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Precocious Puberty
Kelvin Philip Group 99
I.M Sechenov, Moscow.
Normal Puberty
The stage of physical maturation in which an
individual becomes physiologically capable of
sexual reproduct...
The
Reprodcutive
System
Functional by the end of the first trimester of fetal life
Become briefly inactive around the time...
Onset of normal
puberty
Girls : 10- 14 years old
Boys : 12-16 years old
With this disease puberty could begin as early as
...
Phases of puberty
Adrenarche
- Stage of maturation of the cortex of adrenal
glands
- Increase adrenal androgen secretion
G...
Thelarche
-First stage of secondary breast develpment
-Usually noticed as firm, tender lump directly in the
center under t...
Tanner Stages
Develop as a way to classify the time, course, and
progress of changes that occur during puberty.
Based upon...
Female Breasts
Tanner Stages Breast
1 Preadolescent. Elevation of the
papilla only
2 -A small mount is formed by the
eleva...
Male genitalia
Tanner
Stages
Genitalia
1 -The penis , testes and
scrotum are of childhood size
2 -Enlargement of scrotum a...
Pubic Hair
Tanner Stages Female Male
2 Sparse growth of long, slightly pigmented,
downy hair, straight or only slightly
cu...
Growth Spurt
Growth spurt/Peak height velocity
 Female:
 Happens in earlier stages of puberty
 Average age: 12.1
 Male...
Hormonal changes
Increase in:
 Testosterone
 Estrogen
 Prolactin
 Growth Hormone
 Insulin
Precocious Puberty
onset of secondary sexual characters before the age of 8 years in
girls and 9 years in boys.
Two types ...
Rare Causes of Central Precocious Puberty
Idiopathic - 90% of girls
Tumor in brain or spinal cord - Hypothalamic hamartoma...
peripheral- gonadotropin independent. some of the secondary sex
characters appear, but no activation of the normal hypotha...
Other causes
Little evidence, but these may be contributing factors to precocious puberty:
-Environmental Factors
1) Incre...
Hypothalamic
Hamartoma
In hypothalamic hamartoma remain static in size or grow slowly, -
no signs other than precocious pu...
Mental development is usually compatible with chronological age.
Emotional behavior and mood swings are common, but seriou...
How it affects the child
With this disease the kids usually do not achieve
their full height, they may be taller when youn...
Symptoms for girls
Breast development can happen as early as between
6 months and 3 years.
Pubic or underarm hair developm...
Presentation
GIRLS- The average age at onset girls is about 3 yr, vaginal
bleeding as early as 4 mo of age and secondary s...
When to consult
• Boys: Girls:
Age: < 9.5 years old
• Physical Features:
- secondary sex characteristics
Tanner Stage 2 ...
Lab Diagnosis
With sensitive assays, serum LH concentrations are undetectable
in prepubertal children but become detectabl...
A CT Scan of the brain or the abdomen can be used
Also an MRI of the brain or the abdomen can be used
to diagnose the prob...
Treatment
The goal of the treatment is to stop or reverse
sexual development and stop the rapid growth and
bone maturation...
• There is a currently approved hormone treatment is with drugs called LHRH
analogs: synthetic hormones that block the bod...
Long term GnRH agonists, (leuprolide) (Lupron Depot Ped), in
a dose of 0.25-0.3 mg/kg (minimum, 7.5 mg) i.m. once every 4
...
After treatment
In girls, breast size may decrease or there will be
no further development.
In boys, the penis and testicl...
Incomplete Partial
Variants
Isolated manifestations of precocity without development of other
signs of puberty.
Premature ...
However, In some girls, breast development is associated with
definite evidence of systemic estrogen effects, such as grow...
Although it has been considered a benign condition, longitudinal
observations suggest that approximately 50% of girls with...
The majority of idiopathic premature menarche have only 1-3
episodes of bleeding; puberty occurs at the usual time, and
me...
Early breast development
Increased height velocity and
advanced bone age
yes
Associated sec.sexual characters , axillary a...
GnRH stimulation test
LH response
Suppressed
GnRH independent PPGnRH dependent CPP
MRI CNS imaging
Abnormal
Idiopathic CPP...
