Disorders of Puberty

Pembimbing :
Dr. Bina Akura, Sp.A
Ummi Habibah
1110103000027
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Puberty..
Puberty

is a sensitive phase of
physical, mental, and social
development for both girls and
boys.

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Normal Pubertal
Physiology
-

HPG axis (hypothalamic-pituitarygonadal) is essential in turning on

puberty at appropriate times
Pulsatile secretion of GnRH is essential
GnRH is produced in hypothalamus (in
arcuate nucleus)
GnRH travels to the anterior pituitary to
stimulate the production of LH & FSH

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Age of Pubertal
Progression

Females
Thelarche
Generally considered the onset of puberty
Occurs in most girls at 9.5-10.4

Menarche
Mean age of onset = 12 yrs

Adrenarche
Usual onset at approx age 9.4-10.6 yrs

Linear Growth
Generally occurs before Tanner Stage 2 breast development
Generally adds 20-25cm of height in females
GH increases during puberty as well (provides 50% of
growth spurt)
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Males

Gonadarche

Testicular enlargement generally heralds the onset of

puberty (testes > 4ml). This usually starts around
10.8-11.1 yrs.
Initial increases in testicular size are due to increase in
Sertoli (supporting cells)
Average time to complete genital development = 3yrs

Thelarche

2/3 of males will have gynecomastia develop during

puberty (midpubertal)
Gynecomastia results from direct testicular secretion of
estrogen as well as peripheral conversion of
prohormones to estrogen

Pubarche
Linear growth

Peak growth generally occurs after Tanner Stage 5

Generally adds 25-30cm in height for males

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Orchidometer.

Tanner Breast
Development
Breasts (female)
Tanner I

Tanner II

breast begins to become more elevated, and extends beyond the

borders of the areola, which continues to widen but remains in contour
with surrounding breast [11.5-13]

Tanner IV

breast bud forms, with small area of surrounding glandular tissue;

areola begins to widen [10-11.5]

Tanner III

no glandular tissue; areola follows the skin contours of the chest

(prepubertal) [typically age 10 and younger]

increased breast size and elevation; areola and papilla form a

secondary mound projecting from the contour of the surrounding
breast [13-15]

Tanner V

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breast reaches final adult size; areola returns to contour of the

surrounding breast, with a projecting central papilla. [15+]
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Tanner Staging of Puberty

in Males

Tanner I

Tanner II

testicular volume between 6 and 12 ml; scrotum enlarges

further; penis begins to lengthen to about 6 cm [11-12.5]

Tanner IV

testicular volume between 1.6 and 6 ml; skin on scrotum thins,

reddens and enlarges; penis length unchanged [9-11]

Tanner III

prepubertal (testicular volume less than 3.5 ml ; small penis of

3 cm or less) [typically age 9 and younger]

testicular volume between 12 and 20 ml; scrotum enlarges

further and darkens; penis increases in length to 10 cm and
circumference [12.5-14]

Tanner V

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testicular volume greater than 20 ml; adult scrotum and penis

of 15 cm in length [14+]
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Tanner stages.

Classification disorders of
puberty
Precocious

puberty
Defined as the onset of secondary sexual
characteristics before 8 yr age in girls and 9 yr
in boys.
Precocious central of puberty (Gondotropindependent precocius puberty) & precocius
perifer of puberty (Gonadotropin-independent
precocius puberty)

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Central (true) precocious

puberty
Constitutional (idiopathic) precocious
puberty
Hypothalamic neoplasms (most commonly
hamartomas)
Congenital malformations
Infiltrative process (Langerhans-cell type
histiocytosis)
After irradiation
Trauma
Infection

Precocious puberty of
peripheral origin (precocious
pseudopuberty)
Gonadotropin-secreting neoplasms:

Human chorionic gonadotropin-secreting

Ectopic germinomas (pinealomas)
Choriocarcinomas
Teratomas
Hepatoblastomas
Luteinizing hormon-secreting (pituitary adenomas)

Gonadal neoplasms:

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Estrogen-secreting:
Granulosa-theca cell tumors
Gonadal sex-cord tumors
Androgen-secreting:
Arrhenoblastomas
Teratomas
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Congenital adrenal hyperplasia:

21-Hydroxylase (P450c21) deficiency: the most type

11 b -Hydroxylase (P450c11) deficiency
3 b -Hydroxysteroid dehydrogenase deficiency

