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 4 4 .

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 5 5 .

enlargement of thyroid.At puberty – increase secretion of releasing factors by the hypothalamus Pituitary glands All activities increases Manifested by sudden spurt in height. adrenal cortex activity. skin pigmentation Cyclical production of 5/25/12 gonadotrophin and estrogen in 6 6 .

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variation often occur in Tanner signs of puberty are 5/25/12 usually present by the age 9 or 9 9 Definite .STAGES OF PUBERTY Growth Breast Pubic spurt development (thelarche) (menarche) hair growth (Adrenarche) hair growth Menstruation Axillary 70% of girls.

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 5/25/12 10 10 6 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 5/25/12 .

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13 13 Race Social Family birth 5/25/12 order .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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Axillary hair Appears During later the 2yrs before the menarche the genital tract develops phase itself often preceded by mucoid vaginal discharge Menstrual 5/25/12 15 15 .

Other Changes during puberty Apart from development of secondary sexual characters and growth spurt other changes are organs changes changes 16 16 Gonads Sex Pelvis Skin Psychological 5/25/12 Hormonal .

Factors Geographical Genetic Body weight Health Socioeconomic Family background 5/25/12 17 17 .

Puberty Precocious puberty Delayed puberty 5/25/12 18 18 .

5/25/12 increased in 19 19 .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.

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Precocious Puberty Isosexual Heterosexu al Incomplete Premature thelarche Premature adrenarche Complete Central Combined Peripheral 5/25/12 21 21 .

Precocious Puberty Isosexual Complete Central 5/25/12 22 22 .

Complete Central isosexual puberty Systemic True 90% Cyclic estrogen effect or gonadotrophin dependent release of gonadotrophin Classification: Idiopathic or organic brain disease common 5/25/12 Idiopathic : Most 23 23 .

CT scan.Growth Rate spurt is rapid with short duration of progression vary health is not impaired General  USG. 5/25/12 etc Incidence Other 24 24 .functional follicular ovarian cyst of POF and infertility is not increased causes are to be excluded before diagnosis – MRI.

Precocious Puberty Isosexual Complete Central 5/25/12 Peripheral 25 25 .

Peripheral precocious puberty   Pseudoprecocious puberty Gonadotrophin independent tumour Classification: Ovarian Adrenal tumour – estrogen secreting .rare exogenous administration of sex steroids 5/25/12 hypothyroidism 26 26 IatrogenicPrimary .

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 5/25/12 27 27 Palpable Treatment .

Mc Cune Albright syndrome Rare – girls Triad 1. Precocious puberty multiple area of fibrous dysplasia of bone café au lait spots of the skin facial asymmetry or skeletal deformities 5/25/12 shows dysplastic lesions 28 28 . . 3. 2. X-ray .

Café au lait skin pigmentation 5/25/12 29 29 .

Facial asymmetry 5/25/12 30 30 .

The cortex is very thin in many areas overlying the expansile lytic lesion.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. making it difficult to determine if a fracture has occurred 5/25/12 31 31 . distal ulna and radial bones. metacarpals. carpels.

Precocious Puberty Isosexual Complete combined Central 5/25/12 Peripheral 32 32 .

Combined CAH Virilizing adrenal tumours 5/25/12 33 33 .

Precocious Puberty Isosexual Incomplete Premature thelarche Premature adrenarche Complete Central Combined Peripheral 5/25/12 34 34 .

Incomplete precocious puberty No systemic estrogen effect One pubertal change is clinically apparent of superficial cell desquamated from vaginal mucosa or bone age Absence 5/25/12 35 35 .

vagina are normal infantile.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. labia.  36 36 .  There are no other signs of puberty development and the growth is normal. and there is no pubic hair.  As long as the vulva. then nothing 5/25/12 is done.

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 .Benign and needs no therapy Commonly No occurs between 1and 4 yrs of age.

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 38 38 .

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Premature Adrenarche   Adrenal androgen increases increase 17 hydroxyprogesterone –ACTH stimulation 21 hydroxylase deficiency Shows 5/25/12 40 40 .

History examination –identify staging Physical Tanner Height Incomplete precocious pubertyserial observation for at least 6 months 5/25/12 41 41 .Diagnosis To distinguished heterosexual and isosexual puberty.

sources of estrogen – medical history Cune Albright – clinical features 5/25/12 42 42 Iatrogenic Mc .Diagnosis contd Thyroid Serum dysfunction can be evaluated by thyroid profile. HCG concentrations are elevated in the presence of trophoblastic disease.

Diagnosis contd 5/25/12 43 43 .

To distinguish incomplete (Premature thelarche) from complete precocious puberty Serum estradiol Prolactin LH GnRH stimulation test 5/25/12 44 44 .

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Incomplete precocious puberty Premature adrenarche Cranial 17 CT scan. alpha hydroxyprogesterone level at baseline and following intravenous ACTH stimulation 5/25/12 46 46 .

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 5/25/12 47 47  Increase .

A

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 syndromeTestolactone

– 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 5/25/12 .

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 52 52 .

Other 53 53 . In 5/25/12 premature adrenarche. differential diagnoses include virilization caused by congenital adrenal hyperplasia and an adrenocortical or gonadal tumor.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.

Moderately elevated levels of serum androgen other than DHEAS. 5/25/12 Marked elevation of 54 serum 54 . 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.The bone age is usually within 2 standard deviations of the chronological age. bone age advancement.

5/25/12 55 55 Or The 15% . cryptamenorrhoea.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.

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 • .Causes Hypergonadot rophic hypogonadism Gonadal dysgenesis • Pure gonadal dysgenesis (46xx.

Diagnosis Thorough Previous Physical Height   history illness examination: and weight secondary sexual characters growth pattern 5/25/12 57 57 .

height Short stature (<147 cm) – chronic  illness or pituitary lesions turner syndrome hypothyroidism laurence moon biedl syndrome Hypothalamic   5/25/12 58 58 .

2. 3. 2.Normal 1. 5.Weight: Underweight 1. 5/25/12 XY gonadal dysgenesis . 4. : malnutrition malabsorption syndrome aneroxia nervosa Excessive dieting other psychiatric diseases weight or obese : 59 59 . constitutional delay.

Investigations    Physical examination karyotyping FSH level – Increased in ovarian failure in hypopituitarism and prolactin Decreased Thyroid Ultrasound X ray pituitary 5/25/12 60 60 .

3 mg 5/25/12 61 (conjugated estrogen) daily is 61  Unopposed . 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.Treatment Treatment  is directed according to the etiology Assurance.

 combined estrogen and progestin sequential regimen is started cases of hypergonadotrophic hypogonadism should have chromosomal study to exclude intersexuality.  5/25/12 62 62 .

Thank you 5/25/12 63 63 .