Dr. Vaman Khadilkar

Report
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
+ve Feedback
GnRh
Pit
- ve Feedback
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
Study
Urban
Rural
Dudhe J Y et al (Central India) 2012
13.5
13.6
Deb R (Meghalaya) 2011
12.1
13.2
Rao S (Maharashtra) 1998
12.1
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 57% 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
TH
160
140
120
100
80
Bone age is 11 y
60
40
Predicted Adult Ht 143
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
1st child of Cut-off
non-consanguinous
age old Sanjay
Mean
marriage
with no signs1.9
of puberty and small
0-6m brought2.9
penis
6-12m
4.1
2.1
3.6
• His 1-5y
height was on5.2
3rd centile, weight
on 75th centile,
5y-puberty
4 75th centile
MPH
50th centile.6BMI was above
• 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 25th centile
– 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 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
Thank
You !
[email protected]

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