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Some definitions
Childhood stages (5 stages )
1- newborn (first 28 days )
2- infant (first years )
3- toddler(between 1-3 years )
4- preschool (between 3-5 years )
5- school age (6-12 years )
Adolescence: is a period between childhood and adulthood
Early adolescence (10-14 years )
Middle adolescence (15-17 years )
Late adolescence (18-21 years )
Physiology of puberty L4
– Between early childhood and 8-9 yr of age
(prepubertal stage ), the hypothalamic–pituitary–
gonadal axis is dormant as reflected by undetected
level of LH & sex hormones testosterone in boys and
Estradiol in girls .
– 1-3 yr before onset of puberty, low serum level of LH
demonstrate during sleep in pulsatile fashion.
28
،‫نيسان‬
23 3
• The pulsatile secretion of gonadotropins is
responsible for enlargement and
maturation of the gonads and the
secretion of the sex hormones
28
،‫نيسان‬
23 4
• in mid puberty, the level of LH become evident
even during day time and occur at about 90-
120 minutes intervals
• It is clear that GnRH is the primary if not the
only , hormone responsible for the onset and
progression of puberty
28
،‫نيسان‬
23 5
• The effect of gonadal steroid (testosterone
and estradiol) on bone growth and
osseous maturation are critical , both
aromatase deficiency and estrogen
receptor defect result in delayed
epiphyseal closure and tall stature in
affected boys,
28
،‫نيسان‬
23 6
that is mean estrogen rather than androgen
are responsible for process of bone
maturation and epiphyseal fusion and
cessation of growth
Estrogen also mediate the increased
production of growth hormone which also
responsible with sex hormone for pubertal
growth spurt.
28
،‫نيسان‬
23 7
28
،‫نيسان‬
23 8
Precedes menarche
In female
The onset of puberty vary and more correlated
with osseous than with chronological maturation
age
In female, breast bud (thelarche ) is first sign of
puberty (10-11yrs) followed by pubic hair (6-12
months later ), the interval to menarche usually 2-
2.5 yrs.
peak height velocity usually start early 12 yr of age
in girls & always precedes menarche (12.75yr)
28
،‫نيسان‬
23 9
Stages of female puberty
Thelarche10-
11years
Pubic hair 6-12 mo.
later
Menarche
2-2.5 yrs later
28
،‫نيسان‬
23 10
Growth spurt precedes menarche
In male
• In male, growth of testes (>2.5 cm) and thinning
of scrotum is the first sign of puberty followed by
pigmentation of scrotum and enlargement of
penis. pubic hair then appeared. axillary hair
appear in mid puberty in male unlike female
growth acceleration appears after puberty. in
male growth spurt usually 2 yrs later than in
females .and growth may continue beyond 18
yrs .of age
28
،‫نيسان‬
23 11
Stages of male puberty
1-
Testes>2.5cm
& thinning of
scrotum
2-Pigmentation
of scrotum
&elongation of
male genitalia
3-Pubic
hair
4-Axillary
hair in mid
puberty
28
،‫نيسان‬
23 12
Precocious puberty :
• Definition : onset of breast development
before age of 8 yrs. in girls & onset of
testicular development before age of 9 years
in boys .
•
28
،‫نيسان‬
23 13
Etiology
 Central (gonadotropin dependent true precocity )
 Idiopathic
 Brain lesions
 Hypothalamic hamartoma (most common )
 Brain tumors
 Prolonged untreated hypothyroidism
 Combined peripheral &central
 Treated CAH congenital adrenal hyperplasia
 Familial male precocity
 Peripheral (gonadotropin independent precocious puberty)
28
،‫نيسان‬
23 14
Girls
 Isosexual (feminizing )
o Ovarian tumors
o Teratoma
o Exogenous androgens
o Mc Cune –Albright syndrome
 Heterosexual (masculinizing)
o CAH
o Exogenous androgen
o Adrenal &ovarian tumors
28
،‫نيسان‬
23 15
Boys
 Isosexual ( Masculinizing)
o CAH
o Leydig cell tumor
o hCG secreting tumors
o teratoma
o exogenous androgen
 heterosexual (feminizing )
o exogenous androgen
28
،‫نيسان‬
23 16
28
،‫نيسان‬
23 17
4.5 yrs
Bone ae 9 yrs
10 yrs old female presented at 15 months old age with irregular
bleeding .central precocious puberty
28
،‫نيسان‬
23 18
‫نورالزهراء‬
On decapeptyle GRH
analogs injection
Agenesis of left
parietal lobe
Arachnoid cyst
Clinical features
 hair underarm &genitalia &for boys on face
 acne
 adult body odor
 sexual development ( breast & testes )
 emotional changes
 mood swing
28
،‫نيسان‬
23 19
Diagnosis
o Hormonal levels (sex hormones)
 Low estradiol level or undetected in girls
 High testosterone (detectable in boys
 Detectable level of LH in girls & boys
o bone age (advance) osseous maturation
o ultrasound for adrenal &uterus size
 MRI or CT scan role out tumors
28
،‫نيسان‬
23 20
Treatment :
• GnRH .analogs are effective in arresting
pubertal progression in patient with
central precocity like decapeptyle
(zoladex ) monthly IM injection .
