Puberty dr-arshiyasultana-110312042731-phpapp02

PUBERTYPUBERTY

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Arshiya Sultana Lecturer, Dept. of Obstetrics & GynaecologyNIUM, 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 ?

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Fetal and Infancy Fetal 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.

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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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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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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

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STAGES OF PUBERTYSTAGES OF PUBERTY

Growth spurt Breast development (thelarche) Pubic hair growth (Adrenarche)Menstruation (menarche)Axillary hair growth70% of girls, variation often

occur in TannerDefinite signs of puberty are

usually present by the age 9 or 10 years 04/08/23 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

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Tanner staging of breast Tanner staging of breast development – development – Marshall and Marshall and

Tanner (1969)Tanner (1969)

Elevation of papilla

Elevation of papilla & breast on a small mount, increased in areolaFurther 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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MenarcheMenarche

occurs at any between 9 to 17 yrsIn India -13.5 yrs Age of menarche varies familyRaceSocial classFamily size, birth orderEnvironmentDietGeneral health

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Axillary hairAxillary hairAppears laterDuring the 2yrs before the

menarche the genital tract develops

Menstrual phase itself often preceded by mucoid vaginal discharge

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Other Changes during Other Changes during pubertypubertyApart from development of

secondary sexual characters and growth spurt other changes are

GonadsSex organsPelvisSkin changesPsychological changesHormonal

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Factors Factors Geographical GeneticBody weightHealth Socioeconomic Family background

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PubertyPuberty

Precocious puberty

Delayed puberty

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Precocious pubertyPrecocious pubertyTanner 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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Precocious Precocious PubertyPuberty

Isosexual Heterosexual

Complete Incomplete

Central

Peripheral

Combined

Premature thelarche

Premature adrenarche

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Precocious Precocious PubertyPuberty

Isosexual

Complete

Central

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Complete Complete Central isosexual pubertyCentral isosexual puberty

Systemic estrogen effectTrue or gonadotrophin dependent90% Cyclic release of gonadotrophinClassification: Idiopathic or organic

brain diseaseIdiopathic : Most common70% Underlying etiology unknown

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Growth spurt is rapid with short duration

Rate of progression varyGeneral health is not impaired USG- functional follicular ovarian cystIncidence of POF and infertility is not

increasedOther causes are to be excluded

before diagnosis – MRI, CT scan, etc30% cases may have organic brain

disease

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Precocious Precocious PubertyPuberty

Isosexual

Complete

Central Peripheral

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Peripheral precocious Peripheral precocious pubertypuberty

Pseudoprecocious puberty Gonadotrophin independentClassification:Ovarian tumour Adrenal tumour – estrogen secreting

- rareIatrogenic- exogenous

administration of sex steroidsPrimary hypothyroidismMC Cune Albright syndromeOvarian cyst- estrogen secreting can

cause PPP04/08/23 26

Ovarian tumourOvarian tumourIt is common cause for PPPGranulosa theca cell tumour –

benign, estrogen secreting, confined to one ovary,

Palpable rectal abdominal examination or USG

Treatment : unilateral salpingoopherectomy

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Mc Cune Albright Mc Cune Albright syndromesyndromeRare – girlsTriad1. Precocious puberty2. multiple area of fibrous dysplasia of

bone3. café au lait spots of the skin facial asymmetry or skeletal deformitiesX-ray shows dysplastic lesionsFluctuation of estrogen levels and low

gonadotrophin- independent of GnRH stimulation

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Café au lait skin Café au lait skin pigmentationpigmentation

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Facial asymmetryFacial asymmetry

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X ray showing dysplastic X ray showing dysplastic lesionlesion

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 Precocious PubertyPuberty

Isosexual

Complete

Central Peripheral

combined

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CombinedCombinedCAHVirilizing adrenal tumours

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Precocious Precocious PubertyPuberty

Isosexual

Complete Incomplete

Central

Peripheral

Combined

Premature thelarche

Premature adrenarche

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Incomplete precocious Incomplete precocious pubertypubertyNo systemic estrogen effectOne pubertal change is clinically

apparentAbsence of superficial cell

desquamated from vaginal mucosa or bone age

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

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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. 

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Benign and needs no therapyCommonly occurs between 1and

4 yrs of age.No progression1/3th regression1/10 progressionEstradiol level < 20 ng/mlGnRH 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.

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Premature AdrenarchePremature Adrenarche

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Premature AdrenarchePremature Adrenarche Adrenal androgen increases increase 17

hydroxyprogesterone –ACTH stimulation

Shows 21 hydroxylase deficiency

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DiagnosisDiagnosisTo distinguished heterosexual

and isosexual puberty- HistoryPhysical examination –identify Tanner stagingHeight Incomplete precocious puberty-

serial observation for at least 6 months

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Diagnosis Diagnosis contdcontd

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

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Diagnosis Diagnosis contdcontd

To distinguish incomplete To distinguish incomplete (Premature thelarche) from (Premature thelarche) from

complete precocious pubertycomplete precocious puberty

Serum estradiolProlactinLHGnRH stimulation test

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Incomplete precocious puberty - Incomplete precocious puberty - Premature adrenarchePremature adrenarche

Cranial CT scan, 17 alpha hydroxyprogesterone

level at baseline and following intravenous ACTH stimulation

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To distinguish Peripheral PP To distinguish Peripheral PP from central precocious from central precocious

puberpubertyty

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

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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.

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TreatmentTreatmentIncomplete forms – self limitingHypothyroid –thyroid

replacement therapyIatrogenic Ovarian and adrenal tumours –

removed

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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,

abdominalcramping04/08/23 50

Idiopathic – GnRH analogs are reported as being sucessful in the treatment of IPP and central nervous system .

Therapy – early – increase the heightLong acting GnRH agonistDeslorelin 4-8 ug/kgLeuprolide acetate 20-60ug/kg Buserelin 20-30 ug/kg

Leuprolide acetate IM 60ug/kg every 4 weeks

Buserelin 1200-1800 ug/kg intranasally

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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.

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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.

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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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.

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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.

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Causes Causes

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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

DiagnosisDiagnosisThorough historyPrevious illnessPhysical examination:Height and weight secondary sexual characters growth pattern

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heightheight

Short stature (<147 cm) – chronic illness turner syndromeHypothalamic or pituitary lesions hypothyroidism laurence moon biedl syndrome

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Weight:Weight:Underweight :1. malnutrition2. malabsorption syndrome3. aneroxia nervosa4.Excessive dieting5. other psychiatric diseasesNormal weight or obese :1. constitutional delay, 2.XY gonadal dysgenesis3.Kallman syndrome4. pituitary tumours, PCOS, 5.Adrenal abnormalities and other causes

of secondary amenorrhoea.

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InvestigationsInvestigations Physical examination karyotyping FSH level – Increased in ovarian

failure Decreased in hypopituitarismThyroid and prolactinUltrasoundX ray pituitaryMRICT scanLaparoscopy

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Treatment 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

Unopposed estrogen 0.3 mg (conjugated estrogen) daily is given for first 6 months

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combined estrogen and progestin sequential regimen is started

cases of hypergonadotrophic hypogonadism should have chromosomal study to exclude intersexuality.

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