Adolescence

TANNER STAGES(SMR) IV Pubic hair (both male and female)

-adult-like(dark color and dense) hair quality, extending across pubis but sparing medial thighs -ages (13-15)

TANNER STAGES(SMR) III Pubic hair (both male and female)

-hair becomes more coarse and curly, and begins to extend laterally -ages (11.5-13)

TANNER STAGES(SMR) V Pubic hair (both male and female)

-hair extends to medial surface of the thighs -ages (15+)

TANNER STAGES(SMR) II Pubic hair (both male and female)

-small amount of long, downy(fine,soft,short and straight ) hair with slight pigmentation at the base of the penis and scrotum (males) or on the labia majora (females) -ages (10-11.5)

Abnormal variants of Puberty?

1.Precocious Puberty. 2. Delayed puberty

What is Adrenarche?

Adrenarche is the onset of androgen-dependent body changes such as growth of axillary and pubic hair, body odor, and acne

What is the first sign of pubertal development in girls? a) estrogenization of vaginal mucosa b) pubarche c) menarche d) breast buds e) talking back to their mothers

Breast bud development(Thelarche)

Causes of Central Precocious Puberty (CPP)?

Central Precocious Puberty (CPP) 1.Idiopathic 2.Central Nervous System Lesions -.Hypothalamic hamartomas - Other tumors (neurofibromas, craniopharyngioma, etc.) - Malformations (septo-optic dysplasia, hydrocephalus, arachnoid cyst, etc.) - Infection (brain abscess, meningitis, etc.) - Trauma (post-surgical, irradiation, injury, etc.) 3.Peripheral Precocious Puberty states

Congenital Adrenal Hyperplasia

Congenital Adrenal Hyperplasia Ambiguous genitalia - Virilization in females - Males incomplete masculinization Advanced somatic development - Precocious puberty - Adult short stature Diagnose with clinical and laboratory findings

Constitutional Growth Delay and short stature("late bloomers").

Constitutional Growth Delay ("late bloomers"). Short stature in childhood Low/normal growth velocity Constitutional Short Stature: -Delayed growth in one parent( family history)but average final stature -Normal birth history and growth for first few months -Short stature with: =Period of severe growth deceleration lasting 1-3 yrs and may begin at less than 2 years =Bone age lags chronological age (BA<CA) =delayed onset of puberty =Absence of physical or psychological disease =Final height consistent with mid-parental height Reassurance or a short course of hormonal treatment

DELAYED PUBERTY

DELAYED PUBERTY Lack of secondary sexual characteristics by age 14 years in boys or 13 years in girls -smaller than their peers -short stature exaggerated by lack of pubertal growth spurt More common in boys than girls Lack of progression through the stages of puberty within 4.5 to 5 years after onset Psychologically and socially devastating

Differential Diagnosis of Delayed Puberty?

Differential Diagnosis of Delayed Puberty 1.Constitutional Growth Delay 2.Hypopituitarism - idiopathic or acquired --Multiple pituitary hormone deficiencies --Kallmann syndrome 3.Chronic illness, malnutrition 4.Hypothyroidism 5.Hyperprolactinemia 6.Gonadal failure --Turner syndrome --Klinefelter syndrome 7.Miscellaneous disorders

EVALUATION of Delayed Puberty?

EVALUATION History - delayed puberty in one or both parents - trauma or medical injury - eating disorders, nutritional status - anosmia - rigorous physical training Physical exam - dysmorphic features - body measurements Bone age x-ray - onset of puberty correlates better with bone age than with chronologic age Gonadotropins - random LH and FSH levels helpful only if elevated TSH, T4, prolactin CNS imaging - suspected intracranial pathology Karyotype - Turner syndrome, Klinefelter syndrome

Tanner staging Genitalia males

Genitals (male) Tanner I testicular volume less than 1.5 ml; small penis of 3 cm or less (prepubertal) (typically age nine and younger) Tanner II testicular volume between 1.6 and 6 ml; skin on scrotum thins, reddens and enlarges; penis length unchanged Ages(9-11) Tanner III testicular volume between 6 and 12 ml; scrotum enlarges further; penis begins to lengthen to about 6 cm Ages (11-12.5) Tanner IV testicular volume between 12 and 20 ml; scrotum enlarges further and darkens; penis increases in length to 10 cm Ages(12.5-14) Tanner V testicular volume greater than 20 ml; adult scrotum and penis of 15 cm in length Ages(14+)

