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50 Cards in this Set

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What is 1st hormonal sign of puberty?
Increase in LH pulsatility at night
What is the 2nd hormonal sign of puberty?
What does this lead to?
LH and FSH pulses occurring thruout the day
This leads to increasing estrogen levels from the growing ovarian follicle → positive feedback from E2 finally initiates LH surge and ovulation → elevated progesterone levels + luteal phase
Why do many teens have irregular periods?
Because ovulation is often inconsistent 1-2 yrs after menarche
If teenagers continue to have irregular periods >2yrs after menarche, what might this indicate?
A reproductive disorder
What non-androgen hormone does estrogen stimulate?
Growth hormone → IGF-I → increased somatic growth
What non-androgen hormone does estrogen stimulate?
Growth hormone → IGF-I → increased somatic growth
What is adrenarche?
What is produced?
At what age does it occur?
1) Increased androgen production by the adrenal glands. It is NOT essential for normal ovarian function, and gonadal function is NOT essential for proper adrenal function.
2)The adrenals produce increased quantities of DHEA, DHEAS, and androstenedione
3) 6-8 yrs old
What effect does estrogen have on bone?
What is the difference between estrogen and growth hormone on bones?
1) Increases bone growth (esp axial skeleton); promotes epiphyseal plate fusion (precocious puberty = grow early, but are short)
2) Growth hormone has more effect on long bones
What is the body type of a hypogondal pt?
Of a GH-deficient pt?
1) Short trunk + long arms/legs
2) Long trunk + short arms/legs
What factors determine when puberty begins?
Family history
Race (African American = earlier)
Altitude (lower = earlier)
Distance to equator (closer = earlier)
Urban vs rural (urban = earlier)
Obese or blind = earlier
What are factors that can lead to delayed puberty?
Diabetes
Extreme obesity
Poor nutrition
Excessive stress
Over-exercise
What is Frisch's critical body fat theory?
Pt needs 17-22& body fat to initiate puberty (good for anorexic or obese teens)
But, this does not explain all teens
At what age is the HPG axis functional?
20 WEEKS of life (prenatal)
When do LH and FSH levels first peak?
When do they first flare?
20wks gestational age
First 1-2 years of life
What is the accepted age range for onset of puberty?
8-14 yrs
African American girls may start before 8 (this is normal)
What two ages are markers for delayed puberty needing workup?
No secondary sexual characteristics by 14
Secondary sexual characteristics but no menarche by 16
Should we workup isolated breast or pubic hair development in absence of other puberty signs?
Usually warrants observation to exclude precocious puberty
What is premature adrenarche thought to be an early sign of?
PCOS later in life
Which gender more commonly gets precocious puberty? (ratio?)
Delayed puberty?
Girls (5:1)
Boys
How long (average) does puberty take in girls vs boys?
Girls: 3yrs
Boys: 5yrs
Isosexual vs heterosexual precocious puberty?
Iso: Premature puberty compatible with individual's genetic sex
Iso is way more common type!!
Hetero: premature puberty with changes OPPOSITE of individual's genetic sex
In girls this means xs androgen production from adrenals or ovaries
GnRH-dependent vs GnRH-independent precocious puberty?
Please give 3 alternate terms for each name
Dependent: Premature re-activation of HPG axis → the hypothalamic pulse generator starts up too early
AKA: True, Complete, Central
Indie: Early sex steroid production NOT from HPG axis
AKA: Pseudo-puberty, Incomplete, Partial
What is “mixed picture” GnRH precocious puberty?
Peripheral hormone production leading to HPG activation
What percent of precocious puberty is idiopathic in girls?
In boys?
Girls: 75%
Boys: 40%
More MRI use might help find subtle changes that reduce these figures!!
What causes GnRH dependent precocious puberty? (2)
How to treat each?
Idiopathic; tx w/ GnRH agonists (leuprilide acetate)
Tumors (hamartomas = MC); tx w/ surgery, rads, GnRH agonists
What causes GnRH independent precocious puberty? (7)
How to treat each?
1) Functional ovarian cyst; surgery(?)
2) Granulosa cell tumor; surgery
3) Adrenal steroid producing tumor; surgery
4) McCune-Albright (aka polyostotic fibrous dysplasia)
5) Genetic mutation in gonadotropin-R, causing autonomous ovarian estrogen production (constitutional activation); tx = testolactone (inhibits aromatase)
6) Hypothyroidism; hormone replacement
7) Exogenous drug ingestion; stop taking drug!
In female, what is order of puberty? (4)
Thelarche (breast bud)
Pubarche (pubes)
Max growth velocity
Menarche
What is only form of precocious puberty in which bone growth is delayed rather than advanced?
Primary hypothyroidism
What should you ask in H&P of pt w/ precocious puberty?
5 H, 6P
H: Growth & puberty milestones
Family history (reproductive anomalies?)
Any exogenous drug ingestion?
Symptoms of thyroid disease?
