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Dilemmas in Puberty

Dilemmas in Puberty. Dr. Vaman Khadilkar MD, DNB, MRCP, DCH (London) Paediatric and Adolescent Endocrinologist Ira Clinic, Pune Jehangir hospital and Bharati Vidyapeeth Medical College Pune & Bombay Hospital, Mumbai.

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Dilemmas in Puberty

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  1. Dilemmas in Puberty Dr. VamanKhadilkar MD, DNB, MRCP, DCH (London) Paediatric and Adolescent Endocrinologist Ira Clinic, Pune Jehangir hospital and BharatiVidyapeeth Medical College Pune & Bombay Hospital, Mumbai

  2. Dr. Vaman KhadilkarMD, DNB, MRCP, DCH (London)Pediatric & Adolescent Endocrinologist • President – Indian Society for Pediatric & Adolescent Endocrinology 2013-14 • Consultant Pediatric Endocrinologist, Jehangir Hospital, Pune and Bombay Hospital, Mumbai • Associate Professor, Pediatric Endocrinology, BharatiVidyapeeth Medical College, Pune • DNB & MD teacher • PhD (Doctorate) guide University of Pune • Trained at Great Ormond Street Hospital, London • Referee for Journal of Pediatric Endocrinology and Metabolism, London and Indian Pediatrics Journal • More than 75 Indexed publications in Pediatric Endocrinology and more than 300 Presentations in State, National and International conferences

  3. +ve Feedback - ve Feedback Neuro - Endocrine Changes Of Puberty Cerebral cortex Hypthalamic Gonadostat GnRh Pit "The Switch" LH, FSH Gonads Gonads Sex Steroids

  4. Puberty – Secular Trends

  5. The Average Age of Menarche Data From Scandinavia

  6. What Is the Mean Age of Menarche in India Now? No national Data Available

  7. What Is Precocious Puberty? • Premature sexual maturation before the normal age of onset of puberty • Dilemma - What is normal and should the age cut off be changed from 8 to 7 or 6 in girls?

  8. What Is Normal Timing of Puberty? • Appearance of secondary sexual characters after the age of 8 years in girls and 9 years in boys is considered normal at present • In United States of America especially in black girls it is seen that signs of secondary sexual characters appear before 8 years in 5-7% of the population

  9. What Is Normal Timing of Puberty? • Early thelarche is noted in many parts of the world • The time interval between thelarche is menarche has become longer • Thus the timing of onset is early but tempo may be variable and hence observation of the tempo of puberty is essential • There is no such evidence in boys – timing of attainment of testicular volume of 4 ml almost remains constant

  10. What Is Normal Timing of Puberty? • Studies show that for girls between the age of 6 and 8 who had signs of precocity, incidence of neurological disease is not uncommon • It is therefore important to retain the previous cut-off limits of 8 for girls and 9 for boys at least for the present time

  11. Dilemma - Why Should I Treat Precocity &Do I need to treat every precocious puberty ?

  12. What Are The Reasons To Treat Precocious Puberty? • Final height Reduction - Stunting • Psychosocial problems in coping with the changing body image, social interactions and Menarche

  13. Growth Chart of a Girl With Precocious Puberty TH Bone age is 11 y Predicted Adult Ht 143

  14. Who Needs Treatment? • Precocious physical signs of puberty – RAPIDLY ADVANCING • Significantly advanced BA • Decreased predicted adult height • Pubertal response to GnRh testing The definition of each of these variable is subjective and NOT absolute

  15. Precocious Puberty – Who Don’t Need Treatment • Girls with slow progressive variety do not need treatment • Generally if the bone age advancement is less than 2 years – Does not need treatment • If two height predictions at least 6 months apart do not show progressive reduction in the predicted adult height – No treatment

  16. Equivocal Cases - Treat or Not? • Equivocal Cases • CA between 6–8 yr • BA not as advanced • Predicted height still close to MPH • GnRH testing unclear

  17. Equivocal Cases – Treat or Not? • Adequate follow-up • Rate of progression of physical changes • Linear growth • Bone maturation • Estimates of adult height • Stimulated gonadotropin levels

