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Disorders of Puberty
Ummi Habibah1110103000027
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Pembimbing : Dr. Bina Akura, Sp.A
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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 PhysiologyHPG axis
(hypothalamic-pituitary-gonadal) is essential in turning on puberty
at appropriate timesPulsatile 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 ProgressionFemalesThelarcheGenerally considered
the onset of pubertyOccurs in most girls at 9.5-10.4MenarcheMean
age of onset = 12 yrsAdrenarche Usual onset at approx age 9.4-10.6
yrsLinear GrowthGenerally occurs before Tanner Stage 2 breast
developmentGenerally adds 20-25cm of height in femalesGH increases
during puberty as well (provides 50% of growth spurt)
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MalesGonadarcheTesticular 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 = 3yrsThelarche2/3 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 estrogenPubarcheLinear
growthPeak growth generally occurs after Tanner Stage 5Generally
adds 25-30cm in height for males
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Orchidometer.
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Tanner Breast DevelopmentBreasts (female)Tanner I no glandular
tissue; areola follows the skin contours of the chest (prepubertal)
[typically age 10 and younger] Tanner II breast bud forms, with
small area of surrounding glandular tissue; areola begins to widen
[10-11.5] Tanner III 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
increased breast size and elevation; areola and papilla form a
secondary mound projecting from the contour of the surrounding
breast [13-15] Tanner V 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 MalesTanner I prepubertal
(testicular volume less than 3.5 ml ; small penis of 3 cm or less)
[typically age 9 and younger] Tanner II testicular volume between
1.6 and 6 ml; skin on scrotum thins, reddens and enlarges; penis
length unchanged [9-11] Tanner III 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 12 and 20 ml;
scrotum enlarges further and darkens; penis increases in length to
10 cm and circumference [12.5-14] Tanner V testicular volume
greater than 20 ml; adult scrotum and penis of 15 cm in length
[14+]
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Tanner stages.
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Classification disorders of puberty Precocious pubertyDefined as
the onset of secondary sexual characteristics before 8 yr age in
girls and 9 yr in boys.Precocious central of puberty
(Gondotropin-dependent precocius puberty) & precocius perifer
of puberty (Gonadotropin-independent precocius puberty)
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Central (true) precocious pubertyConstitutional (idiopathic)
precocious pubertyHypothalamic neoplasms (most commonly
hamartomas)Congenital malformationsInfiltrative process
(Langerhans-cell type histiocytosis)After
irradiationTraumaInfection
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Precocious puberty of peripheral origin (precocious
pseudopuberty)Gonadotropin-secreting neoplasms:Human chorionic
gonadotropin-secretingEctopic germinomas
(pinealomas)ChoriocarcinomasTeratomasHepatoblastomasLuteinizing
hormon-secreting (pituitary adenomas)Gonadal
neoplasms:Estrogen-secreting:Granulosa-theca cell tumorsGonadal
sex-cord tumorsAndrogen-secreting:ArrhenoblastomasTeratomas**
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Congenital adrenal hyperplasia:21-Hydroxylase (P450c21)
deficiency: the most type11 b -Hydroxylase (P450c11) deficiency3 b
-Hydroxysteroid dehydrogenase deficiencyTreatment hydrocortisone
(10-20 mg/m2 body surface area), mineralocorticoid
replacementAdrenal neoplasms:AdenomasCarcinomasAutonomous gonadal
hypersecretion:CystsMcCune-Albright syndromeIatrogenic
ingestion/absorption of estrogens or androgens**
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Treatment for precocious Central : GnRHaPerifer :Medroksi
progesteron asetatSiproseron asetatKetokonazolTestolakton
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Variation of normal pubertyThelarche prematureAdrenarche
prematureGynecomastia
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Gynecomastia Physiologic pubertal of GynecomastiaNeonatal of
gynecomastia Puberty of gynecomastiaPathologic of
gynecomastiaTumorsHypogonadismHyperthyroidsmChronic disease
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Constitutional Delay Puberty MultifactorialFathers has similar
patternoften in boysNormal size at birth
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Constitutional Delay Puberty
By three years of ageDecrease height ,BA, growth velocityBy
usual age of puberty immaturity become more noticeable as the
approaches with somatic and sexual pubertal At older age than
typicalPuberty occurring spontaneously
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*Constitutional Delay PubertyNo history of systemic
illness.Normal nutrition.Normal P/E.Normal hormones
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*Constitutional Delay PubertyDelayed 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 PubertyGrowth velocity and height are
usually appropriate for bone age Delay in the reactivation of the
GnRH pulse generator Adrenarche and gonadarche occur later
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Constitutional Delay PubertyOutcome is benignNormal physical
development, sexual andreproductive function
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Constitutional Delay PubertyHypogonadotropin 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 familyGH treatment Treatment for
BA>12yDont Treatment for BA
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SHORT STATUREYESPATHOLOGICNORMAL GROWTH VELOCITY?NONORMAL
VARIANTBODY PROPORTION?DYSMORPHISMW/H INDEX?YES
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SHORT STATUREPATHOLOGICPROPORTIONALW/H ENDOKRINGH
DEFICIENCYHYPOTHYROIDCORTISOL EXCESSPSEUDOHYPOPARATHYROIDW/H
MALNUTRITIONCHRONIC INFECTIONCHRONIC DISEASE
(ORGANIC)PSYCHOSOCIALIUGRDYSMORPHIC DYSPROPORTIONATE
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SHORT STATURENORMAL VARIANTFAMILIAL SSCONSTITUTIONAL DELAY OF
GROWTH AND PUBERTYBA = CAFINAL HEIGHT < 3RD
PERCENTILEAPPROPRIATE WITH PGHBA < CAFINAL HEIGHT
=NORMALAPPROPRIATE WITH PGHPOSITIVE FAMILY HISTORY OF CDGP
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