Endo Flashcards
Criteria for growth failure
Height below 1st percentile,
Or height >2SD below mid-parental height
GH level consistent with GH deficiency
<10 ng/mL in each of 2 provocative tests
GH deficiency is almost universal 5 yr after therapy with a total dose of
> = 35 Gy
End organ resistance to GH due to mutation of the GH receptor
Laron syndrome
Concurrent treatment with GH and __ has been used to interrupt puberty and delay epiphyseal fusion and prolong growth
GnRH agonist
Criteria for stopping treatment of GH
- 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
The diagnosis of DI is established by serum osmolality __ and urine osmolality __. Unlikely if serum osmolality is __ and urine osmolality is __.
Serum osm >300 mOsm/kg Urine osm <300 mOsm/kg Unlikely if Serum osm <270 Urine osm >600
DI, DM, optic atrophy, deafness
Wolfram syndrome
Triphasic response following neurosurgery
- Transient DI (12-48 hr)
- SIADH (up to 10 days)
- Permanent DI
__ is the cause of 50% of cases of “idiopathic” central DI
Hypophysitis
Urine sodium excretion in CSW
> 150 meq/L
ANP >20pmol/L
Gold standard for diagnosis of GH excess
Failure to suppress GH to <5 ng/dL after 1.75 g/kg oral glucose challenge
Competes with GH for binding to the GH receptor
Pegvisomant
Bromocriptine
Classification
Indication
Dopamine agonist
GH and prolactin oversecretion
Octreotide
Classification
Indication
Somatostarin analog
GH excess
Precocious puberty is defined by onset of secondary sexual characteristics before age __ in girls and __ in boys
Before age 8 in girls, 9 in boys
Most common lesion causing precocious puberty
Hypothalamic hamartomas
Most common cause of acquired hypothyroidism
Chronic lymphocytic thyroiditis
Autosomal dominant disorder characterized by hyperplasia or neoplasia of the endocrine pancreas, anterior pituitary and parathyroid glands
MEN type 1
Autosomal dominant disorder characterized by parathyroid adenomas and fibro-osseus jaw tumors
Hyperparathyroidism-jaw tumor syndrome
Most consistent and characteristic radiographic finding in hyperparathyroidism
Resorption of subperiosteal bone, best seen along the margins of the phalanges of the hands
salt-losing forms of adrenal hyperplasia
21-hydroxylase deficiency
lipoid adrenal hyperplasia
deficiency of 3B-hydroxysteroid dehydrogenase
Treatment of acute adrenal insufficiency
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
Treatment for aldosterone deficiency
fludrocortisone
Second gold standard test for secondary adrenal insufficiency
metyrapone (inhibitor of steroid 11B-hydroxylase)
Diagnosis of 21-hydroxylase deficiency is most reliably established by measuring __ before and 30 or 60 min after an intravenous bolus of ACTH
17-hydroxyprogesterone
Salt wasting crisis, incomplete virilization in boys, mild virilization of girls. Diagnosis?
3B-hydroxysteroid deficiency
Midnight cortisol level __ strongly suggest Cushing syndrome
> 4.4 mcg/dL
Result of single-dose dexamethasone suppression test in NORMAL individuals (dexa 25-30 mcg/kg given at 11 PM)
plasma cortisol level of <5 mcg/dL at 8 am the next morning
Most common site of origin of pheochromocytomas?
adrenal medulla
Outside the adrenal medulla, usual location of pheochromocytoma?
abdominal sympathetic chain, near the aorta at the level of the inferior mesenteric artery or at its bifurcation (more often on the right side)
Best sensitivity and specificity for pheochromocytoma in children
plasma normetanephrine
next best is plasma norepinephrine
Taken up by chromaffin tissue and is useful for localizing small tumors
1311-metaiodobenzylguanidine (MBIG)
If an adrenal mass is nonfunctional and larger than __, recommendations are to proceed with surgical resection
4-6 cm
Functional tumors require removal
Nephropathy, ambiguous genitalia, bilateral Wilms tumor
Denys-Drash syndrome
Children with longstanding diabetes and no insulin reserve require about __ if prepubertal, __ at midpuberty and __ by the end of puberty
0.7 u/kg/d if prepubertal
1 u/kg/d at midpuberty
1.2 u/kg/d by the end of puberty
Normally, the rostral end of the neural tube closes on the __ day and the caudal neuropore closes on the __ day of development
rostral 23rd
caudal 27th
