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Precocious puberty causes, types and treatment 2023 | by Dr.lalit b.

     PRECOCIOUS PUBERTY



Defination:


The term precocious puberty is reserved for girls who 

exhibit any secondary sex characteristics before the 

age of 8 or menstruate before the age of 10.

Precocious puberty may be isosexual where the 

features are due to excess production of estrogen. It 

may be heterosexual where features are due to excess 

production of androgen (from ovarian and adrenal 

neoplasm).


            Precocious puberty for little boy



Causes of precocious puberty :


GnRH dependent—80% (complete, central,

isosexual or true)

constitutional—most common

Juvenile primary hypothyroidism

Intracranial lesions—trauma, tumor or infection

Incomplete

Premature thelarche

Premature puberche

Premature menarche


GnRH independent

(precocious pseudopuberty or peripheral)

(excess estrogen or androgen)

Ovary

Granulosa cell tumor

Theca cell tumor

leydig cell tumor

chorionic epithelioma

androblastoma

mccune-albright syndrome

adrenal

Hyperplasia

Tumor

Liver

Hepatoblastoma

Iatrogenic

estrogen or androgen intake


Pathology :


constitutional

It is due to premature activation of hypothalamo -

pituitary ovarian axis. There is secretion of

gonado tropins and gonadal steroids due to premature

release of GnRH. Bone maturation is accelerated,

leading to premature closure of the epiphysis and

curtailed stature. If menstruation occurs, they may

be ovulatory. The changes in puberty progress in an

orderly sequence.


intracranial lesions

Meningitis, encephalitis, craniopharyngioma, neuro-

fibroma or any tumor—hypothalamic or pineal gland.

Mccune-albright syndrome is characterized

by sexual precocity, multiple cystic bone lesions

(polyostotic fibrous dysplasia), endocrinopathies

and café-au-lait spots on the skin. Sexual precocity is

due to early and excessive estrogen production from

the ovaries. FSH, LH levels are low. There may be

associated hyperthyroidism, hyperparathyroidism,

and acromegaly.



      BMI risk factor on precocious puberty 


Premature thelarche

It is the isolated development of breast tissue before

the age of 8 and commonly between 2 and 4 years of

age. Either one or both the breasts may be enlarged

(Fig. 5.2). There is no other feature of precocious

puberty. It generally requires no treatment.


Premature pubarche

Premature pubarche is isolated development of

axillary and or pubic hair prior to the age of 8

without other signs of precocious puberty. The

premature hair growth may be due to unusual

sensitivity of endorgans to the usual low level of

hormones in the blood during childhood. Rarely,

there may be signs of excess androgen production

due to adrenal hyperplasia or tumor or androgenic

ovarian tumor (Leydig cell tumor, androblastoma,

etc.).


Premature menarche

Premature menarche is an isolated event of

cyclic vaginal bleeding without any other signs of

secondary sexual development. The cause remains

unclear but may be related to unusual endocrine

sensitivity of the endometrium to the low level of

estrogens.

Chorionic epithelioma, hepatoblastoma are the

ectopic sources of human chorionic gonadotropin and

may cause sexual precocity.



Diagnosis


True precocious

Constitutional type is the commonest one but the

rare one is to be kept in mind. The diagnosis is made

by:

♦ History of early menarche of mother and sisters

♦ The pubertal changes occur in orderly sequence

♦ Tanner stages

♦ No cause could be detected.

The basic investigations, to confirm or to exclude

some pathologic lesions, include:

♦ X-ray hand and wrist (non-dominant) for bone

age. Acceleration of growth is one of the earliest

clinical features of precocious puberty

♦ Pelvic sonography to exclude ovarian pathology

♦ Skull X-ray, CT scan, or MRI brain—to exclude

intracranial lesion

♦ Serum hCG, FSH, LH

♦ Thyroid profile (TSH, T4)

♦ Serum estradiol, testosterone, 17 OH proge-

sterone, dehydroepiandrosterone (DHEA).

♦ Electroencephalogram.



Treatment :


Constitutional or idiopathic type

The goals are:

 To reduce gonadotropin secretions.

 To suppress gonadal steroidogenesis or coun-

teract the peripheral action of sex steroids.

 To decrease the growth rate to normal and slowing

the skeletal maturation.

 To protect the girl from sex abuse.


The drugs used are:

 GnRH agonist therapy arrests the pubertal

precocity and growth velocity significantly. The

agonists suppress the premature activation of

hypothalamopituitary axis due to down regulation

and thereby diminished estrogen secretion.

GnRH agonist therapy is the drug of choice

in cases with GnRH dependent precocious

puberty. Therapy should be started as soon

as the diagnosis is established. GnRH agonist

therapy suppresses FSH, LH secretion, reverses

the ovarian cycle, establishes amenorrhea,

causes regression of breast, pubic hair changes,

and other secondary sexual characteristics. This

drug should be continued till the median age of

puberty.

Dose: Buserelin nasal spray 100 mg daily. It can slow

down the process of skeletal maturation. Depot forms

(goserelin or leuprolide) once a month can be used

. Dose is adjusted to maintain the serum

estradiol below 10 pg/mL.

Medroxyprogesterone acetate—30 mg daily

orally or 100–200 mg. IM weekly to suppress

gonadal steroids. It can suppress menstruation

and breast development but cannot change the

skeletal growth rate.

Cyproterone acetate—It acts as a potent

progestogen, having agonist effects on proge-

sterone receptors.

Dose—70–100 mg/m2

/day orally for 10 days starting

from 5th day of cycle.

Danazol—It produces amenorrhea and arrest

breast development. But there is no effect on

growth rate or skeletal maturation.

Duration of therapy

The drugs should be used up to the age of 11 years.

However, individualization is to be done.


~ Thank you for reading .......

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