Endo

Question 1

Criteria for growth failure

Answer 1

Height below 1st percentile,

Or height >2SD below mid-parental height

Question 2

GH level consistent with GH deficiency

Answer 2

<10 ng/mL in each of 2 provocative tests

Question 3

GH deficiency is almost universal 5 yr after therapy with a total dose of

Answer 3

> = 35 Gy

Question 4

End organ resistance to GH due to mutation of the GH receptor

Answer 4

Laron syndrome

Question 5

Concurrent treatment with GH and __ has been used to interrupt puberty and delay epiphyseal fusion and prolong growth

Answer 5

GnRH agonist

Question 6

Criteria for stopping treatment of GH

Answer 6

• Decision by the patient that he or she is tall enough
• growth rate <1 in/yr
• bone age >14 yr in girls, >16 in boys

Question 7

The diagnosis of DI is established by serum osmolality __ and urine osmolality __. Unlikely if serum osmolality is __ and urine osmolality is __.

Answer 7

Serum osm >300 mOsm/kg
Urine osm <300 mOsm/kg
Unlikely if
Serum osm <270
Urine osm >600

Question 8

DI, DM, optic atrophy, deafness

Answer 8

Wolfram syndrome

Question 9

Triphasic response following neurosurgery

Answer 9

1. Transient DI (12-48 hr)
2. SIADH (up to 10 days)
3. Permanent DI

Question 10

__ is the cause of 50% of cases of “idiopathic” central DI

Answer 10

Hypophysitis

Question 11

Urine sodium excretion in CSW

Answer 11

> 150 meq/L

ANP >20pmol/L

Question 12

Gold standard for diagnosis of GH excess

Answer 12

Failure to suppress GH to <5 ng/dL after 1.75 g/kg oral glucose challenge

Question 13

Competes with GH for binding to the GH receptor

Answer 13

Pegvisomant

Question 14

Bromocriptine
Classification
Indication

Answer 14

Dopamine agonist

GH and prolactin oversecretion

Question 15

Octreotide
Classification
Indication

Answer 15

Somatostarin analog

GH excess

Question 16

Precocious puberty is defined by onset of secondary sexual characteristics before age __ in girls and __ in boys

Answer 16

Before age 8 in girls, 9 in boys

Question 17

Most common lesion causing precocious puberty

Answer 17

Hypothalamic hamartomas

Question 18

Most common cause of acquired hypothyroidism

Answer 18

Chronic lymphocytic thyroiditis

Question 19

Autosomal dominant disorder characterized by hyperplasia or neoplasia of the endocrine pancreas, anterior pituitary and parathyroid glands

Answer 19

MEN type 1

Question 20

Autosomal dominant disorder characterized by parathyroid adenomas and fibro-osseus jaw tumors

Answer 20

Hyperparathyroidism-jaw tumor syndrome

Question 21

Most consistent and characteristic radiographic finding in hyperparathyroidism

Answer 21

Resorption of subperiosteal bone, best seen along the margins of the phalanges of the hands

Question 22

salt-losing forms of adrenal hyperplasia

Answer 22

21-hydroxylase deficiency
lipoid adrenal hyperplasia
deficiency of 3B-hydroxysteroid dehydrogenase

Question 23

Treatment of acute adrenal insufficiency

Answer 23

IV D5NSS
Hydrocortisone 
10 mg for infants
25 mg for toddlers
50 mg for older children
100 mg for adolescents
x2-3 as stress dosing

Question 24

Treatment for aldosterone deficiency

Answer 24

fludrocortisone

Question 25

Second gold standard test for secondary adrenal insufficiency

Answer 25

metyrapone (inhibitor of steroid 11B-hydroxylase)

Question 26

Diagnosis of 21-hydroxylase deficiency is most reliably established by measuring __ before and 30 or 60 min after an intravenous bolus of ACTH

Answer 26

17-hydroxyprogesterone

Question 27

Salt wasting crisis, incomplete virilization in boys, mild virilization of girls. Diagnosis?

Answer 27

3B-hydroxysteroid deficiency

Question 28

Midnight cortisol level __ strongly suggest Cushing syndrome

Answer 28

> 4.4 mcg/dL

Question 29

Result of single-dose dexamethasone suppression test in NORMAL individuals (dexa 25-30 mcg/kg given at 11 PM)

Answer 29

plasma cortisol level of <5 mcg/dL at 8 am the next morning

Question 30

Most common site of origin of pheochromocytomas?

Answer 30

adrenal medulla

Question 31

Outside the adrenal medulla, usual location of pheochromocytoma?

Answer 31

abdominal sympathetic chain, near the aorta at the level of the inferior mesenteric artery or at its bifurcation (more often on the right side)

Question 32

Best sensitivity and specificity for pheochromocytoma in children

Answer 32

plasma normetanephrine

next best is plasma norepinephrine

Question 33

Taken up by chromaffin tissue and is useful for localizing small tumors

Answer 33

1311-metaiodobenzylguanidine (MBIG)

Question 34

If an adrenal mass is nonfunctional and larger than __, recommendations are to proceed with surgical resection

Answer 34

4-6 cm

Functional tumors require removal

Question 35

Nephropathy, ambiguous genitalia, bilateral Wilms tumor

Answer 35

Denys-Drash syndrome

Question 36

Children with longstanding diabetes and no insulin reserve require about __ if prepubertal, __ at midpuberty and __ by the end of puberty

Answer 36

0.7 u/kg/d if prepubertal
1 u/kg/d at midpuberty
1.2 u/kg/d by the end of puberty

Question 37

Normally, the rostral end of the neural tube closes on the __ day and the caudal neuropore closes on the __ day of development

Answer 37

rostral 23rd

caudal 27th