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A boy with tall stature and delayed puberty

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1 A boy with tall stature and delayed puberty
Professor of Pediatric Endocrinology Isfahan University Of Medical Sciences M. Hashemipour

2 Case: An 18 year old boy was referred because of delayed puberty .
Reduced facial and body hair .

3 What do you ask him?

4 Social and family history
Student Non-smoker His older brother had spontaneous puberty at the age of 13 years His mother’s menarche occurred at the age of 15 years He was born after an uncomplicated delivery He has always been healthy At present , he is in his last year of high school

5 Sense of smell Unremarkable past medical history

6 What's important in Physical examination?

7 . His target height (TH) is 178cm
He has not experienced a pubertal growth spurt His height velocity has continued in a prepubertal pattern

8 Arm span - Height =7cm

9 Physical examination physical examination reveals
No pubertal genital development Testicular volume is 2ml on each side Moderately pubichair &axillary hair

10 Physical Examination What physical features
suggest a pubertal disorder ?

11 Eunuchoid Long legs Lack of pubertal development Gynecomastia

12 Investigations What additional growth related information would you need before making a diagnosis?

13 Bone age Serum testosterone level Upper to lower segment

14 Upper to lower segment =0.8
BA=15 Y Tes= 0.6ng/ml Upper to lower segment =0.8

15 Problem Definition How would you diagnose his pubertal complaint?

16 He is normal and will develop normally
He has constitutional delay of growth and puberty He has delayed puberty He has hypogonadotropin hypogonatism

17 Pituitary failure

18 Medical history What further information from his medical history would be relevant in dealing with his complaint?

19 surgery or medical treatment for undescended testes
Member of his family has experienced fertility problems Normal sense of smell Headaches visual disturbances psychosocial situation

20 His mother recalls that at birth His testes were undescended
Medical history His mother recalls that at birth His testes were undescended . After 2years, his testes had not descended spontaneously, and needed orchiopexy

21 bear child Investigation
His family history revealed that one of his maternal aunts was not able to bear child

22 What's your plan?

23 cdpuberty kallman kallmann Pituitary failure

24 Measure Gonadotropin

25 Investigation To exclude : Hypergonadotropic hypogonadism
Why would you collect basal levels of LH and FSH? To exclude : Hypergonadotropic hypogonadism Hypogonadotropic hypogonadism To make a distinction between HH & CDGP

26 levels of LH and FSH are low

27 Advanced Investigations
It is sometimes difficult to make a distinction between delayed puberty and hypogonadotropic hypogonadism. GnRH test may be helpful

28 Investigation GnRH tests are performed:
60min 30min 0min 0.1 Testosterone (nmol.L) 1.6 1.8 <1 LH(U/L 1.2 1.9 1 FSH(U/L) How would you interpret these results?

29 The rise in Gonadotropin in response to GnRH is minimal
Investigation In hypothalamic dysfunction The rise in Gonadotropin in response to GnRH is minimal

30 Differential Diagnoses
He likely has HH BA=14.5 years: indicates that he has a pubertal disorder.

31 What differential diagnoses would you make ?

32 Impaired GnRH secretion (kallmann syndrome )
Constitutional delay of G&P Isolated gonadotropin deficiency Chronic disease

33 Investigations What additional test would be useful in making a diagnosis?

34 An olfactometric (smell) test
An MRI or CT of hypothalamic/ pituitary region family history

35 What is your opinion about induction of his puberty?

36 For psychosocial reasons , induction of puberty is indicated
Further delay of pubertal development would compromise normal development of bone mass.

37 Therapy He asks you what his options are with respect to pubertal induction and fertility

38 Therapy Long –term treatment for fertility
If he has intact LH&FSH producing pituitary cells Long –term treatment for fertility HCG and HMG Biosynthetic LH /FSH preparation pulsatile GnRH Could be used to induce gonadal development and spermatogenesis .

39 Therapy He was treated with testosterone
He was pleased with his pubertal development. He reached a final height of 199 cm at the age of 21 years

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