17. kuliah 1 precociuos puberty

8/12/2019 17. Kuliah 1 Precociuos Puberty

1/33

Wy Bikin Suryawan/Md Arimbawa


2/33


3/33

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


4/33

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


5/33

Definition

Appearance of secondary

sexual characteristics : boys

< 9 years and girls < 8 yearsold (- 2SD)

Sex steroid Estrogen: female

Testosterone:male


6/33

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


7/33

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


8/33

Variant

premature thelarche

premature adrenarche

gynecomastia


9/33

idiopathic

CNS tumor

non-tumor: post infection, radiation, trauma,

congenital

iatrogenic

Delayed diagnosis of GIPP


10/33

Always isosexual

Normal sequence of puberty

Hormonal profile: increased gonadotropin

and sex steroid


11/33

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


12/33

Isosexual)

McCune Albright

Severe hypothyroid

heterosexual adrenal: tumor, CAH

tumor ovarium:

arrhenoblastoma


13/33

Trias

Precocious puberty /

endocrine hyperactivity

Fibrodysplasia Caf au lait


14/33

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


15/33

self-limited condition occurring before sixyears of age

characterized by the appearance of pubic

and no further secondary sexualdevelopment.normal growth patterns


16/33

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


17/33

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


18/33

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


19/33

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


20/33

Breast enlargement in males

common in teenage years, lasting 2 years

differentiate with obese boys lipomastia

no mammae disk

Pathological causes must be sought


21/33

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.


22/33

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


23/33

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


24/33

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.


25/33

Gonadotropin dependent or independent?

Etiology?


26/33

Hypothalamus

Pituitary

GnRH

Gonad

LH/FSH

E2or T

(-)

H-P-G axis


27/33

Hypothalamus

Pituitary

GnRH

Gonad

LH/FSH

Sex steroid

(-)

H-P-G axis in GDPP

Primary


28/33

Hypothalamus

Pituitary

GnRH

Gonad

LH/FSH

(-)

Extra Gonadal

H-P-G axis in GIPP

Sex steroid


29/33

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),


30/33

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),


31/33

According to the etiology

GDPP idiopathic: GnRH agonis

GIPP : medroxy-progesteron,

ketoconazole, dllVariant: observation


32/33

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


33/33

Not all pubertal disorders are pathologic

Early increase of sex steroid should be

thoroughly investigated

GnRH agonist = drug of choice forGDPP

17. kuliah 1 precociuos puberty

Documents