By Umoh Emmanuel

WHAT IS PRECOCIOUS PUBERTY?This is the onset of sexual maturation at any

age that is 2.5 SD earlier than the normal age for the population.

In other words: development of sexual maturation before the ages of 8-9 years in girls and boys.

CLASSIFICATIONI. TRUE PRECOCIOUS PUBERTY (GnRH-Dependant):a. Idiopathicb. CNS lesions: Hamartomas, Craniopharyngioma, etcc. Primary hypothyroidism II. PSEUDOPRECOCIOUS PUBERTY ( GnRH-independent):a. Isolated precocious thelarcheb. Isolated precocious menarchec. Estrogen-secreting tumors of the ovary or adrenals in girls d.  Ovarian cysts e. McCune-Albright syndrome   f. Peutz-Jeghers syndrome   g. IatrogenicIII.  CONTRASEXUAL PRECOCITY (ISOLATED VIRILIZATION):a. Isolated precocious adrenarcheb. Congenital adrenal hyperplasia   c. Androgen-secreting ovarian or adrenal neoplasm    d. Iatrogenic

NORMAL PUBERTAL DEV. CHART

True Precocious Puberty This results from early maturation of the

hypothalamic- pituitary-gonadal axis.Serum gonadotropins, gonadal pulsitality and

sex steroid concentrations are in the normal postpubertal range.

idiopathic precocious puberty seems to be the most common cause of CPP.

Neurogenic TPP seems to be found more frequently in extremely young girls with the earliest onset of puberty.

EtiologyCNS lesions identified include neoplasms, trauma,

hydrocephalus, postinfectious encephalitis, congenital brain defects, and such genetic disorders as neurofibromatosis type 1 and tuberous sclerosis.

The most commonly identified neurogenic neoplasms found in TPP include hamartomas, astrocytomas, and pituitary microadenomas

Hamartomas are congenital hypothalamic malformations that histologically contain fiber bundles, glial cells and GnRH- secreting neurons and often act as a mini-hypothalamus.

Contd.Girls with severe primary hypothyroidism can

develop true precocious puberty.These girls have elevated gonadotropins in

addition to high TSH levels.The associated precocity may result from

cross-activation of the FSH receptor by the high circulating TSH or from direct stimulation of the ovary by the gonadotropins.

MANAGEMENTDIAGNOSIS:The management of true precocious puberty

requires identification of underlying CNS lesions, if present, or in other children identification of a pubertal gonadotropin response to GnRH that is usually associated with idiopathic true precocious puberty and occasionally with a hamartoma.

Hence we do: Imaging of the CNS and a GnRH challenge test.

Contd. bone age X-rays are helpful to identify the

advance physiologic age associated with true precocious puberty.

Ovarian imaging, thyroid and hCG testing may also compliment the evaluation.

FSH AND LH LEVELS.ULTRASOUND OF THE ADRENAL GLANDS.

TANNER STAGING

TANNER STAGING 2

ORCHIDOMETER

TREATMENTAdminister GnRH analogues: they are

modifications of the native hormones which have greater resistance to degradation and increased affinity for the pituitary GnRH receptors.

They induce down-regulation of receptor function, resulting in temporary, reversible inhibition of the hypothalamic-pituitary-ovarian axis as reflected by minimal or no response to GnRH stimulation and regressionof the manifestation of puberty.

Gonadotropic Independent Preococious Puberty (GIPP)GIPP can originate from the gonads, the

adrenals, from extragonadal or intragonadal sources of human chorionic gonadotropin, or from exogenous sources.

In girls, functionally autonomous ovarian cysts are the most common cause of GIPP.

Ovarian follicles up to 8mm in diameter are common in normal prepubertal girls and may appear or regress spontaneously, but rarely secrete significant amounts of estrogen

McCune-Albright syndromeclassically includes the triad of hyperpigmented caf?au-lait spots progressive polystotic fibrous dysplasia of the bones

and GnRH-independent sexual precocity.At least 2 of these features must be present to consider

the diagnosis.The sexual precocity of McCune Albright syndrome is

due to autonomously functioning follicular cystsTestolactone, an aromatase inhibitor , has been shown

to be effective treatment for the GnRH independent phase of this condition.

When the shift from gonadotropin independent to gonadotropin dependent puberty takes place, GnRH analog therapy then becomes effective.

Image of McCune-Albright syndrome

PREMATURE THELARCHEIsolated development of the breast tissue

prior to age 8 yrs, most commonly occurring between 1 and 3 years of age. It may affect 1 or both breasts.

On examination, the somatic growth pattern is not accelerated, bone age is not advanced and smear of vaginal secretion fails to show estrogen effect.

Occurs on exposure to exogenous estrogen, as happened in Puerto Rico in the 1970’s.

Image of premature thelarche

PREMATURE PUBARCHEDefined as the appearance of pubic or axillary hair

prior to age 7 years in white girls and 6 years in black girls. Such hair growth may be idiopathic and of clinical significance.

It usually results from an earlier than-usual increase in the secretion of androgens by adrenal glands.

Thorough evaluation of the gonadal and adrenal function should be made to exclude such abnormalities.

Signs of sever androgen excess( clitoral enlargement, growth acceleration, acne) should prompt further investigation for rare virilazation tumor.

Pathogenesis of pp.

PREMATURE MENARCHEDenotes the appearance of cyclic vaginal

bleeding in children in the absence of other signs of secondary sexual development.

It could be related to increased end-organ sensitivity of the endometrium to low prepubertal levels of estrogens.

Diagnosis is formulated by exclusion following investigation of other causes of vaginal bleeding and confirmed when the cyclic nature of the bleeding becomes apparent.

Contrasexual precocityMost girls with contrasexual precocious puberty

present with early appearance of pubic hair or hirsuitism.

The most common cause is a mild form of 21-hydroxylase deficiency , which is present in 0.1-1.0% of the population.

Other more rare forms of congenital adrenal hyperplasia have also been identified in these patients.

Virilizing adrenal (occasionally malignant) and ovarian tumors (e.g., Leydig or Sertoli cell tumors) in young girls can similarly present with virilizing precocious puberty.

CAH

EVALUATION OF PATIENTS WITH PRECOCIOUS PUBERTY

GENERAL CHANGES:Enhancement of general growth is coincident with the

onset of estrogen-stimulated change. The child often exhibits accelerated growth velocity, tall stature for age, and advanced skeletal maturation.

SKIN:Additional androgen-dependent findings include, acne and

adult-type body odor.BREAST:According to TANNER, it is at stage II with areolae having

a broadened, darkened appearance.GENITALIA:Genital changes reflect estrogen-induced thickening of the

genital tissues. Increased vaginal secretions may result in leukorrhea. Dark, coarse pubic hair may be present.

How an Individual Can Cope with Precocious Puberty Educate Yourself About the ChangesRealize that there are a variety of body types —

big, small, and everything in between.Try not to compare yourself with those around

you.Avoid those with negative outlooks; surround

yourself with those who care about youTalk to someone you trust, they could offer

suggestions and make you feel a little less aloneAvoid those with negative outlooks; surround

yourself with those who care about you.Talk to someone you trust, they could offer

suggestions and make you feel a little less alone.

Warning Signs of Effects on Emotional Developmentpoor grades

problems at schoolloss of interest in daily activities anddepression

THE ENDGive your child with precocious puberty OR

your friend or Patient love and support!