Precocious puberty
Precocious puberty
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Precocious Puberty and normal puberty

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Precocious puberty

  1. 1. Precocious Puberty Kelvin Philip Group 99 I.M Sechenov, Moscow.
  2. 2. Normal Puberty The stage of physical maturation in which an individual becomes physiologically capable of sexual reproduction Triggered by increased secretion of LH. Results from changes in the hypothalamus that allow increased secretion of GnRH to stimulate the secretion of LH
  3. 3. The Reprodcutive System Functional by the end of the first trimester of fetal life Become briefly inactive around the time of birth Resume hormonal activity until several months after birth The brain begin to suppress the activity of the arcuate nucleus. Gonadotropin and sex steroid levels fall to low levels for approximately another 8 to 10 years of childhood. Normal puberty happens when there is de-inhibition of the pulse generator in the arcuate nucleus.
  4. 4. Onset of normal puberty Girls : 10- 14 years old Boys : 12-16 years old With this disease puberty could begin as early as age 6 or 7 in girl For boys puberty could begin as early as before age 9.
  5. 5. Phases of puberty Adrenarche - Stage of maturation of the cortex of adrenal glands - Increase adrenal androgen secretion Gonadarche - Earliest gonadal changes - Ovaries and testes begin to grow - Increase production of sex hormones
  6. 6. Thelarche -First stage of secondary breast develpment -Usually noticed as firm, tender lump directly in the center under the nipple(papilla and areola) -Referred to as breast bud (Tanner stage 2) Pubarche -First appearance of the pubic hair -Results from increased levels of androgens from adrenal glands or testes. Menarche -First menstrual bleeding -Possibility of fertility
  7. 7. Tanner Stages Develop as a way to classify the time, course, and progress of changes that occur during puberty. Based upon attainment of secondary sex characteristics, which include genital development in males, breast development in females, and pubic hair development in both genders.
  8. 8. Female Breasts Tanner Stages Breast 1 Preadolescent. Elevation of the papilla only 2 -A small mount is formed by the elevation of the breast and papilla -Areolar diameter enlarges 3 Further enlargement of breast and areola. 4 Projection of the areola and papilla to form a secondary mound above the level of the breast 5 -Mature breast -Areola recessed to the general contour of the breast.
  9. 9. Male genitalia Tanner Stages Genitalia 1 -The penis , testes and scrotum are of childhood size 2 -Enlargement of scrotum and testes - Scrotal skin reddens 3 -Further growth of the testes and scrotum. -Enlargement of penis, mainly in length 4 -Further growth of the testes and scrotum. -Enlargement of penis, mainly in breadth 5 Adult genitalia
  10. 10. Pubic Hair Tanner Stages Female Male 2 Sparse growth of long, slightly pigmented, downy hair, straight or only slightly curled, primarily along the labia. Sparse growth of long, slightly pigmented, downy hair, straight or only slightly curled, primarily at the base of penis. 3 Hair is darker, coarser, and more curled. Hair is darker, coarser, and more curled. 4 Adult type hair, does not extend onto the thighs Adult type hair, does not extend onto the thighs 5 Hair is adult in quantity and type, with extension to the thighs. Hair is adult in quantity and type, with extension to the thighs.
  11. 11. Growth Spurt Growth spurt/Peak height velocity  Female:  Happens in earlier stages of puberty  Average age: 12.1  Male:  Happens in later stages of puberty  Average age: 14.1  Boys have greater velocity at the maximum pubertal growth rate
  12. 12. Hormonal changes Increase in:  Testosterone  Estrogen  Prolactin  Growth Hormone  Insulin
  13. 13. Precocious Puberty onset of secondary sexual characters before the age of 8 years in girls and 9 years in boys. Two types : -central-gonadotropin dependent or true. Stems from hypothalamic-pituitary-gonadal activation with ensuing sex hormone secretion and progressive sexual maturation. Most common. Puberty starts too soon but pattern and timing of processes are normal.