  18. Dilemma 2 – Delayed Puberty How long can I wait and watch?

  19. Delayed Puberty Case 1 Stretched Penile Length Norms (CM) age Mean Cut-off 0-6m 2.9 1.9 6-12m 4.1 2.1 1-5y 5.2 3.6 5y-puberty 6 4 • 15 year old Sanjay 1st child of non-consanguinous marriage brought with no signs of puberty and small penis • His height was on 3rd centile, weight on 75th centile, MPH 50th centile. BMI wasabove 75th centile • Tanner: pubic hair stage 2, axillary hair stage 1, genital stage 1, buried penis spl 4 cm, testes 4 ml • Some gynecomastia/ lipomastia • Am I dealing with CDGP or hypogonadism?

  20. Delayed Puberty Case 1 • Points in favor of delayed puberty (CDGP) • Short stature • Some signs of puberty (pubic hair) • Points in favor of hypogonadism • Relatively small size of penis • Small testicular size for age • Gynecomastia • How should I proceed? • Bone age • HCG stimulation test • GnRha stimulation test

  21. Delayed Puberty – Case 1 • Bone age • 12.2 years (delayed) • HCG stimulation test • Good testosterone rise – In favor of delayed puberty • GnRha stimulation test • Lh rises to above 5 iu/ml • Diagnosis – Constitutional Delay in Growth and Puberty

  22. Stretched Penile Length

  23. Prader Orchidometer

  24. Delayed Puberty Case 2 • 16 year old girl living in Pune city from middle class family • Mother worried about no breast development or any other signs of puberty • Anthropometry: • Height 95th centile Target height 25thcentile • Weight 50th centile • Tanner: A1p1b4b4 • Dilemma – should I wait or investigate?

  25. Delayed Puberty – Case 2 • Points in favor of just delayed puberty • Breast development ++ • Points against • Too tall • Well nourished so why late puberty? • Discordance between breast development and hair growth • What should I do?

  26. Delayed Puberty – Case 2 • Bone age • 14 years • Pelvic ultrasound • No uterus, bilateral solid gonads, like testes • Lh, Fsh, Estradiol, Testosterone • Lh, fsh very high, testo – male range, e2= 20 pg/ml • Karyotype XY normal male

  27. Case 3 • 13 year old boy complains of bilateral breast enlargement of 6 months duration • On examination • Bilateral breast development tender 6 cms • Testes 8 ml, axillary and pubic hair stage 2 • Height 85th centile, weight > 90th centile (MPH 50th centile) • BMI above 85th centile • Dilemma - Should I wait or investigate?

  28. Case 3 • Decided to wait for 3 months – reassured • 3 months later breasts bigger, no progress in puberty. • Investigated • LH 35 miu/ml, fsh 20 miu/ml (both high), • Prolactin, TFTs normal • Testosterone 30ng/ml normal • Karyotype – 46 XXY

  29. Case 4 • 15 year old girl complaints • Facial, chin, upper lip hair growth 6 months • Irregular menses • On examination • Hirsutism - FG score 16 • Clitorial hypertrophy • Dilemma – Is this PCOS or is this CAH?

  30. Case 4 • Investigations • 17 ohp3 ng/dl (not very high) • Testosterone 120 ng/ml (high for female) • LH 15, FSH 5 ( reversed ratio) • PCOS on usg • Synacthen test – more than 5 folds rise in 17ohp and 2 times in cortisol • Diagnosis - Non classical CAH

  31. Conclusions • Secular trend is towards early sexual maturation all over the world and is particularly marked in areas of the world that are in rapid economic transition such as India • In equivocal cases longer follow-up to understand the tempo is essential

  32. Conclusions Contd…. • Although there is a trend towards younger age of maturity the traditional age cut-offs of 8 years for girls and 9 years for boys for the beginning of puberty still STAY • Main reasons to treat precocity in children are prevention of short stature and psychological disturbances

  33. Conclusions Contd…. • Anthopometry often gives a clue about whom to investigate, treat and whom not to • Heterosexual precocity must always be investigated • With delayed puberty – Discordance in clinical signs and anthropometry points towards a non physiological cause

  34. Thank You ! vamankhadilkar@gmail.com

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