Neuro symptoms or hx of CNS insults?
PEx: Ht, wt, %tle for age
Tanner staging
Neuro + thyroid exam
Skin (cafe au lait spots?)
Abdominal exam
Pelvic & rectal exam (masses?)
What labs and rads for precocious puberty w/u?
Serum E2, LH, FSH, TSH, hCG
Hetero: Add DHEAS, 17-OH progesterone, testosterone
Left wrist bone age
Pelvic US or CT (ovarian or adrenal masses?)
Head MRI (tumors?)
Skull XR (cystic lesions of McCune-Albright?)
What is McCune-Albright syndrome characterized by?
Multiple cystic bone lesions
cafe-au-lait spots
precocious puberty
What is w/u & ddx of pt w/ delayed puberty and primary amenorrhea?
Pt has NO breasts, YES uterus, High FSH
High FSH? Hypergonadotropic hypogonadism
Get karyotype
45 XY = Swyer's
45X = Turner's syndrome or mosaicism
46XX = Many things (see other card)
No breasts, yes uterus, high FSH, 46XX pt differential? (6)
1) Pure gonadal dysgenesis
2) 17-OHase deficiency
3) X chromosome deletion
4) FSH-R defect
5) Ovarian destruction: rads/chemo, viral, autoimmune (schmidt's), galactosemia
6) Myotonic dystrophy or Kennedy's disease.
What is w/u and ddx of pt w/ delayed puberty and primary amenorrhea?
Pt has NO breasts, YES uterus, Low/Normal FSH
Low FSH? Hypogonadotropic hypogonadism
Image CNS
Tumor: Pituitary or hypothalamic (craniopharyngioma)
No tumor: Many things (see other card)
No breasts, yes uterus, low FSH, CNS image shows no tumor differential? (7)
1) Kallman's
2) Gonadotropin deficiency
3) Constitutional: anorexia, xs exercise, systemic disease
4) GnRH-R defect
5) Histiocytosis X
6) Pituitary insufficiency
7) Hypothyroidism
What is w/u & ddx of pt w/ delayed puberty and primary amenorrhea?
Pt has NO uterus, YES breasts
Take karyotype + Testosterone levels
XY: Androgen insensitivity
XX: Uterovaginal agenesis
What are MC causes of primary amenorrhea AND delayed puberty?
1) Gonadal dysgenesis (usually Turner's)
2) Uterovaginal agenesis
3) Androgen insensitivtity
4) Anorexia nervosa or extreme exercise
What is the incidence of primary amenorrhea?
<1%
What is secondary amenorrhea?
No menses >6m
No menses for a total of 3 previous cycle intervals
What is the incidence of secondary amenorrhea?
~0.7%
What questions to ask in H&P of amenorrhea?
History of coitus?
Nature and sequence of other pubertal events (primary)
Hx of severe systemic illness or stress?
Changes in sleep, thirst, appetite, smell?
Headaches? Vomiting? Visual field defects? Fatigue? Galactorrhea?
Pt on meds or chemo/rads?
Ht? Wt? Pulse? BP?
Evidence of low estrogen or hirsuitism?
See normal reproductive tract on PEx?
What initial blood tests should be performed for pt w/ amenorrhea?
Thyroid fxn tests
PRL level
Pt w/ galactorrhea and amenorrhea should have which test?
Head MRI or thin-section coronal CT w/ contrast
What is a progesterone challenge test?
200mg IM progesterone in oil OR 300mg PO micronized progesterone OR 10mg PO medroxyprogesterone acetate for 5 d
Positive test = bleeding or spotting w/in 2-7 days of test.
What does a positive progesterone challenge test indicate? (4)
Serum estrogen level > 40pg/mL
The anterior pituitary is making LH and FSH
Endometrium and outflow tract are fxning
If Thyroid fxn tests and PRL are normal, pt is anovulatory
What does a negative progesterone challenge test indicate? (7)
1) Outflow tract defect, e.g.
Asherman's
fibrosis 2/2 severe endometriosis
cervical stenosis
uterine agenesis
imperforate hymen
transverse vaginal septum
2) Hypoestrogen state
Pt has amenorrhea, normal TH/PRL, NO withdrawal bleeding on progesterone challenge – what tests and what do they indicate?
LH & FSH
Low FSH = hypothalamic causes of amenorrhea
High FSH = ovarian failure
What are MC causes of primary amenorrhea?
1) Gonadal failure: Turner's; gonadal agenesis (1/3 of all cases)
2) Mullerian anomalies: Uterovaginal agenesis (1/5 of all cases)
What condition occurs in Mayer-Rokitansky-Kuster-Hauser syndrome?
Uterovaginal agenesis
Primary amenorrhea WITH breasts and WITH uterus? (5)
Hyperandrogenic amenorrhea (PCOS)
Hypothalamic dysfunction
Hypothyroid
Hyperprolactinemia
Obstruction (imperforate hymen os transverse vaginal septum)