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M. Hashemipour

A boy with tall stature and delayed puberty. Professor of Pediatric Endocrinology Isfahan University Of Medical Sciences. M. Hashemipour. Case:. An 18 year old boy was referred because of delayed puberty . Reduced facial and body hair. What do you ask him?. Social and family history.

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M. Hashemipour

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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. 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 andpuberty • He has delayed puberty • He has hypogonadotropinhypogonatism

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

  21. 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 Why would you collect basal levels of LH and FSH? To exclude : • Hypergonadotropichypogonadism • Hypogonadotropichypogonadism • 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 hypogonadotropichypogonadism. GnRH test may be helpful

  28. How would you interpret these results? Investigation GnRH tests are performed:

  29. 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 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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