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69 Cards in this Set
- Front
- Back
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Define Adrenarche
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Onset of andrenal androgen steroidogeneis
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Define Pubarche
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First appearance of pubic hair
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Define Thelarche
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Onset of breast development
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Define Menarche
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Onset of the menstrual cycle
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Define Puberty
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biologic changes that lead to reproduction
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When does adrenarche occur?
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Prior to Gonadarche (usually)
**not a causal relationship |
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What occurs during adrenarche and when does it occur?
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Increase serum DHEA and DEHAS (no physical traits)
in 6-8yo, BEFORE signs of puberty |
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What are the 3 major changes of puberty?
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Full maturation of gonads, external genitalia, and secondary sex characteristics
Change in lean body mass/fat distribution Rapid skeletal growth |
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At what age does puberty begin in girls?
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Between 8 and 12 years
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At what age does puberty begin in boys?
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9 and 14 years
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Characterize Pre-puberty
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"immature brain"
Dormant hypotahlmic-pituitary-gonadal axis Undetectable levels of sex hormones |
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What is the primary event at the start of puberty?
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Pulsatile secretion of GnRH from hypothalamus
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What does GnRH do?
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drives secretion of LH and FSH from anterior pituitary
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What does stimulation with LH and FSH do?
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secretion of gonadal steroid hormones, testosterone, and estardiol
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What is "true puberty?"
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When LH, FSH and gonadal sex steroids increase
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Why is it very important to get a good history?
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To determine when their first degree relatives started puberty (often follows a familial pattern)
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What can alter the puberty process?
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CNS, nutrition status and exogenous/envir influences
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What is the first exam finding of puberty for boys?
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Testicular enlargement
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What is the first exam finding of puberty in girls?
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Breast bud development
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With Tanner stages, what are girls rated for?
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Pubic hair growth and breast development
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With Tanner stages, what are boys rated for?
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Pubic hair and genital development
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With girls, tanner stage 2 breast development develops before....
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stage 2 pubic hair development
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When does the menstrual cycle start in females?
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Around tanner stage 4
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When do most females stop tanner stage growth?
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Stage 5, few go onto stage 6
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In males, what tanner stage is the first sign of puberty?
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Tanner Stage 2: scrotal enlargement
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In males, at which stage does the growth spurt occur?
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Tanner stage 4
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In males, at what stage does chest hair develop?
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Tanner stage 5
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Name the 4 abnormalities of puberty.
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Pubertal Delay
Precocious Puberty (early puberty) Premature Thelarche Premature Adrenarche |
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What is the criteria to diagnose pubertal delay?
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NO sexual maturation in boys by age 14, girls age 13
Also, if delayed menarche has NOT occurred in girls by age 16 (or 5 years after pubertal onset) |
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Is pubertal delay pathological?
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NO, not usually
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What are some causes of pubertal delay?
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Constitutional delay (late bloomers)
Chronic disease Malnutrition/overexercise Aquired (chemo, radiation, surgery) |
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A type of pubertal delay, define HYPERgonadotropic HYPOgonadism.
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Elevated gonadotropins
Due to gonadal failure |
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A type of pubertal delay, define HYPOgonadal HYPOgonadism.
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Inadequate function of gonads, low levels of GnHR
Due to issue with gonadal secretion from hypothalamus/pituitary Low FSH, LH, or both |
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Examples of HYPERgonadotropic HYPOgonadism
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Turner Syndrome
Klinefelter Syndrome Bilateral Gonadal Failure |
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What is Turner's Syndrome?
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XO: normal intelligence/ability
primary gonadal failure with early adrenarche with delayed pubarche |
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How do you diagnose Turner's Syndrome/what labs would you see?
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Elevated DEHA sulfate
Banded Karyotype |
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What complications are associated with Turner Syndrome?
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Gonadal Dysgenesis
Coarctation of aorta Hore-shoe kidneys Duplication of renal collecting system Short Stature |
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What is the most common genetic cause of infertility and hypogonadism in males?
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Klinefelter Syndrome (XXY)
Anuploidy of X Chr.... don't notice until adolescence |
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Characteristics of Klinefelter Syndrome? (6)
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Tall with long limbs
Gynecomastia Learning Disabilities Undervirulization, female pubic hair pattern Small testes and penis Hypospermia |
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What would labs of a Klinefelter patient show?
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HIGH serum FSH and LH
Low testosterone |
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Give examples of HYPOgonadotropic HYPOgonadism.
