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8/12/2019 17. Kuliah 1 Precociuos Puberty
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Wy Bikin Suryawan/Md Arimbawa
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Epidemiology Frequency : girls > boys
Girls: most have a benign central cause
Boys: 50% pathologic peripheral cause.
all boys with precocious puberty should undergodetailed investigation, but in girls additional
investigation can be based on the clinical
impression
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Profiles of Girls with Precocious Puberty(N=438)
Age of onset
between 7-7.9 year olds
6 year olds
< 6 years old.
59.6%
22.4%
18%
Etiology
Gonadotropin Dependent
Gonadotropin independent
97.7%
2.3%
Neurogenic abnormalities(MR/CT skull)
18.4%
Cisternino M, Arrigo T, Pasquino AM, et al. Etiology and Age Incidence of Precocious Puberty in Girls:
A Multicentric Study. J Pediatr Endocrinol Metab.2000;13(suppl 1):695-701
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Definition
Appearance of secondary
sexual characteristics : boys
< 9 years and girls < 8 yearsold (- 2SD)
Sex steroid Estrogen: female
Testosterone:male
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Estrogen Accelerated bone maturation and early epiphyseal
fusion (tall child but short adult)
Uterus, mammary gland Testosterone
genital
Hirsutism, acne, male habitus
General sexual behavior, aggressiveness
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GnRH dependent(central) :
premature reactivation hypothalamus-pituitary-gonad
axis increased gonadotropin increased sex
steroids (dependent) Usually idiopathic
GnRH independent(peripheral):
autonomous sex steroid secretion, depressing the
hypothalamus-pituitary-gonad axis Usually pathologic
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Variant
premature thelarche
premature adrenarche
gynecomastia
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idiopathic
CNS tumor
non-tumor: post infection, radiation, trauma,
congenital
iatrogenic
Delayed diagnosis of GIPP
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Always isosexual
Normal sequence of puberty
Hormonal profile: increased gonadotropin
and sex steroid
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Isosexual adrenal: tumor, CAH
testes : cell Leydig tumor, familial testotoxicosis
gonadotropin-secreting tumor: non CNS: hepatoma, germinoma, teratoma
CNS: germinoma, adenoma (LH secreting)
heterosexual
Increased peripheral aromatization
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Isosexual)
McCune Albright
Severe hypothyroid
heterosexual adrenal: tumor, CAH
tumor ovarium:
arrhenoblastoma
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Trias
Precocious puberty /
endocrine hyperactivity
Fibrodysplasia Caf au lait
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Isosexual or heterosexual (late onset CAH,
tumor adrenal)
Disconcordant of sexual characteristics
(testes volume inappropriate with pubertal
stage - smaller)
Low or normal gonadotropin and increased
sex steroid
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self-limited condition occurring before sixyears of age
characterized by the appearance of pubic
and no further secondary sexualdevelopment.normal growth patterns
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Normal bone ageSlight elevation of serum DHEANormal adrenal steroid hormone levels
Normal sex hormone levelsACTH stimulation test: to exclude late-onset CAH
GnRH test: prepubertal patternNormal imaging studiesNo specific treatment required
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Excude virilization clitoral enlargement, advanced bone age, acne,
rapid growth, and voice change.
rapid progression If virilization present
measure testosterone, 17-OHP and DHEA
USG: adrenal or ovarian tumor 17-OHP or DHEA: CAH
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Isolated appearance of unilateral or
bilateral breast aged 6 months to 3 years
No other signs of puberty or evidence of
excessive estrogen effect (thickening of
the vaginal secretions or bone age
acceleration).
Ingestion or application of estrogen-containing compounds must be excluded
as etiology
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Normal growth rate and bone ageNormal levels of gonadotropins and
estradiol
USG: normal ovaries, prepubertal uterusUsually resolves spontaneously andrequires no treatment
re-evaluation at intervals of 6-12 months toensure that premaure thelarche is not thebeginning of isosexual precocious puberty
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Breast enlargement in males
common in teenage years, lasting 2 years
differentiate with obese boys lipomastia
no mammae disk
Pathological causes must be sought
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Incidence: 50-60% of boys during earlyadolescence
breast tissue usually asymmetric and often
tender. If history and physical examination,
including palpation of the testicles, areunremarkable, reassurance and periodicreevaluation are all that is necessary. Mostcases resolve in one to two years.
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Drugs
sex steroids, hCG, psychoactive
(phenotiazine), antituberculosis,
testosterone antagonist
(ketoconazole, cimetidine,spironolactone)
Malnutrition
Idiopathic (most common)
Tumor producing disease
hepatoma, adrenal, testes, LHand hCG producing tumors
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Familial gynecomastia
X-linked recessive trait or a sex-limited dominant trait
unless associated with hypogonadism no further
evaluation in an otherwise normal boy If severe, gynecomastia cosmetic surgery.
Pathologic gynecomastia
Klinefelter's syndrome: high risk for breast cancer
prolactin-secreting adenomata
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Pathologic gynecomastia
hormone-secreting tumors (testes, hepatoma),
cirrhosis, hypo- and hyperthyroidism.
Drug induced (marijuana, phenothiazines, opiates,amphetamines, digitalis, estrogens, ketoconazole,
spironolactone, isoniazid, tricyclic antidepressants,
cimetidine, etc).
If worsens and associated with psychologic
morbidity bromocriptine, tamoxifen reduction mammoplasty rarely indicated.
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Gonadotropin dependent or independent?
Etiology?
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Hypothalamus
Pituitary
GnRH
Gonad
LH/FSH
E2or T
(-)
H-P-G axis
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Hypothalamus
Pituitary
GnRH
Gonad
LH/FSH
Sex steroid
(-)
H-P-G axis in GDPP
Primary
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Hypothalamus
Pituitary
GnRH
Gonad
LH/FSH
(-)
Extra Gonadal
H-P-G axis in GIPP
Sex steroid
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History
age of onset, progressivity, family history, growth,
symptoms extragonadal cause (adrenal), CNS
complaints, gelactic laughter (hamartoma),previous history: encephalitis, meningitis TB
Physical examination
pubertal stage, signs of virilisation, height, testes
size (small indicative of perpheral cause), CNSsigns, skin (acne, caf au lait),
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Laboratory
gonadotropin, bHCG, 17-OHProgesterone(CAH), cortisol (Cushing syndrome,
adrenal tumor) Imaging
Bone age, pelvic ultrasound, skull x-ray,
CT/MRI, bone survey (McCune Albright),
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According to the etiology
GDPP idiopathic: GnRH agonis
GIPP : medroxy-progesteron,
ketoconazole, dllVariant: observation
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According to etiology GDPP idiopathic: GnRH agonis
Final height = potential genetic height
Preserved fertility
Psychosocial minimal, regression of secondary sex
GIPP : medical
Potential genetic height
Regression of secondary sex
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Not all pubertal disorders are pathologic
Early increase of sex steroid should be
thoroughly investigated
GnRH agonist = drug of choice forGDPP