Puberty and precocious puberty (DOW)

Question 1

Thelarche

Answer 1

is the appearance of breast tissue

Usually the first and most obvious sign of early puberty in girls

Question 2

Adrenarche

Answer 2

is the activation of the adrenal cortex for the production of adrenal androgens, and typically occurs before the onset of puberty.
Pubic and axillary hair may appear before, at about the same time, or well after the appearance of breast tissue

Question 3

Mini-puberty of infancy

Answer 3

The hypothalamic-pituitary-gonadal axis is biologically active in infancy with peak activity between six and eight weeks of age. This state yields sex steroid levels comparable with those seen in early-to-mid puberty, but without peripheral effects.
Hormonal changes after birth: falling levels of maternal estrogens
65-90% of neonates have breast tissue
Vaginal discharge can be observed.
“Witches’ milk” from breasts and even menses can be seen
* Neonatal acne - usually just comedonal in nature- also common (infantile can also develop)

Question 4

Suppression of HPA in puberty

Answer 4

Suppression of hypothalamic-pituitary-gonadal axis is released;
Secretion of high-amplitude pulses of gonadotropin-releasing hormone (GnRH) by the hypothalamus 
Pulsatile increases in the pituitary gonadotropin-luteinizing hormone (LH) and follicle-stimulating hormone (FSH)
Increased LH levels stimulate production of sex steroids by testicular Leydig cells or ovarian granulosa cells.

Question 5

androgens in puberty

Answer 5

Androgens or estrogens at pubertal levels cause the physical changes of puberty
Penile enlargement and sexual hair in boys
Breast development in girls, sexual hair
Pubertal growth spurt.

Question 6

FSH and puberty

Answer 6

Increased FSH levels cause enlargement of the gonads in both sexes and eventually promote follicular maturation in girls and spermatogenesis in boys.

Question 7

genetics and pubertal timing

Answer 7

African American girls begin puberty earlier than Caucasian girls.

Question 8

Puberty Related Issues

Answer 8

anemia
gynecomastia
acne
psychological correlates of puberty
some sports-related injuries
myopia
scoliosis
STIs
dysfunctional uterine bleeding

Question 9

testicular volume and puberty

Answer 9

: early sign of puberty: testicular length is more than 2.5 cm or testicular volume (with Prader orchidometer beads) is 4 mL or more

Question 10

Tanner breast bud stage

Answer 10

stage 2
3- curvy no pokey thing
4- pokey like the number 4

Question 11

Boys’ external genitalia stage 2

Answer 11

Enlargement of scrotum and testes;

Question 12

Precocious Puberty

Answer 12

Puberty before
Age 8 for girls
Age 9 for boys

Question 13

bone maturation and precocious puberty

Answer 13

Early growth spurt initially can cause tall stature, but rapid bone maturation (premature and rapid skeletal maturation and fusion of the epiphyses) can cause linear growth to cease too early and can result in short adult stature
Evaluate with bone age

Question 14

Incomplete Precocious Puberty Classification

Answer 14

Premature pubarche and premature thelarche are two common, benign, normal variant conditions that can resemble precocious puberty but are nonprogressive or very slowly progressive.

Premature thelarche refers to the isolated appearance of breast development, usually in girls younger than 3 years;

Premature adrenarche (=pubarche) refers to appearance of pubic hair without other signs of puberty in girls or boys younger than 7-8 years (usually progression is slow, so ultimate height is okay).

Question 15

central vs peripheral precocious puberty

Answer 15

Central Precocious Puberty (CPP)
= Gonadotropin Dependent Precocious Puberty (GDPP)
= True Precocious Puberty

Peripheral Precocious Puberty
= Gonadotropin Independent (GIPP)
= Pseudoprecocious Puberty

Question 16

Central Precocious Puberty- CNS abnormalities and thyroid

Answer 16

hypothalamic hamartomas
Hamartomas of the tuber cinereum are benign tumors that are the most frequent type of CNS tumor to cause precocious puberty in very young children
These secrete GnRH
Tumors (eg, astrocytomas, gliomas, pinealomas, ependymomas)
Acquired CNS injury caused by inflammation, surgery, trauma, radiation therapy, or abscess

Severe, prolonged primary hypothyroidism can cause precocious puberty

Question 17

how to distinguish central from peripheral precocious puberty

Answer 17

Central precocious puberty can be distinguished from peripheral precocious puberty by measuring LH levels.

