increased to 40 mg/kg body wt till the sign regress
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over

Continue Side

effects: diarrhoea, 5/25/12 abdominalcramping

Idiopathic

GnRH analogs are reported as being sucessful in the treatment of IPP and central nervous Once system . daily SC early the height
injectio increase n

Therapy

Long acting GnRH agonist

Deslorelin Leuprolide

4-8 ug/kg acetate 2051 51

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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. 5/25/12

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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. differential diagnoses include virilization caused by congenital adrenal hyperplasia and an adrenocortical or gonadal tumor. In 5/25/12 premature adrenarche,

Other

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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.
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Marked

elevation

of

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Delayed puberty

breast tissue and/or pubic hair have not appeared by 13-14 yrs of age menarche appears as late as 16 yrs normal upper age limit of menarche is 15 yrs. cases constitutional delay PCOD, cryptamenorrhoea.
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Or

The

15%

Causes
Hypergonadot rophic hypogonadism Gonadal dysgenesis Pure gonadal dysgenesis (46xx, 46xy) Ovarian failure

Hypogonad otrophic hypogonadi sm constitutional delay chronic illness Malnutrition primary

Anatomi c causes

mullerian

imperforat e hymen transerve vaginal 56 septum 56

hypothyoidism 5/25/12 isolated

Diagnosis
Thorough Previous Physical Height

history

illness examination:

and weight

secondary sexual characters growth pattern

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height
Short stature (<147 cm)
chronic

illness or pituitary lesions

turner syndrome hypothyroidism laurence moon biedl syndrome

Hypothalamic

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Weight:
Underweight 1. 2. 3. 4. 5.

malnutrition malabsorption syndrome aneroxia nervosa Excessive dieting other psychiatric diseases weight or obese :
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.Normal 1. 2.

constitutional delay,
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Investigations

Physical examination karyotyping

FSH level Increased in ovarian failure in hypopituitarism and prolactin

Decreased Thyroid

Ultrasound X

ray pituitary
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Treatment
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 estrogen 0.3 mg 5/25/12 61 (conjugated estrogen) daily is 61

Unopposed

combined estrogen and progestin sequential regimen is started cases of hypergonadotrophic hypogonadism should have chromosomal study to exclude intersexuality.

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Thank you
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