This document discusses precocious puberty, which is the early onset of sexual maturation before age 8-9. It classifies types as true (GnRH-dependent) or pseudo (GnRH-independent) precocity. True precocity is usually idiopathic and can be caused by brain lesions or hypothyroidism. Pseudo precocity has causes like ovarian cysts. Evaluation involves physical exams, blood tests, and imaging. Treatment depends on type but may include GnRH analogs. Supporting emotional health for those with precocious puberty is important.

1. By Umoh Emmanuel

3. 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.

4. CLASSIFICATION
p TRUE PRECOCIOUS PUBERTY (GnRH-Dependant):
b. Idiopathic
c. CNS lesions: Hamartomas, Craniopharyngioma, etc
d. Primary hypothyroidism
V. PSEUDOPRECOCIOUS PUBERTY ( GnRH-independent):
f. Isolated precocious thelarche
g. Isolated precocious menarche
h. Estrogen-secreting tumors of the ovary or adrenals in girls
i. Ovarian cysts
j. McCune-Albright syndrome
k. Peutz-Jeghers syndrome
l. Iatrogenic
III. CONTRASEXUAL PRECOCITY (ISOLATED VIRILIZATION):
 Isolated precocious adrenarche
 Congenital adrenal hyperplasia
 Androgen-secreting ovarian or adrenal neoplasm
 Iatrogenic

5. NORMAL PUBERTAL DEV. CHART

6. 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.

7. Etiology
 CNS 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.

8. 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.

9. MANAGEMENT
 DIAGNOSIS:
 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.

10. 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.

11. TANNER STAGING

12. TANNER STAGING 2

13. ORCHIDOMETER

14. TREATMENT
 Administer 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.

15. 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

16. McCune-Albright syndrome
 classically 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 cysts
 Testolactone, 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.

17. Image of McCune-Albright
syndrome

18. PREMATURE THELARCHE
 Isolated 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.

19. Image of premature thelarche

20. PREMATURE PUBARCHE
 Defined 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.

21. Pathogenesis of pp.

23. PREMATURE MENARCHE
 Denotes 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.

24. Contrasexual precocity
 Most 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.

25. CAH

26. 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.

27. How an Individual Can Cope with
Precocious Puberty
 Educate Yourself About the Changes
 Realize 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 you
 Talk to someone you trust, they could offer suggestions
and make you feel a little less alone
 Avoid 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.

28. Warning Signs of Effects on
Emotional Development
 poor grades
problems at school
loss of interest in daily activities and
depression

29. THE END
 Give your child with precocious puberty OR
your friend or Patient love and support!