HPO

Question 1

Def’n of primary amenorhea

Answer 1

no menses by age 14 w/o sex characteristics

no menses by age 16 w/ sex characteristics

Question 2

Secondary amenorrhea

Answer 2

No menses in 6/12

Question 3

Most common causes of primary amenorrhea

Answer 3

1) Gonadal dysgenesis (50% 45X, 25% mosaic, 25% 46XX)
2) Mullerian
3) AIS (male pseudohermaphrodite)

Question 4

Most common causes of hypo hypo

Answer 4

CNS tumors, RT, head injury
Kallman’s syndrome
Anorexia, chronic disease
Endocrine - hypoT, hyperPRL, Cushings, Addisons

Question 5

DDx of +breast/-uterus

Answer 5

MRKH, AIS

Question 6

DDx of +breast/+uterus

Answer 6

Hypoth-stress, drugs, anorexia, illness
Pit-PRL,Thy,tumor,idiopathic
Adrenal-CAH,cushings
Ovarian-POF,gonadal dysgenesis, PCOS
Uterine-senechia,pregn,septums,agenesis

Question 7

DDx of -breast/-uterus

Answer 7

17,20-desmolase deficiency
Agonadism
17 hydroxylase deficiency

Question 8

DDx of -breast/+uterus

Answer 8

Gonadal failure-Turners, Pure Gonadal Dysgenesis (46XX-perrault,46XY-Swyers)
POF
CNS
Hypopit
Genetic-PraderWilli

Question 9

Most common causes of Hyper Hypo

Answer 9

Normal karyotype: RT, chemo, POF, Pure Gonadal Dysgenesis (46XX-perrault,46XY-Swyers)
AbN karyotype: Aromatase def, 17a hydroxylase def, 17,20 desmolase def, congenital lipoid AH

Question 10

Indication for bone age?

Answer 10

No breast development

Question 11

Phases of tx for delayed puberty

Answer 11

After GH (if necessary)
1-breast development (low dose E)
2-establish N menses (E/P)
3-longterm maintenance of N estrogen (HRT or OCP)

Question 12

AIS

Answer 12

46 XY, maternal X-linked recessive, absent/sparse hair, N or slightly elevated T, short vagina, breasts+

Question 13

MRKH

Answer 13

46 XX, multifactorial inheritance, normal hair, normal female T, associated with scoliosis/renal, breasts+

Question 14

Antiandrogen meds for hirsutism

Answer 14

Spironolactone (competes for receptor in skin)
CPA (inhibits gonadotropin release)
Flutamide (nonsteroid antiandrogen)
Finasteride (5a reductase blocker)
Topical Rx - Vaniqua/Eflornithine

Question 15

Diagnosis of POI

Answer 15

2 x FSH in menopausal range 1 month apart

Question 16

Incidence of POI

Answer 16

~1% of women

1:1000 <40

Question 17

DDx of POI

Answer 17

Most cause unknown
Turners
Pure gonadal dysgenesis
Single gene defects (Fragile X, Fanconi's, CAH, Galactosemia)
Autoimmune
Rads, chemo, toxins

Question 18

Workup for POI

Answer 18

HCG, FSH, PRL, Karyotype, TSH, FMR1 gene testing, Adrenal Abs, Pelvic U/S
Progesterone w/drawl test

Question 19

Markers of ovarian reserve

Answer 19

AMH
Inhibin B
Antral Follicle count
FSH
Estradiol

Question 20

INdications to investigate for precocious puberty?

Answer 20

Pubertal changes under 7 (<6 for AA)

Question 21

DDx of true/central precocious puberty

Answer 21

Idiopathic (75%)
CNS - GnRH secreting hamartomas, craniopharyngiomas, gliomas, NF, hydrocephalus, SOD, tuberous sclerosus, post-meningitis

Question 22

DDx of peripheral precocious puberty

Answer 22

Ovarian cyst/tumor (ie. granulosa cell, thecal cell)
Adrenal disease (ie. CAH, tumor)
Hypothyroidism
McCune Albright
Ectopic Gn secretion
Exogenous steroid exposure

Question 23

Single most useful test in precocious puberty

Answer 23

Bone age. If true/CPP, bone age is increased

Question 24

Tx for precocious puberty

Answer 24

GnRH agonists (except for McCune Albright - wont work. Use AI’s)

Question 25

Order of events in puberty

Answer 25

TAGM
Thelarche, Adrenarche, Growth peak, Menarche
Over 4.5 yrs

Question 26

Tanner stages

Answer 26

I-none
II-breast buds
III-elevated tissue, coarse curly hair
IV-secondary mound, hair across pubis
V-adult size breasts, hair medial thights