PUBERTY
Puberty
The cascade of events initiated by the
release of pulsatile GnRH from
prepubertal and central negative
inhibition that results in increased
levels of gonadotropin and steroids
with appearance of secondary sexual
characteristics and eventual adult
function (menarche, ovulation)
Stages of Pubertal Development
1. Accelerated growth
2. Adrenarche
3. Breast development
(Thelarche)
4. Menarche
Adrenarche
The growth of pubic and axillary hair that is due
to an increased production of adrenal androgens
Stage 1: Pre-adolescent
Adrenarche Vellus hair only and hair is similar to development
over anterior abdominal wall (ie. no pubic hair)
Stage 2
There is sparse growth of long, slightly pigmented,
downy hair or only slightly curled hair, appearing
along labia
Stage 3
Hair is darker, coarser, more curled, and spreads to
the pubic junction
Stage 4
Adult-type hair; area covered is less than that in
most adults; there is no spread to the medial
surface of thighs
Stage 5: Adult
Adult-type hair with increased spread to medial
surface of thighs; distribution is as an inverse
triangle
THELARCHE
1. Preadolescent
Only papilla is elevated
2. Breast bud stage
Breast and papilla are elevated as small
mound. Areola diameter is enlarged
3. Further enlargement with no separation
of their contours
4. Areola and papilla project to form a
secondary mound above the level of
the breast
5. Mature stage
Projection only of papilla because of
recession of areola to general contour
of the breast
1. FSH and then LH levels rise moderately before the
age of 10 and are followed by a rise in estradiol
2. As gonadal estrogen increases (gonadarche), breast
development, female fat distribution, and vaginal and
uterine growth occur. Skeletal growth rapidly increases
as a result of initial gonadal secretion of low levels of
estrogen, which increases the secretion of growth
hormone, which in turn stimulates the production of
IGF-I
Stages of Pubertal Development
3. Adrenal androgen (adrenarche) and, to a lesser
degree, gonadal androgen secretion cause pubic and
axillary hair growth.
4. At midpuberty, sufficient gonadal estrogen secretion
proliferates the endometrium, and the first menses
(menarche) occurs
5. Postmenarcheal cycles are initially anovulatory.
Sustained, predictable positive LH surge responses to
estradiol with ovulation are late pubertal events
Etiology
1. Hypothalamus or pituitary
fail to provide appropriate
gonadotropin
2. Ovaries fail to produce
adequate amounts of
estradiol
3. Ovulatory dysfunction
4. Endometrial tissue
abnormalities
5. Obstruction of outflow
tract
Primary amenorrhea
Secondary sexual characteristic
No Yes
Uterus Uterus
Uterus absent Uterus present Uterus absent Uterus present
Karyotype FSH Karyotype Outflow obstruction
Low High XX XY Hymen imperforata
XY
Karyotype
PAIS Rokitansky CAIS
XX XO
CAH HH Turner
15% of primary amenorrhea
Normal secondary
development & external
female genitalia
Normal female range
testosterone level
Absent uterus and upper
vagina
Karyotype 46-XX
15-30% renal, skeletal and
middle ear anomalies
Imperforate Hymen
Androgen Insensitivity
Normal breasts but no sexual hair
Normal looking female external genitalia
Absent uterus and upper vagina
Karyotype 46, XY
Male range testosterone level
Treatment
- gonadectomy after puberty + HRT
Turner Syndrome
FSH 156 mIU / ml
LH 34,6 mIU / ml
E2 < 20,0 pg / ml
Kromosom 45, XO
Secondary amenorrhea
Secondary amenorrhea
Ashermans Syndrome
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