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Deficit in GNRH
Kallman Syndrom X-linked CAH Malnutrition Chronic Illness Repetitive Strenuous Exercise |
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What causes deficiencies in GnRH?
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Defect in hypothalamus
Defect in anterior pituitary Cranial Radiation |
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Describe Kallman Syndrome
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Hypothalamus doe NOT contain GnRH cells
Lack secondary sex characteristics X-linked (male) and Autosomal Dominant form (female) |
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What is the most common associated symptom of Kallman Syndrome?
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Anosmia or Hypo-osmia (lack of smell)
Rarely show bimanual synkinesis |
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Define Precociouse Puberty in a girl
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Breast/pubic hair development <7yo
OR Menarche<9yo **more common in girls** |
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Define Precocious Puberty in males
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Testicular changes, penile enlargement or pubic/axillary hair <9yo
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What are some theories as to why puberty begins earlier?
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Obesity/Overnutrition
Early exposure to exogenous hormones (insecticides/hormone and steroid supplements to farm animals) |
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Who is most likely to develop precocious puberty?
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African Americans (or a person whose first degree relatives also developed early)
Puberty can begin as early as 6yo |
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Name the four divisions of Precocious Puberty
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Central Precocious Puberty (gonadotropin dependent, "true"
Peripheral Precocious Puberty (Gonadotropin independent, "pseudopuberty") Incomplete Precocious Puberty: -Premature Thearche -Premature Adrenarche |
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What is the underlying mechanism of Central Precocious Puberty?
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early onset of gonadotropin mediated puberty (early activation of the hypothalamus)
more common in girls than boys...benign in girls, pathological in boys |
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What are cause of central precocious puberty?
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Idiopathic
Tumor (astrocytoma, glioblastoma, germinoma) Congenital anomaly (hydrocephalus) Infection (encephalitis/meningitis) Radiation/Trauma/Ischemia |
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How do you diagnose Central Precocious Puberty?
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Full Hx, look for CNS cause
LH increase (take sample during sleep) GnRH stimulation test MRI of head for ALL BOYS! |
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How does a GnRH stimulation test Work?
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Detects activation of hypothalamic-pituitary axis
Inject synthetic GnRH into patient and measure LH |
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What is a positive GnRH stimulation test?
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INCREASE in LH >5IU/L
A pituitary ***already exposed*** to endogenous GnRH would respond to the injection with a RISE in LH |
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If a pituitary gland had NO prior exposure to endogenous GnRH, what would happen?
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only produce a small amount of LH
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If you have a positive GnRH stimulation test, what do you follow up with?
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Cranial MRI!
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Describe Peripheral Precocious Puberty
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INDEPENDENT of hypothalamic-pituitary-gonadal axis
Caused by peripheral production of male/female sex hormones |
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Causes of Peripheral Precocious Puberty in boys
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Adrenal Tumor
Leydig Tumor cell Tumor Nonclassic CAH B-hcg-producing tumors |
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Causes of Peripheral Precocious Puberty in girls
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Adrenal tumor
Virulizing ovarian tumors Feminizing ovarian tumors nonclassic CAH McCune-Albright syndrome |
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How do you evaluate for peripheral Precocious puberty in boys?
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GnRH stimulation test
Serum FSH/LH Testosterone B-hcg levels |
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How do you evaluate peripheral precocious puberty in girls?
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Serum FSH, LH, and estradiol
GnRH stimulation test |
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Describe McCune-Albright Syndrome
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Café au lait spots
Polyostotic dysplasia Precocious puberty Hyperthyroidism Initially, suppress LH w/ GnRH test and growth/puberty all normalizes |
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Polyostoic fibrous dysplasia includes... (3)
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Ineffective growth of multiple bones
Patchy areas of osteoporosis/bone thickening Repeated fractures |
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Describe Premature Thelarche
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Occurs within first 2 years of life
Visible/palpable breast tissue Normal growth pattern, no pubic hair development until appropriate |
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What causes premature Thelarche?
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TRANSIENT activation of hypothalamic-pituitary-gonadal axis
Temporary ovarian follicular stimulation/release low estrogen levels |
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How do you workup or treat premature thelarche?
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NONE.... unless growth spurt or pubic hair develops
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Describe Premature Adrenarche
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Early onset of pubic OR axillary hair
No development of breasts/enlargement of testes Normal growth without advanced bone age Usually girls, after 5yo |
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How do you evaluate and treat premature adrenarche?
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Not neccessary
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When do you become concerned and start looking for an issue?
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1. Testicular/penile enlargement
2. Menses begin early 3. combo of growth and pubertal development |