In central precocious puberty (GDPP), basal LH levels are often elevated into the pubertal range and show a pubertal (heightened) response to GnRH stimulation.

In peripheral precocious puberty (GIPP), the LH level is low at baseline and fails to respond to GnRH stimulation.

Question 18

Precocious Puberty in Girls -Findings

Answer 18

Breast enlargement
* Usually the first and most obvious sign of early puberty is usually which may initially be unilateral.
The most reliable sign of increased estrogen production is breast enlargement.
Distinguishing glandular breast tissue from fat, which can mimic true breast tissue, is essential. Examining the patient while she is in the supine position usually minimizes the chance of misinterpreting fat as true breast enlargement.

• Pubic and axillary hair may appear before, at about the same time, or well after the appearance of breast tissue.

Question 19

enlargement of the clitoris

Answer 19

clitoris indicates significant androgen excess that must be promptly evaluated.

Question 20

Precocious Puberty in Boys -Findings

Answer 20

The earliest sign of central precocious puberty (CPP) is enlargement of the testes, which depends on increased production of follicle-stimulating hormone (FSH); testicular length is more than 2.5 cm or testicular volume (with Prader orchidometer beads) is 4 mL or more*

Question 21

most common cause of peripheral precocious puberty in girls

Answer 21

Large functioning follicular cyst of the ovaries

Question 22

McCune-Albright Syndrome

Answer 22

2 of the following 3 features:

Polyostotic fibrous dysplasia

Café-au-lait skin pigmentation

Autonomous endocrine hyperfunction

Other endocrine syndromes including hyperthyroidism, acromegaly, and Cushing syndrome

Question 23

important part of the history with precocious puberty

Answer 23

neurologic:
CNS risk factors, including infections, perinatal asphyxia, head trauma, neoplasms, or prior radiation therapy
Personality changes, increased appetite, headaches, seizures, and/or visual changes

Question 24

Androgenic effects:

Answer 24

Acne, hirsutism, increased muscle mass, and clitoromegaly in females  will focus the differential diagnosis toward androgenic causes of precocious puberty.

Question 25

important workup and labs for precocious puberty

Answer 25

Initial work up may include bone age (radiograph of the nondominant hand and wrist)

In GDPP (central), basal LH levels are often elevated into the pubertal range and show a pubertal (heightened) response to GnRH stimulation.
In GIPP, the LH level is low at baseline and fails to respond to GnRH stimulation.

Question 26

MRI of the head for precocious puberty

Answer 26

The younger the child with central precocious puberty, the greater the chance of finding CNS pathology (among children younger than 6 years).
For boys younger than 9 years, the incidence of CNS findings is much higher than in girls, and MRI should be part of the evaluation.

Question 27

Treatment: Central Precocious Puberty

Answer 27

GnRH analogs or agonists
Example: Leuprolide
Increases final adult height

Question 28

Treatment: Peripheral Precocious Puberty

Answer 28

Does not respond to GnRH agonist therapy

Tumors of the testis, adrenal gland, and ovary: surgical management

Question 29

most frequent type of CNS tumor to cause precocious puberty in very young children

Answer 29

hypothalamic hamartoma

Question 30

Hypothalamic Hamartoma - associated stuff

Answer 30

Classically a triad of precocious puberty, developmental delay, and seizures

cause intractable epilepsy with frequent gelastic and other mixed seizure types, intellectual disability, behavioral disturbance with severe temper tantrums, and precocious puberty.