Precocious Puberty H/P:

HISTORY Determine the growth pattern General assessment - health, nutrition, medications CNS trauma/infections, HAs, visual changes Pubertal symptoms - duration, progression Onset of puberty in other family members Exposure to exogenous hormones PHYSICAL EXAM # Tanner staging True breast development vs. fatty tissue Visual inspection of the vagina to assess estrogen stimulation - non-stimulated mucosa stimulated mucosa - glistening red - stimulated mucosa stimulated mucosa - pinkish #Other pubertal changes: acne, body odor and axillary hair Funduscopic and visual field evaluations Pigmented lesions (McCune-Albright Albright syndrome, neurofibromatosis) Neurologic assessment

Precocious Puberty: LABORATORIES AND IMAGING STUDIES

LABORATORIES AND IMAGING STUDIES Bone age x-ray - advanced with precocious puberty - children with premature thelarche or children with premature adrenarche will not have advanced bone ages Gonadotropins - random levels may not be elevated -in most cases in most cases don't do them GnRH stimulation test -children with CPP will have a brisk rise in LH and FSH - prepubertal children or those with PPP will not show a rise in LH and FSH levels Estradiol or testosterone T4, TSH, DHEA-S, 17-hydroxyprogesterone - severe primary hypothyroidism - late onset congenital adrenal hyperplasia - adrenal tumors Pelvic ultrasound - identify large ovarian cysts or neoplasms - assess development of the uterus and ovaries High resolution head MRI - girls younger than 5 years old, all boys with CPP Imaging of the adrenals/gonads

What is Menarche ?

Menarche is the onset of menstruation.

Normal Pubertal Variants ?

Normal Pubertal Variants : =No height acceleration or advancement in the bone age =Re-evaluation every 4-6 months is needed 1. Premature thelarche early breast development without other signs of early breast development without other signs of puberty occurs before age 2 years or after age 6 years 2. Premature adrenarche early development of pubic hair early development of pubic hair usually, occurs between ages 6 and 8 years

Short stature : normal variants vs Pathological variants

Normal variations of slowed growth include constitutional delay and familial or genetic factors. Pathological conditions resulting in short stature include malnutrition or malabsorption, chronic diseases, skeletal or bone diseases, and endocrinopathies.

Familial short stature?

Normal variations of slowed growth include constitutional delay and familial or genetic factors. Familial Short Stature: -Short parents -Normal birth history and gestational weight -Short stature with: =Normal linear growth velocity for age =Bone age consistent with chronological age =Normal age for onset of puberty =Absence of physical or psychological disease

What is PHV?Peak high velocity ?

PHV has been calculated by measurements of standing height taken every 3 months during the pubertal transition. PHV is the greatest increment over a period of 12 months, and the age at PHV is the midpoint of that interval. In general, PHV immediately precedes onset of menarche, with an age range of 10-15.25 years. (The mean age of PHV is 11.9 years in girls and 14.2 years in boys

PRECOCIOUS PUBERTY

PRECOCIOUS PUBERTY Onset before age 8 years in girls or age 9 years in boys Central Central - reactivation of the HPG axis - 5 times more common in girls; most cases are idiopathic -neurologic causes in > 60% of boys Peripheral

Causes of Peripheral Precocious Puberty (PPP)?

Peripheral Precocious Puberty (PPP) 1. Congenital Adrenal Hyperplasia 2. Ovarian cysts 3. Autonomous (McCune-Albright syndrome, testotoxicosis) 4. Exogenous hormones 5. Severe primary hypothyroidism 6. Tumors - adrenal, gonadal (gonadoblastomas, etc.), hepatoblastomas, etc.

What is Pubarche?

Pubarche is the appearance of sexual hair. is the appearance of sexual hair.

