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37 Cards in this Set

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what are the male and female manifestations of 17-alpha-hydroxylase deficiency
Both have HYPERTENSION + HYPOKALEMIA (high aldosterone, low cortisol/sex hormones)

XY: psuedohermaphroditism: externally female (lack of DHT), no reproductive structures because they still have sertoli cells and MIF.

XX: extrenally females with normal internal sex organs but lacking secondary sexual characteristics (sexual infantilism)
hypotension, hyperkalemia + masculinization and ambigous genitalia in female
21-hydroxylase deficiency

*have decrease aldosterone, cortisol, increased sex hormones.
*increased ACTH
hypertension + high sex hormones
11-beta-hydroxylase deficiency

*remember 11-DOC is a mineralocoricoid and is ssecreted in excess

*11-DOC also does NOT cause feedback inhibition on ACTH so it will continue to increase.
What 3 effects does PTH have on the kidney
1. decreased Na/PO4 co-transport in the PCT
2. decrease Ca2+ reabsorption in the DCT
3. stimulate 1-alpha-hydroxylase
How does PTH activate osteoclasts?
PTH --> osteoblasts --> RANK-L, M-CSF ---> osteoclast differentiation
How does Mg2+ affect PTH secretion
decrease Mg2+ = decrease PTH

common causes of decreased Mg2+ include diarrhea, aminogylcosides, diuretics, alcohol abuse.
what are the steps of vitamin D synthesis starting from sunlight?
1. provitamin D3 is converted to vitamin D3 using sunlight
2. vitamin D3 is converted to 25-OH vitamin D using CYP450
3. 25-OH vitamin D is converted to 1,25 vitamin D3 using 1 alpha hydroxylase

*24,25 OH2 vitamin Dis inactive form
how do you differentiate PITUITARY adenoma cushing's from ectopic ACTH?
dexamethasone test:

pituitary adenoma will decrease cortisol after high dose of dexamethasone (synthetic glucocorticoid)

ectopic ACTH (small cell carcinoma) or ADRENAL adenoma will have high cortisol no matter the dexamethasone dose.
explain secondary hyperaldosteronism
kidney perception of low intravascular volume (cirrhosis, renal artery stenosis, CHF, nephrotic disease) causes activation of aldosterone.

see high renin levels (as opposed to primary hyperaldosteronism from an alodosterone secreting tumor)
what serum levels are expected in addison's disease
since there is destruction of all 3 layers of the adrenal cortex- there is no aldosterone or cortisol.

no aldosterone = hyperkalemia, acidosis, hypotension

no cortisol = hypotension also

*treat with steroids ASAP
urine levels of ____ are elevated in a child suffering from a tumor of the adrenal medulla (although it can occur anywhere along the sympathetic chain)
neuroblastoma

HVA (homovanillic acid) a breakdown product of dopamine

*associated with n-myc
potbellied pale puffy faced child with protruding umbulicus and protuberant tongue
cretinism

*usually from lack of dietary iodine, sporatically can occur in defect in T4 formation or no thyroid
histological findings of person with painful very tender thyroid that developed after a flulike illness.
subacute thyroiditis (de quervian's) has granulomatous inflammation

*also elevated ESR, jaw pain
focal patches of hyperfunctioning follicular cells working independently of TSH due to mutation of TSH receptor
toxic multinodular goiter

*different than grave's - no exopthalmos, pre-tibial myxedema
3 histologic findings on papillary carcinoma of thyroid
1. ground glass nuclei (orphan annie)
2. psamomma bodies (calcified)
3. nuclear grooves

*increased risk with childhood radiation
serum levels for primary hyperparathyroidism
-Ca
-PO4
-alk phos
-PTH
-Ca = high
-PO4 = low
-alk phos = high
-PTH = high
serum levels for secondary hyperparathyroidism
-Ca
-PO4
-alk phos
-PTH
secondary is usually due to chronic renal disease and low Vit D --> low Ca2+

-Ca = low
-PO4 = high
-alk phos = high
-PTH = high
3 cardinal signs of Albright's hereditary osteodystrophy (psuedohypoparathyroidism)
Caused by unresponsiveness to PTH
1. short height
2. hypocalcemia
3. shortened 4th/5th digits
two possible treatments for prolactinoma
bromocriptine
cabergoline

-both are dopamine agonists
how do you diagnose acromegaly (serum)
high IGF-1
treatment for central DI and nephrogenic DI
DI = low ADH effect = peeing all the time dilute urine

central = intranasal desmopressin (ADH)

nephrogenic = need some way to reabsorb water- hydrochlorathiazide, indometacin (inhibits prostaglandins, which normally inhibit ADH), amiloride
5 causes of SIADH (too much ADH = too much water reabsorbed)
1. ectopic ADH (small cell lung cancer)
2. CNS disorders/head trauma
3. pulmonary disease.
4. drugs - cyclophosphamide
histological differences in DM1/DM2
DM1 = islet lymphocytic infiltrate

DM2 = islet amyloid deposit
what are the 3 rapid acting insulins?
Lispro, Aspart (both peak @ 30 mins)
Regular insulin

*remember effects of insulin- increase glycogen storage in liver/muscle, protein synthesis, TG storage, K uptake
what are the two long acting insulins
Glargine
Detemir
mechanism of tolbutamine/chlorprpamide
close K+, Ca influx --> release of insulin (only works if you have some working beta cells, so useless in DM1)
side effect of first generation sulfonylurease and second generation
1st gen = tolbutamine, chloropropamide - disulfiram like effects

2nd gen = glyburide, glimepiride, glipizide - hypoglycemia

*contraindicated in pregnancy can cause insulin release in baby
mechanism of metformin
deccrease gluconeogenesis, increase glycolysis, increase peripheral glucose uptake (increase insulin sensitivity)
diabetes drug that has side effect of lactic acidosis and should be avoided in patient with renal failure
metformin (biguanide)
mechanism of rosiglitazone
increases sensitivity of insulin by binding to PPAR-Y nuclear transcription regulator
4 side effects of pioglitazone
weight gain
edema
hepatotoxicity
CV toxicity
what two drugs can inhibit intestinal brush border alpha glucosidases and decrease postprandial hyperglycemia
acarbose
miglitol

side effects = gi distrubances
what diabetes drug decreases glucagon
pramlintide

side effect = hypoglycemia
what diabetes drug is a GLP-1 analog that increases insulin and decreases glucagon
exenatide

se = pancreatitis
what two steps of T3/T4 synthesis do propylthiouracil/methimazole block?
organification (TG + I2 --> MIT+DIT)
coupling (MIT+DIT --> T3/T4)
3 side effects of propylthiouracil/methimazole
skin rash
agranulocytosis
aplastic anemia

methimazole is a possible teratogen
what 4 diseases does somatostatin (octeotride) treat
acromegaly
carcinoid
gastrinoma
glucagonoma