endocrinology

Question 1

craniopharyngioma - pearl

Answer 1

must be considered when child older than 5 is not growing 2 inches per year –> GH deficiency

Question 2

FSH produces and stimulates in women

Answer 2

stimulates ovaries to produce estrogen

Question 3

FSH produces and stimulates in men

Answer 3

stimulates seminiferous tubules to produce sperm

Question 4

LH in men

Answer 4

leydig cells to produce androgens

Question 5

cause of boys precocious puberty

Answer 5

hydrocephalus, CNS infections, CP, hamartomas, tumors, head trauma cause increase FSH and LH

Question 6

Mccune albright syndrome

Answer 6

bony changes (fibrous dysplasia), skin findings (hyperpigmented macules, cafe au lait spots) and peripheral precocious puberty
- testicular enlargement

Question 7

testotoxicosis

Answer 7

testes enlarge bilaterally independent of HPGA

Question 8

beta HCG tumors

Answer 8

boys

• tumors in chest, pineal gland, gonad, liver
• stimulates Leydig cells to secrete androgens

Question 9

hypogonadotropic hypogonadism

Answer 9

inactivity of hypothalamus and pituitary gland
- low FSH and low LH, low testosterone and low estradiol with flat GnRH test
Sx: delayed puberty and delayed growth

Question 10

hypergonadotropic hypogonadism

Answer 10

high FSH and high LH levels with low testosterone or low estradiol levels, no abnl hypothalamus or pituitary gland
Sx: delayed puberty

Question 11

kallman syndrome

Answer 11

isolated gonadotropic deficiency with anosmia

- delayed puberty

Question 12

lawrence moon biedl syndrome

Answer 12

obesity, retinitis pigmentosa, hypogonadism, polysyndactyly

- delayed puberty

Question 13

causes of undervirilized male (pseudoherm)

Answer 13

errors or testosterone, gonadal intersex, partial androgen insensitivity

Question 14

causes of virilized female (pseudoherm)

Answer 14

congenital adrenal hyperplasia (21 hydroxylase deficiency most common cause, presents with increased blood pressure), virilizing drug during pregnancy, virilizing tumor during pregnancy

Question 15

congenital adrenal hyperplasia

Answer 15

auto rec
- most common cause of ambiguous genitalia, 21 hydrolxylase deficiency
- increased androgens
Sx: chronic salt wasting, FTT, electrolyte abnormalities –> 21 hydroxylase deficiency
- hypertensive, hypokalemic –> 11 beta hydroxylase

Question 16

waterhouse friderichsen

Answer 16

adrenal insufficiency with meningococcemia

Question 17

tx of adrenal crisis

Answer 17

IV fluids with 5% dextrose in ns, steroids given

Question 18

most common cause of congenital hypothyroidism

Answer 18

thyroid dysgenesis (absent thryoid or thyroid hypoplasia
- 1/3 have ectopic thyroid gland)

Question 19

vitamin d resistant rickets

Answer 19

familial hypophophatemia
- most common form of rickets in US, x linked dom
- renal tubular phosphorus leak –> low phosphorus
Sx: bowing of legs, short stature, rachitic rosary, craniotabes, frontal bossing delayed suture closure

Question 20

beckwith wiedemann syndrome

Answer 20

LGA, macroglossia, umbilical hernia, hyperinsulinemic hypoglycemia