• Medroxyprogesterone acetate some
time used to :suppress puberty and
arrest menses
28
،‫نيسان‬
23 21
Peripheral precocity :
1. Testolactone which inhibit conversion of
testosterone to estrogen
2. Ketoconazole  inhibit steroid biosynthesis
200mg tds
3. Cyproterone acetate  Potent progestin &
antiandrogen, inhibit androgens at the receptor
level / suppress gonadal & adrenal
steroidogenesis : antigonadotrophic 100 mg/m2,,
2 divided doses
28
،‫نيسان‬
23 22
Delayed puberty
• Will be presented in gynecological course in
details by gynecologist lecturer
28
،‫نيسان‬
23 23
Test question
Regarding puberty
a) Cyproterone acetate , inhibit androgens at the receptor level
b) hypothalamic –pituitary –gonadal axis is active in prepubertal state
c) in mid puberty the level of LH become evident even during day time
d) Androgen rather than estrogen are responsible for process of bone
maturation and epiphyseal fusion and cessation of growth
e) Menarche occur immediately after thelarche
28
،‫نيسان‬
23 24
Case scenario
• - As medical student evaluating
a 9-year-old girl for a health
supervision visit. In preparation
for the encounter, you briefly
explain that there is a typical
sequence and timing of
pubertal events.
28
،‫نيسان‬
23 25
• Which of the following sequence of pubertal findings
is MOST often observed?
a. Pubarche, thelarche, menarche, peak
height velocity
b. Pubarche, thelarche, peak height
velocity, menarche
c. Thelarche, pubarche, menarche, peak
height velocity
d. Thelarche, pubarche, peak height
velocity, menarche
28
،‫نيسان‬
23 26
27
Thank
You

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L4 endo.pptx

  • 1.
  • 2. Some definitions Childhood stages (5 stages ) 1- newborn (first 28 days ) 2- infant (first years ) 3- toddler(between 1-3 years ) 4- preschool (between 3-5 years ) 5- school age (6-12 years ) Adolescence: is a period between childhood and adulthood Early adolescence (10-14 years ) Middle adolescence (15-17 years ) Late adolescence (18-21 years )
  • 3. Physiology of puberty L4 – Between early childhood and 8-9 yr of age (prepubertal stage ), the hypothalamic–pituitary– gonadal axis is dormant as reflected by undetected level of LH & sex hormones testosterone in boys and Estradiol in girls . – 1-3 yr before onset of puberty, low serum level of LH demonstrate during sleep in pulsatile fashion. 28 ،‫نيسان‬ 23 3
  • 4. • The pulsatile secretion of gonadotropins is responsible for enlargement and maturation of the gonads and the secretion of the sex hormones 28 ،‫نيسان‬ 23 4
  • 5. • in mid puberty, the level of LH become evident even during day time and occur at about 90- 120 minutes intervals • It is clear that GnRH is the primary if not the only , hormone responsible for the onset and progression of puberty 28 ،‫نيسان‬ 23 5
  • 6. • The effect of gonadal steroid (testosterone and estradiol) on bone growth and osseous maturation are critical , both aromatase deficiency and estrogen receptor defect result in delayed epiphyseal closure and tall stature in affected boys, 28 ،‫نيسان‬ 23 6
  • 7. that is mean estrogen rather than androgen are responsible for process of bone maturation and epiphyseal fusion and cessation of growth Estrogen also mediate the increased production of growth hormone which also responsible with sex hormone for pubertal growth spurt. 28 ،‫نيسان‬ 23 7
  • 9. In female The onset of puberty vary and more correlated with osseous than with chronological maturation age In female, breast bud (thelarche ) is first sign of puberty (10-11yrs) followed by pubic hair (6-12 months later ), the interval to menarche usually 2- 2.5 yrs. peak height velocity usually start early 12 yr of age in girls & always precedes menarche (12.75yr) 28 ،‫نيسان‬ 23 9
  • 10. Stages of female puberty Thelarche10- 11years Pubic hair 6-12 mo. later Menarche 2-2.5 yrs later 28 ،‫نيسان‬ 23 10 Growth spurt precedes menarche
