Pubertal disorders Dr Olcay Evliyaoğlu Pediatric Endocrinology Department Puberty Sexual differentiation begins on 4-5th week of gestational age. ADOLESENCE Physical, sexual, physcological development. Transition to adulthood from childhood Not an adult nor child Time of transition Establishment of individualism. PUBERTY Secondary sex characteristics are developed Includes Physical growth Sexual development Psychological development Growth spurt, disturbution of muscle and fat bone maturation and fusion of epiphysical centers Adult height At the end of this period spermatogenesis in boys, ovulation in girls occur STRESS What makes HHG axis work MATURATİON + GLUTAMAT + + CENTRAL NERVOUS SYSTEM GABA + LEPTIN ENDROFIN - - + HYPOTHALAMUS - - GnRH - - + + PİTUİTARY PRL - LH - - - FSH - + TEKA + OVARY GRANULOZA ANDROGEN ESTROGEN INHIBIN NUTRİTİON Gonadarche Hypothalamus Pituitary GnRH pulse frequency and amplitude increase LH pulse frequency and amplitude increase At early stages at nights With progression of puberty pulses occur during day Gonadarche LH Teca cells FSH androstenedion Granulosa cells Estradiol (E2) Gonadarche LH Leydig cells testosteron Adrenarche Increase in the secretion of adrenal androgens Appearance of pubic hair (pubarche) Axillary hair Adrenarche Hypothalamus Pituitary Adrenal cortex CRH ACTH Adrenal androgens Genetic and environmental conditions effect timing of puberty Environmental factors: Socioeconomic factors Nutrition General health Geographical place lived Nutrition and puberty Menarche is earlier in obese girls Malnutrition , chronic diseases, and excessive physical activity delay puberty and menarche LEPTİN Codes Ob gene Synthesized in adipose tissue. Leptin signals stimulate central nervous system Leptin levels are decreased in excessive physical activity, anorexia nevrosa and amenorea. hypothalamus Leptin LHRH secreting neurons LHRH pituitary gonadotrophs Leptin mRNA Adipose cells LH, FSH Timing of puberty is related to bone age rather than chronological age BA< CY ------------- delay in puberty BA = CA ------------- puberty on expected age BA>CA -------------early puberty (puberty precocous) In girls the first sign of puberty is telarche, generally observed on 10 - 11 ages, menarche is observed 2-3 years after telarche. In girls growth spurt occurs 2 years earlier than boys. Growth spurt in girls is at the time of telarche, In boys growth spurt occur at the time when testes volumes are 10 ml. Telarche Pubarche Menarche Pubertal development in girls breast development (tanner staging) 1. Prepubertal 2. Palpation of breat bud 3. Palpation of breat tissue with out any changes in contures 4. Development of breast tissue 5. Adult type breast Pubic hair 1.No hair 2. Appearance of short hair on labium majora 3. Black and thic hair on mons pubis 4. Adult type hair but not spread to inguinal area 5. Adult type with the shape of inverted triangle Tanner staging Girls pubic hair Stage 1: no pubic hair Stage 2: lightly pigmented localized on labiums Stage 3: coarser, darker , more curved, increased in amount spread to pubis Stage 4: curved and increased still less than an adult. Stage 5: adult women triangle spread to thighs Tanner staging Breast development Stage 1: prepubertal Stage 2: breast budding Stage 3: enlargement of breast tissue and areola Stage 4: Areola and papilla forms secondary mound . Stage 5: Mature Tanner stage 4 2-3years after telarche Menarche Tanner stage 4 2-3years after telarche Menarche FSH secretion Dominant follicule estrogen Follicular phase LH secretion Ovulation corpus luteum Progesterone Endometrium prepared If no implantation uterine bleeding Luteal phase Pituitary hormones Ovarian hormones ovulation follicule corpus luteum corpus albicans ovary İstihdam Seçim dominant istihdam Endometrium Follicular /proliferative phase Luteal /secretuary phase Luteal phase + LH + estrogen estrogen - Ovulation corpus luteum Progesterone endometrium is prepared for implantation If no implantation occurs Corpus luteum regression Progesterone levels decline Endometrium can not hold itself-desquamation Spiral arteriols in the functional and basal layers of endometriumun contracts and approx in 7 gün days bleeding stops Luteal phase 14+2 days In boys initial finding of puberty is enlargement of testes. This is followed by scrotum pigementation and thinning of scrotal skin. Penis enlarges. Pubic hair develops. Growth spurt occurs at 14-16 years and usually continues until 18 years. Pubertal development in boys Tanner staging Prepubertal testes diameter < 2.5 cm= 4 ml Testes volumes 1-3 ml P1 4-9 ml P2 10-14 ml P3 15-20 ml P4 > 20 ml P5 Tanner staging Pubic hair stage 1: no pubic hair Stage 2: little pubic hair Stage 3: pubic hair on pubis Stage 4: adult type hair but no invasion on tighs Stage 5 : adult type hair spread to thighs