  14. 14. Rare Causes of Central Precocious Puberty Idiopathic - 90% of girls Tumor in brain or spinal cord - Hypothalamic hamartoma Brain defect present at birth Injury to brain or spinal cord, severe head trauma, hydrocephalus, myelomeningocele McCune-Albright syndrome Genetic disease, affects bones and skin color, causes hormonal problems Congenital adrenal hyperplasia Group of inherited disorders, causes abnormal hormone production by adrenal glands Hypothyroidism Condition where thyroid doesn’t produce enough hormones
  15. 15. peripheral- gonadotropin independent. some of the secondary sex characters appear, but no activation of the normal hypothalamic-pituitary- gonadal interplay. May be isosexual or heterosexual (contrasexual) Less common Occurs without the hormone in your brain that usually causes puberty to start (Gn-RH) Caused by the release of estrogen or testosterone into the body because of problems with the ovaries, testicles, adrenal glands or pituitary gland •
  16. 16. Other causes Little evidence, but these may be contributing factors to precocious puberty: -Environmental Factors 1) Increase in hormones found in food 2) Exposure to household toxins (BPA) Obesity ~106 lbs is the weight when puberty starts -Leptin, a hormone involved in puberty, is secreted in fatty tissue (more fatty tissue=more leptin=earlier puberty)
  17. 17. Hypothalamic Hamartoma In hypothalamic hamartoma remain static in size or grow slowly, - no signs other than precocious puberty. For symptomatic , manifestations may be present for 1-2 years before the tumor can be detected radiologically Include diabetes insipidus, adipsia, hyperthermia, unnatural crying or laughing (seizures), obesity, and cachexia. Visual signs (proptosis, decreased visual acuity, visual field defects) may be the first manifestation of an optic glioma.
  18. 18. Mental development is usually compatible with chronological age. Emotional behavior and mood swings are common, but serious psychological problems are rare Natural course- rapid- most common pattern. characterized by rapid physical and osseous maturation, leading to a loss of height potential. Slow-this pattern characterized by parallel advancement of osseous maturation and linear growth, with preserved height potential. spontaneously regressive or unsustained central precocious puberty – marks the need for longitudinal observation
  19. 19. How it affects the child With this disease the kids usually do not achieve their full height, they may be taller when younger compared to their peers but when older they are short Girls can become more emotional, moody, and irritable at a younger age. Boys can become more aggressive and develop a sex drive that is very inappropriate for their young age.
  20. 20. Symptoms for girls Breast development can happen as early as between 6 months and 3 years. Pubic or underarm hair development at a very young age A growth “spurt” before all of their peers, they may seem taller at a younger age Menstruation begins earlier Acne at a young age Also mature body odor at a young age
  21. 21. Presentation GIRLS- The average age at onset girls is about 3 yr, vaginal bleeding as early as 4 mo of age and secondary sexual charecters at 6 mo of age. Estradiol levels vary from normal to markedly elevated (>900 pg/mL), are often cyclic, and can correlate with the size of the cysts.
  22. 22. When to consult • Boys: Girls: Age: < 9.5 years old • Physical Features: - secondary sex characteristics Tanner Stage 2 - 5 Age: < 8 years old • Physical Features: - breast growth Tanner Stage 3-5 If at Tanner Stage 2, there must be other features (e.g. a recent growth spurt).
  23. 23. Lab Diagnosis With sensitive assays, serum LH concentrations are undetectable in prepubertal children but become detectable in 50-75% of girls and a higher percentage of boys with central sexual precocity. Measurement during sleep increases diagnostic power and reveals the pulsatile LH secretion. GnRH stimulation test. Predominant LH response over FSH after iv administration of GnRH or agonist like Leuprolide. However in girls, LH:FSH ratio can remain low until mid-puberty. In such girls with “low” LH response, the central nature of sexual precocity can be proved by detecting pubertal levels of estradiol (>50 pg/mL), 20-24 hr after stimulation with leuprolide.
  24. 24. A CT Scan of the brain or the abdomen can be used Also an MRI of the brain or the abdomen can be used to diagnose the problem. Pelvic ultrasound reveals progressive enlargement of ovaries followed by uterus to pubertal size. Osseous maturation advances. An MRI scan usually shows normal enlargement of the pituitary gland, during puberty; it may also reveal CNS pathology.