Treatment hydrocortisone (10-20 mg/m2 body

surface area), mineralocorticoid replacement
Adrenal neoplasms:
Adenomas
Carcinomas

Autonomous gonadal hypersecretion:

Cysts
McCune-Albright syndrome

Iatrogenic ingestion/absorption of estrogens or

androgens

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Treatment for precocious

Central

GnRHa
Perifer

:
Medroksi progesteron asetat
Siproseron asetat
Ketokonazol
Testolakton
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Variation of normal
puberty
Thelarche

premature
Adrenarche premature
Gynecomastia

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Gynecomastia

Physiologic

pubertal of
Gynecomastia
Neonatal of gynecomastia
Puberty of gynecomastia
Pathologic of gynecomastia
Tumors
Hypogonadism
Hyperthyroidsm
Chronic disease
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Constitutional Delay
Puberty
Multifactorial
Fathers

has similar pattern

often in boys
Normal size at birth
.

.
.

Constitutional Delay Puberty

By three years of age
Decrease height ,BA, growth velocity
By usual age of puberty
immaturity become more noticeable as the
approaches with somatic and sexual
pubertal
At older age than typical
Puberty occurring spontaneously

Constitutional Delay
Puberty
No

history of systemic illness.

Normal nutrition.
Normal P/E.
Normal hormones

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Constitutional Delay
Puberty
Delayed

puberty.
Delayed bone age.
a short adolescent with bone age delay
greater than three years is more likely to
have a pathologic problem .

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Constitutional Delay
Puberty
Growth

velocity and height are

usually appropriate for bone age
Delay in the reactivation of the
GnRH pulse generator
Adrenarche and gonadarche occur
later

Constitutional Delay Puberty

Outcome

is benign
Normal physical development,
sexual and
reproductive function

Constitutional Delay
Puberty
Hypogonadotropin
hypogonadism
Adrenarche at a normal age
Higher DHEAS than CDG
Failure

of a rise in Gonadotropin
or sex steroid by age 18

Treatment
Assurance to family
GH treatment
Treatment for BA>12y
Dont Treatment for BA<10y or CA<12
Oxandrolon
Transdermal patch and gel
preparations of testosterone

SHORT
SHORTSTATURE
STATURE
NORMAL
NORMALGROWTH
GROWTHVELOCITY?
VELOCITY?

YES
YES
YES
YES

NO
NO

NORMAL
NORMAL
VARIANT
VARIANT

PATHOLOGIC
PATHOLOGIC

BODY
BODYPROPORTION?
PROPORTION?

W/H
W/HINDEX?
INDEX?

DYSMORPHISM
DYSMORPHISM

SHORT
SHORTSTATURE
STATURE
PATHOLOGIC
PATHOLOGIC
PROPORTIONAL
PROPORTIONAL
W/H
W/H

ENDOKRIN
ENDOKRIN
GH
GHDEFICIENCY
DEFICIENCY
HYPOTHYROID
HYPOTHYROID
CORTISOL
CORTISOLEXCESS
EXCESS
PSEUDOHYPOPARATHYR
PSEUDOHYPOPARATHYR
OID
OID

DYSPROPORTIONATE
DYSPROPORTIONATE
W/H
W/H

MALNUTRITION
MALNUTRITION
CHRONIC
CHRONICINFECTION
INFECTION
CHRONIC
CHRONICDISEASE
DISEASE
(ORGANIC)
(ORGANIC)
PSYCHOSOCIAL
PSYCHOSOCIAL
IUGR
IUGR

DYSMORPHIC
DYSMORPHIC

SHORT
SHORTSTATURE
STATURE

NORMAL
NORMALVARIANT
VARIANT

FAMILIAL
FAMILIALSS
SS

BA
BA==CA
CA
RD
FINAL
FINALHEIGHT
HEIGHT<<33RD
PERCENTILE
PERCENTILE
APPROPRIATE
APPROPRIATEWITH
WITH
PGH
PGH

CONSTITUTIONAL
CONSTITUTIONALDELAY
DELAYOF
OF
GROWTH
GROWTHAND
ANDPUBERTY
PUBERTY

BA
BA << CA
CA
FINAL
FINALHEIGHT
HEIGHT=NORMAL
=NORMAL
APPROPRIATE
APPROPRIATEWITH
WITHPGH
PGH
POSITIVE
POSITIVEFAMILY
FAMILYHISTORY
HISTORY
OF
CDGP
OF CDGP

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