1. DELAYED PUBERTY & HYPOGONADISM
M. Šnajderová
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to copyright were removed

2. DELAYED PUBERTY

3. Etiology of delayed puberty in boys and in girls

4. SPECTRUM OF DELAYED PUBERTY
Hypogonadism
CDGP
Normal
puberty

5. Causes of delay or lack of pubertal development 1
Hypergonadotropic states
(primary gonadal failure)
chromosomal, genetic disorders, and syndromes
(47, XXY, TS, 45X/46 XY…)
Acquired (autoimmune, chemotherapy, infectious, irradiation, surgical, torsion, traumatic)

6. Causes of delay or lack of pubertal development 2
Hypogonadotropic states
(hypothalamic-pituitary defect or lag)
Gonadotropin deficiency
acquired
congenital, genetic, syndromes
endocrinopathies

7. Causes of delay or lack of pubertal development 3
Dealyed or deferred function
CDG/P
chronic illness
Drug abuse
Excessive energy expenditure, exercise
Exogenous obesity
Endocrinopathies
Malnutrition
Psychiatric illness

8. Examination History Physical examination (growth, Tanner, weight)
Laboratory evaluation (LH, FSH, E2, T, … T4, TSH, DHEAS, BA, MRI …)

9. Constitutional delay of growth and puberty
Prepubertal FSH, LH, T (GnRH test)
Transient GH deficiency
 IGF-I, FSH a LH
Psychologic troubles !!!
Final height?
? Any chronic disease? Nutrition? Physical activity?

10. DELAYED PUBERTY – THERAPY
Induction of puberty
Hormonal substitution therapy
Fertility ???
(hypergonadotropic x hypogonadotropic hypogonadism)

11. GnRH test ??? HYPOTHALAMUS HYPOTHALAMUS PITUITARY FSH,  LH
PITUITARY  LH,  FSH
GONADS  T, E2
GONADS  T, E2
hCG
GONADS  T
Therapy: GnRH, hCG, gonadotrophins, (T, E)
Therapy: hCG,
gonadotrophins, (T, E)

12. Permanent hypogonadism

13. ↓hormonal hypothalamus production putuitary gonads HYPOGONADISM
Inborn errors, genetics, neoplasm (Rx, CH, surgery) trauma, inflammation, autoimmunity, after therapy…
↓hormonal
production
hypothalamus
putuitary
gonads
Functional disturbance
HYPOGONADISM

14. Predicted height Final height M2 Induction of puberty induction
High dose of E2
Induction of puberty and HRT in girl
With hypergonadotropic hypogonadism
Optimal, very good effect
Predicted height
Final height M2
Induction of puberty
induction

15. Sexual development and normal function
PUBERTY DISORDERS
Early diagnostics
Therapy and follow-up
AIM
Sexual development and normal function
of reproductive axis
(if possible)