1. Puberty Dr Haider Al Shamma’a

2. Objectives Know the definition of puberty The student should be able to understand and describe the physiology of the pre-puberty and puberty The student able to detect the normal sequence of puberty The student should be able to detect the somatic changes associated with puberty The student can diagnose abnormal puberty ( history, examination )

3. Objectives continue…. Able to request the suitable investigations to diagnose the cause and type of abnormal puberty Can describe the outline of treatment for precocious puberty

4. Puberty :- is the sequence of events in the transformation of a child into young adult with the development of secondary sexual characteristics and reproductive capability

5. During this process there are Physical Endocrine Psychological changes a Increased sex steroid hormone

6. Age of onset 10 – 16 years Onset depend on:- 1.Genetic factors 2.Geographic location 3.Nutritional status 4.Psychological factors

7. Endocrinological changes of puberty The physiology of pre-pubertal period 1.Fetal period 2.Neonatal period 3.Childhood period 4.Late pre-pubertal period 5.Pubertal onset

8. Fetal period By 20 wks. FSH & LH start to increase Some ovarian follicular developments E2 → -ve feedback →↓FSH & ↓LH Most estrogens from placental origin

9. New born period ↓↓ estrogen and progesterone (from placental origin) → FSH ↑ & LH ↑ At 3 months FSH & LH near adult level but estrogen remain low FSH & LH gradually reduced reaching lowest level at 4 years of age

10. Childhood period (4-10 years)  Low estrogen  Low FSH & LH Suppression of FSH & LH due to 2 mechanisms 1.Intrinsic central inhibition of hypothalamic GnRH release ( main mechanism) 2.Very sensitive –ve feedback inhibition

11. Late prepubertal period ( 6-9 years) Gradual ↑ androgen from zona reticularis of the adrenals ( ↑ DHEA, ↑DHEAS & androstendione ) → pubic and axillary hair growth ( pubarche & adrenarche )

12. Pubertal onset (11 years) ↓sensitivity of the –ve feedback ( ie higher estrogen needed to suppress the pituitary secretion of FSH & LH ) Loss of the intrinsic CNS inhibition of GnRH from the hypothalamus At first nocturnal GnRH secretion then Adult type pulsetile pattern ( every 90-120 min )

13. Pubertal onset (11 years) ↑ovarian follicular developments ↑ steroid production ( E2) Development of secondary sexual characteristics By mid – late puberty maturation of the +ve feedback mechanism of E2 on LH release and ovulatory cycle established at 14 -16 years

14. Somatic changes 1.Breast buding ( thelarche) 2.Axillary & pubic hair ( adrenarche,pubarche) 3.Accelerated linear growth (growth spurt) 4.First menstrual cycle (menarche) 5.Adult shape breast and pubic hairs * Somatic changes takes about 4.5 years average, ( range 1.5 - 6 years )

17. Precocious puberty

18.  Precocious puberty :- the development of any sign of secondary sexual maturation at an age earlier than 2.5 standard deviations below the expected age of puberty 8 years  Incidence 1/ 10000  Male / female ratio 1/5  75% of all cases are constitutional idiopathic  Typically taller than their peers as children but short as adult due to premature closure of epiphyses

19. Classification of female precocious puberty 1.Heterosexual precocious puberty (male type puberty) a. virilizing neoplasm (tumors secreting androgens ) ovarian adrenal b. congenital adrenal hyperplasia c. exogenous androgen exposure

20.  2. Isosexual precocious puberty:- (female type maturity) A. complete I. true precocious puberty i) idiopathic (constitutional) 90% ii) brain disease 10% II. Pseudo precocious puberty i) ovarian tumor secret estrogen ii) adrenal tumor iii) hypothyroidism in girls* (delayed bone age) iv) exogenous v) McCune –Albright syndrome vi) Peutz-Jegher syndrome (associated with ov.tumor) B. incomplete I. Premature thelarche (breast bud only) II. Premature pubarche ( pubic hair only ) 50% CNS disease III. Premature adrenarche ( axillary hair only )

21. True precocious puberty

22. McCune-Albright syndrome

23. Polyostotic fibrous dysplasia McCune-Albright syndrome

24. True precocious puberty There is activation of the hypothalamus pituitary ovarian axis (like normal adults) Normal sequence of events ( 1 st breast bud,2 nd axillary & pubic hair, 3 rd accelerated growth, menarche )

25. Diagnosis of precocious puberty History:- Similar family history Sequence of sexual maturation Speed of sexual maturation Exposure to drug or hormones CNS (meningitis, encephalitis, abscess, TB, tumor, neurofibromatosis trauma, seizures, headache, blurring of vision, numbness, muscle weakness,… etc.)

26. Physical examination Vital signs Record of serial height and weight Detailed record of secondary sex.charec. Tanner staging Abd. Pelvic exam * (no PV) Thorough neurological exam. Skin exam (café au lait macules, neurofibroma, acne.) Galctorrhea

27. Investigations  Skull X ray  Serial bone age estimations X ray hands&elbow  CT, MRI head sella turcica & hypothalamous  U/S, MRI abdomen & pelvis  FSH, LH, hCG  DEHAS, androsteindione, testosterone, E2, P4  Prolactin  Thyroid functon tests  GnRH stimulation test  EEG  Visual field

28. Treatment of precocious puberty 75% constitutional true precocious puberty The most important problem of untreated girls is short stature (< 150 cm )* due to early closure of epiphysis of long bones

29. Treatment GnRH analogues (triptorrilin, decapeptyl ) It is similar to GnRH but it is of long action attach to pituitary GnRH receptors permanently and cause stimulation of FSH & LH at first week and then cause down regulation of pituitary receptors ie. Decrease the number of functioning receptors → suppression of the pituitary gland → suppression of ovarian activities.

30. Triptorrilin injection every 28 days Till reach the determined height by 13-14 years then stop GnRH analogues and the hypothalamo - piuitary – ovarian axis resume normal activities

31. Treatment of other types of precocious puberty GnRH analogues are ineffective in cases of pseudo-precocious puberty CNS diseases have unfavorable outcomes

32. Treatment of other types of precocious puberty Ovarian, adrenal, brain tumors treated surgically Exogenous hormones by remove source McCune-Albright syndrome by antiestrogen (aromatase inhibitor Lotrizole + orthopedic care Hypothyroidism by thyroxine

33. Precocious thelarche no treatment usually transient Premature pubarche more common in boys 50% CNS disease ………exclude CNS disease …..no treatment Premature adrenarche……exclude adrenal disease …………..no treatment

34. THANK YOU