Term
| Describe adrenal blood supply? |
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Definition
inf phrenic--->superior adrenal art
aorta--------->middle adrenal art
renal art------>inferior adrenal art
left renal vein <------left adrenal vein
IVC<--------------right adrenal vein |
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Term
| difference between left vs right adrenal vein drainage? |
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Definition
left adrenal vein goes to left renal vein
right adrenal v goes direct to IVC |
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Term
Adrenal cortex
layers and secretion products? |
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Definition
Glomerulosa -->aldosterone
Fasciculata ----> glucorcorticoids
Reticularis -----> androgens + estrogens (minor role) |
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Term
| from where is adrenal cortex derived? |
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Definition
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Term
| enzyme common to all layers of adrenal cortex = ? |
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Definition
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Term
| basic steroid hormone production pathway = ? |
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Definition
| Cholesterol->progesterone-->androgens-->cortisol-->aldosterone |
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Term
| Adrenal cortex vs medulla innervation source? |
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Definition
cortex has none
medulla is from splanchnic nerve |
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Term
| Adrenal medulla basic lymphatic drainage? |
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Definition
| -->subdiaphragmatic and renal LN |
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Term
Hypothal-pit-adrenal axis
-basic physio |
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Definition
| CRH from hypothal-->ant pit, releases ACTH-->stims adrenal cortex--> cortisol |
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Term
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Definition
aa breakdown, lipolysis, gluconeogenesis
-inc blood glucose 2/2 production and insulin resistance
-inotropic effect |
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Term
| general pattern of cortisol blood levels? |
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Definition
Dinural
high am, low pm (peak = 4-6 a.m.
-also inc with stress |
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Term
| origin of adrena medulla? |
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Definition
| neural crest cells (ectoderm) |
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Term
| basic catacholamine synth pathway? |
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Definition
TYR-->dopa-->dopamine-->NE-->Epi
(TYR OHylase = Rate lim step @ TYR->Dopa) |
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Term
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Definition
only enzyme that converts NE -->Epi
ONLY found at adrenal medulla |
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Term
|
Definition
NE-->Normetanephrine
epi-->Normetanephrine
--->Normeta and metanephrine produce VMA |
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Term
| MC site of extra-adrena neural crest tissue? |
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Definition
Organ of Zuckerkandl (aortic bifurcation)
also retroP |
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Term
| what needs supplementation after bilateral adrenalectomy? |
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Definition
glucorcorticoids and mineralocorticoids
-prevents addisons dz (adrenal insuff)
-prevents Nelsons syndrome |
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Term
CAH
what are the three types? |
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Definition
21 OHylase deficiency
11 OHylase deficiency
17 OHylase deficiency |
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Term
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Definition
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Term
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Definition
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Term
| PPhys of 21 OHylase deficiency? |
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Definition
| Inc 17-OH progesterone = inc testosterone |
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Term
|
Definition
1. HypoTN (salt wasting crisis @ 2nd-3rd week of life)
2. Virilization in females. Precocious puberty in males |
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Term
|
Definition
1. 20 cc/kg bolus, hydrocort, glucose vs Salt wasting
2. long term: hydrocort, fludrocort, +/-genitoplasty for female |
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Term
11 OHylase def
-hormone level effect? |
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Definition
increase 11-deoxycortisone
inc testosterone |
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Term
11 OHylase deficiency
-sxs |
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Definition
HTN (salt saving, since deoxycort is increased and acts as mineralocorticoid)
virilization of females, precocious puberty in males |
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Term
|
Definition
hydrocort, possible genitoplasty for females
NO Fludrocortisone |
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Term
| 17 OHylase def hormone level effect? |
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Definition
| Inc progesterone, pregnenolone |
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Term
|
Definition
HTN (salt saving)
Males: ambiguous genitalia at birth
Females: miss puberty (tx w/estrogen to induce puberty) |
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Term
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Definition
Hydrocortisone, possible genitoplasty for MALES
NO Fludrocortisone |
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Term
CAH
-which subtypes should not receive a specific corticosteroid?
