Transcript Dr. Vaman Khadilkar
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
Dr. Vaman Khadilkar
MD, 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, Bharati Vidyapeeth 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
Neuro - Endocrine Changes Of Puberty Cerebral cortex Hypthalamic Gonadostat GnRh Pit LH, FSH +ve Feedback - ve Feedback 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 Study Dudhe J Y et al (Central India) 2012 Deb R (Meghalaya) 2011 Rao S (Maharashtra) 1998 Urban Rural 13.5
13.6
12.1
12.1
13.2
15.4
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 200 180 160 140 120 TH 100 80 60 Bone age is 11 y Predicted Adult Ht 143 40 20 0 0 1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 18
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)
• 15 year old Sanjay 1 st child of non-consanguinous 4.1
2.1
• His height was on 3 rd MPH 50 th centile, weight on 75 centile. BMI was above 75 th th centile, 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 95 th centile Target height 25 th centile – Weight 50 th 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 85 th centile, weight > 90 th 50 th centile) centile (MPH – BMI above 85 th 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 ohp 3 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