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
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
+ve Feedback - ve Feedback Neuro - Endocrine Changes Of Puberty Cerebral cortex Hypthalamic Gonadostat GnRh Pit "The Switch" LH, FSH Gonads Gonads Sex Steroids
Puberty – Secular Trends
The Average Age of Menarche Data From Scandinavia
What Is the Mean Age of Menarche in India Now? No national Data Available
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?
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
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
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
Dilemma - Why Should I Treat Precocity &Do I need to treat every precocious puberty ?
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
Growth Chart of a Girl With Precocious Puberty TH Bone age is 11 y Predicted Adult Ht 143
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
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
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
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
Dilemma 2 – Delayed Puberty How long can I wait and watch?
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?
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
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
Stretched Penile Length
Prader Orchidometer
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?
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?
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
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?
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
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?
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
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
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
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
Thank You ! vamankhadilkar@gmail.com