  • 11. In male • In male, growth of testes (>2.5 cm) and thinning of scrotum is the first sign of puberty followed by pigmentation of scrotum and enlargement of penis. pubic hair then appeared. axillary hair appear in mid puberty in male unlike female growth acceleration appears after puberty. in male growth spurt usually 2 yrs later than in females .and growth may continue beyond 18 yrs .of age 28 ،‫نيسان‬ 23 11
  • 12. Stages of male puberty 1- Testes>2.5cm & thinning of scrotum 2-Pigmentation of scrotum &elongation of male genitalia 3-Pubic hair 4-Axillary hair in mid puberty 28 ،‫نيسان‬ 23 12
  • 13. Precocious puberty : • Definition : onset of breast development before age of 8 yrs. in girls & onset of testicular development before age of 9 years in boys . • 28 ،‫نيسان‬ 23 13
  • 14. Etiology  Central (gonadotropin dependent true precocity )  Idiopathic  Brain lesions  Hypothalamic hamartoma (most common )  Brain tumors  Prolonged untreated hypothyroidism  Combined peripheral &central  Treated CAH congenital adrenal hyperplasia  Familial male precocity  Peripheral (gonadotropin independent precocious puberty) 28 ،‫نيسان‬ 23 14
  • 15. Girls  Isosexual (feminizing ) o Ovarian tumors o Teratoma o Exogenous androgens o Mc Cune –Albright syndrome  Heterosexual (masculinizing) o CAH o Exogenous androgen o Adrenal &ovarian tumors 28 ،‫نيسان‬ 23 15
  • 16. Boys  Isosexual ( Masculinizing) o CAH o Leydig cell tumor o hCG secreting tumors o teratoma o exogenous androgen  heterosexual (feminizing ) o exogenous androgen 28 ،‫نيسان‬ 23 16
  • 18. 10 yrs old female presented at 15 months old age with irregular bleeding .central precocious puberty 28 ،‫نيسان‬ 23 18 ‫نورالزهراء‬ On decapeptyle GRH analogs injection Agenesis of left parietal lobe Arachnoid cyst
  • 19. Clinical features  hair underarm &genitalia &for boys on face  acne  adult body odor  sexual development ( breast & testes )  emotional changes  mood swing 28 ،‫نيسان‬ 23 19
  • 20. Diagnosis o Hormonal levels (sex hormones)  Low estradiol level or undetected in girls  High testosterone (detectable in boys  Detectable level of LH in girls & boys o bone age (advance) osseous maturation o ultrasound for adrenal &uterus size  MRI or CT scan role out tumors 28 ،‫نيسان‬ 23 20
  • 21. Treatment : • GnRH .analogs are effective in arresting pubertal progression in patient with central precocity like decapeptyle (zoladex ) monthly IM injection . • Medroxyprogesterone acetate some time used to :suppress puberty and arrest menses 28 ،‫نيسان‬ 23 21
  • 22. Peripheral precocity : 1. Testolactone which inhibit conversion of testosterone to estrogen 2. Ketoconazole  inhibit steroid biosynthesis 200mg tds 3. Cyproterone acetate  Potent progestin & antiandrogen, inhibit androgens at the receptor level / suppress gonadal & adrenal steroidogenesis : antigonadotrophic 100 mg/m2,, 2 divided doses 28 ،‫نيسان‬ 23 22
  • 23. Delayed puberty • Will be presented in gynecological course in details by gynecologist lecturer 28 ،‫نيسان‬ 23 23
  • 24. Test question Regarding puberty a) Cyproterone acetate , inhibit androgens at the receptor level b) hypothalamic –pituitary –gonadal axis is active in prepubertal state c) in mid puberty the level of LH become evident even during day time d) Androgen rather than estrogen are responsible for process of bone maturation and epiphyseal fusion and cessation of growth e) Menarche occur immediately after thelarche 28 ،‫نيسان‬ 23 24
  • 25. Case scenario • - As medical student evaluating a 9-year-old girl for a health supervision visit. In preparation for the encounter, you briefly explain that there is a typical sequence and timing of pubertal events. 28 ،‫نيسان‬ 23 25
  • 26. • Which of the following sequence of pubertal findings is MOST often observed? a. Pubarche, thelarche, menarche, peak height velocity b. Pubarche, thelarche, peak height velocity, menarche c. Thelarche, pubarche, menarche, peak height velocity d. Thelarche, pubarche, peak height velocity, menarche 28 ،‫نيسان‬ 23 26