  25. 25. Treatment The goal of the treatment is to stop or reverse sexual development and stop the rapid growth and bone maturation. hormone (GnRH) analogues. Usually results in a decrease in growth Combining growth hormone (GH) to GnRH treatment results in a better outcome final height in girls
  26. 26. • There is a currently approved hormone treatment is with drugs called LHRH analogs: synthetic hormones that block the body’s production of the sex hormones that are causing the early puberty. • LHRH Analogues: Drugs that act like luteinizing hormone releasing hormone (LHRH) are known as LHRH analogues. These drugs turn off the signal for testosterone production by the testicles. By turning off the signal, hormone levels are reduced and cancer cells are not exposed to male hormones. LHRH analogues are given as a small injection under the skin of the abdomen every month or every three months.
  27. 27. Long term GnRH agonists, (leuprolide) (Lupron Depot Ped), in a dose of 0.25-0.3 mg/kg (minimum, 7.5 mg) i.m. once every 4 wk. - Other preparations : (D-Trp6-GnRH [Decapeptyl], goserelin acetate [Zoladex]) are approved for treatment of precocious puberty in other countries. Alternatively, histrelin (Supprelin LA), a subcutaneous 50 mg implant with effects lasting 12 mo, is approved by the FDA . Intranasal and subcutaneous injection formulae are also available. IUGR at greater risk of short stature as adults and require more-aggressive treatment of precocious puberty, possibly in conjunction with human growth hormone (hGH) therapy.
  28. 28. After treatment In girls, breast size may decrease or there will be no further development. In boys, the penis and testicles may shrink and go back to the expected size for their age. In both, the growth height comes to a halt and the behavior goes back to normal according to their age.
  29. 29. Incomplete Partial Variants Isolated manifestations of precocity without development of other signs of puberty. Premature thelarche- isolated breast development that most often appears < 2 yr of life. Sporadic and asymmetric. ; activating mutations of the GNAS1 gene encoding the α-subunit of the GS protein have been described in some patients without other signs of McCune-Albright syndrome. Menarche occurs at the expected age, and reproduction is normal. Basal serum levels of FSH and the FSH response to GnRH stimulation >normal girls Plasma LH and estradiol are consistently < limits of detection. Ultrasound examination of ovaries show a few small (<9 mm) cysts.
  30. 30. However, In some girls, breast development is associated with definite evidence of systemic estrogen effects, such as growth acceleration or bone age advancement. Pelvic sonography shows enlarged ovaries or uterus. This condition, referred to as exaggerated or atypical thelarche, differs from central precocious puberty because it spontaneously regresses. PREMATURE PUBARCHE(ADRENARCHE)-appearance of sexual hair before the age of 8 yr in girls or 9 yr in boys without other evidence of maturation. Girls >boys. Associated with reduced 3β-hydroxysteroid dehydrogenase activity, and an increase in C-17,20-lyase activity. Leads to increased DHEA, androstenedione and 17HOP
  31. 31. Although it has been considered a benign condition, longitudinal observations suggest that approximately 50% of girls with premature adrenarche are at high risk for -hyperandrogenism and -polycystic ovary syndrome, alone or more often in combination with other components of the metabolic syndrome (insulin resistance possibly progressing to type 2 diabetes mellitus, dyslipidemia, hypertension, increased abdominal fat) as adults. Premature Menarche-is a diagnosis of exclusion. In girls with isolated vaginal bleeding in the absence of other secondary sexual characters, more common causes such as vulvovaginitis, a foreign body, or sexual abuse, and uncommon causes such as urethral prolapse and sarcoma botryoides(embryonal rhabdomyosarcoma) must be carefully excluded.
  32. 32. The majority of idiopathic premature menarche have only 1-3 episodes of bleeding; puberty occurs at the usual time, and menstrual cycles are normal. Plasma levels of gonadotropins are normal, but estradiol levels may be elevated, probably owing to episodic ovarian estrogen secretion. Occasional patients are found to have ovarian follicular cysts on ultrasound.
  33. 33. Early breast development Increased height velocity and advanced bone age yes Associated sec.sexual characters , axillary and pubic hair yes no age <2years Infantile mamoplasia >2 year Pelvic ultrasound evaluation of gonads and uterus Pubertal GnRH stimulation test Prepubertal Premature thelarche
  34. 34. GnRH stimulation test LH response Suppressed GnRH independent PPGnRH dependent CPP MRI CNS imaging Abnormal Idiopathic CPP no yes Organic CPP TSH, T4, hCG,ovarian, adrenal imaging
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Precocious Puberty and normal puberty

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