Pubic hair (both male and female) TANNER STAGES(SMR)

Pubic hair (both male and female) Tanner I -no pubic hair at all (prepubertal) -(typically age 10 and younger) Tanner II -small amount of long, downy(fine,soft,short and straight ) hair with slight pigmentation at the base of the penis and scrotum (males) or on the labia majora (females) -ages (10-11.5) Tanner III -hair becomes more coarse and curly, and begins to extend laterally -ages (11.5-13) Tanner IV -adult-like(dark color and dense) hair quality, extending across pubis but sparing medial thighs -ages (13-15) Tanner V -hair extends to medial surface of the thighs -ages (15+)

Precocious Puberty: THERAPY

THERAPY Central PP - GnRH agonists =bind to and stimulate the GnRH receptors =continuous stimulation stops gonadotrope secretion of LH and FSH Tumors - CNS = resection, chemotherapy and/or radiation as indicated =hypothalamic hamartoma can be treated with a GnRH agonist - Adrenal/Gonadal tumors - resection Congenital adrenal hyperplasia -physiologic glucocorticoid replacement Hypothyroidism - thyroid replacement Large ovarian cysts - resection McCune-Albright syndrome, et al - Ketoconazole, testolactone or spironolactone

SMR(sexual maturity rating)

Tanner staging!

What is the first sign of pubertal development in boys? a) penile growth b) growth spurt b) growth spurt c) pubarche d) increased muscle mass e) testicular growth

Testicular growth

What is thelarche?

Thelarche is the onset of female breast development.

The most common cause of precocious puberty in girls is: a) idiopathic b) congenital adrenal hyperplasia c) CNS tumor d) ovarian cyst e) severe acquired hypothyroidism

a) idiopathic

Tanner Stage III Breasts (female)

breast begins to become more elevated, and extends beyond the borders of the areola areola continues to widen but remains in contour with surrounding breast ages 11.5-13

Tanner Stage II Breasts (female)

breast bud forms, with small area of surrounding glandular tissue areola begins to widen ages 10-11.5

Tanner Stage V Breasts (female)

breast reaches final adult size areola returns to contour of the surrounding breast, with a projecting central papilla ages 15+

The most common cause of delayed puberty in boys is: a) CNS lesion b) hypothyroidism c) Noonan's syndrome d) constitutional growth delay e) Klinefelter syndrome

d) constitutional growth delay

What is the typical chronologic progression of pubertal changes in progression of pubertal changes in boys? a) testicular growth--> PHV-->penile growth-->adrenarche b) Penile growth -->testicular growth-->adrenarche-->PHV c) testicular growth-->adrenarche-->PHV--> penile growth d) testicular growth-->adrenarche-->penile growth-->PHV

d) testicular growth-->adrenarche-->penile growth-->PHV

What is the typical chronologic progression of pubertal changes in girls? a) thelarche--> adrenarche-->menarche--> peak height velocity (PHV) peak height velocity (PHV) b) thelarche-->menses-->PHV-->adrenarche c) adrenarche-->thelarche-->PHV-->menarche d) thelarche-->adrenarche-->PHV--> menarche e) thelarche-->PHV-->adrenarche-->menarche

d) thelarche-->adrenarche-->PHV--> menarche

Tanner Stage IV Breasts (female)

increased breast size and elevation areola and papilla form a secondary mound projecting from the contour of the surrounding breast (mound-on-mound) ages 13-15

Tanner Stage I Breasts (female)

no glandular tissue areola follows the skin contours of the chest (prepubertal) typically age 10 and younger

Tanner II Genitals (male)

testicular volume between 1.6 and 6 ml; skin on scrotum thins, reddens and enlarges; penis length unchanged Ages(9-11)

Tanner IV Genitals (male)

testicular volume between 12 and 20 ml; scrotum enlarges further and darkens; penis increases in length to 10 cm Ages(12.5-14)

Tanner III Genitals (male)

testicular volume between 6 and 12 ml; scrotum enlarges further; penis begins to lengthen to about 6 cm Ages (11-12.5)

Tanner V Genitals (male)

testicular volume greater than 20 ml; adult scrotum and penis of 15 cm in length Ages(14+)