-which subtypes are amenable to genitoplasty in M vs F? |
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Definition
-11 and 17OHylase def==>NO FLUDROCORT
-21 and 11 ==>genitoplasty for FEMALES
-17 ==>Genitoplasty for males |
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Term
| basic labs/imaging for pt with malignant HTN? |
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Definition
24 hour urine studies
serum studies
renal/carotid US
CT CAP |
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Term
| % of people with adrenal incidentaloma? |
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Definition
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Term
MCC adrenal incidentaloma
bengin vs malig |
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Definition
benign = non-functional adrenal adenoma
malig = met from separate primary (MC = lung CA) |
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Term
Adrenal and mets
-MC mets to adrenal? |
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Definition
lung (MC), breast, melanoma, renal
(adrenal is MC site for mets)? |
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Term
| CT findings suggestive of benign adrenal mass? |
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Definition
Hypodense (< 10 Hounsfield units, lipid based)
well circumscribed |
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Term
| FNA indications for asymptomatic adrenal mass? |
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Definition
-CT: > 10 HF units, or < 50% washout at 10 min @ contrast CT
-previous CA or suspicious of a met
-young age (less likely benign) |
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Term
| Surgery indications for asympt adrenal mass? |
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Definition
-greater than 5 cm
-Ominous: complex, hemorrhagic, irregular, heterogen, dense vascular
-functional, enlarging, +FNA |
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Term
| schedule for following asympt adrenal mass? |
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Definition
| CT abd q3 months x 1 year-->then yearly |
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Term
|
Definition
aspirate
-->clear = follow it and resect if it recurs
-->bloody = resection |
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|
Term
FNA shows agniomyolipoma
-->mgmt? |
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Definition
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Term
|
Definition
1st: 24 hr urine free cortisol + serum ACTH
-high cortisol, low ACTH = adrenal lesions (hyprplsa vs adenoma)
-high cortisol, high ACTH = pituitary vs ectopic source
2nd: if high/high-->give high dose dexameth sup test
-->if suppresses ACTH, then pit source, if not then ectopic |
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Term
| most sensitive test for cushing? |
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Definition
| 24 hr urine free cortisol |
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Term
| use of NP-59 scintigraphy in cushing dx? |
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Definition
is taken up by adrenal adenomas, but not CA
-ddx hyperplasia vs adenoma |
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Term
|
Definition
iatrogenic = MCC overall
Cushing disease = pituitary adenoma = MCC non-iatrogenic cushings syndrome |
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Term
|
Definition
-trans-sphen rsxn-->XRT for residual tumor
-Possible bilateral adrenalectomy (medically with mitotane) if cant find pit adenoma |
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Term
| pt with cushings disease but cant find pit source? |
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Definition
consider bilateral adrenalectomy
-surgically vs medically w/mitotane |
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Term
| what needs supplementation after trans-spehnoidal cushings disease surgery vs bilateral adrenalectomy? |
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Definition
-trans sphen: 3 years of glucorcorticouds
-bilat adrenal: life long glucocort = mineralocort |
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Term
| MC source of extra-pit cushing syndrome? |
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Definition
small cell lung CA
(not suppressed with high dose dexameth test) |
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Term
| Imaging options to localize Ectopic ACTH secreting tumor? |
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Definition
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Term
| Top 3 causes of non-iatrogenic cushing syndrome |
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Definition
1 = pit adenoma
2 = ectopic tumor (MCC Small cell lung)
3 = adrenal adenoma (low ACTH) |
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Term
Adrenocortical carcinoma
-MC age? |
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Definition
| bimodal (before age 5, after 5th decade) |
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Term
Adrenocortical carcinoma
-% functional
-% advanced at dx/ |
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Definition
50% functional
80% advanced |
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Term
Adrenocortical carcinoma
-sxs |
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Definition
-90% virilization of females /precocc puberty @ males,
-feminization in men, masculization in women can occur
-HTN, abd pain, weight loss, weakness |
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Term
|
Definition
Radical adrenalectomy (takes kidney)
-debulking can inc survival
-mitotane post op = inc disease free survival |
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Term
|
Definition
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Term
| Medical tx options for cushing syndrome? |
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Definition
@ diffuse adrenal hyperplasia, or unresectable cause
-metyrapone--| steroid prod
-aminoglutethimide--| cholest conversion
-ketoconazole --| inhib steroid prod
mitotane (Op-DDD) -->adrenal -lytic (vs adrenocortical CA) |
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Term
| indications for bilateral adrenalectomy? |
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Definition
Unresectable ectopic ACTH tumor (if slow growing)
Pit adenoma that cant be found
bilateral adrenal hyperplasia (medical first) |
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Term
| difference between gluco and mineralocorticoid |
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Definition
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Term
|
Definition
| ectopic NCC or sympathetic ganglia |
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Term
|
Definition
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Term
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Definition
| 10% are.. malignant, bilateral, pediatric, familial, extra-adrenal, a/w MEN |
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Term
| what type of pheo is most likely malignant? |
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Definition
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Term
| What genetic syndromes have pheos? |
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Definition
MEN 2A/B
VHL, NF type 1, Tuberous sclerosis, Sturge Weber, familial paragnanlioma |
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Term
Pheo dx
-highest specificity test? |
|
Definition
24 hour urine- Epi, NE, VMA, metanephrines/normeta
-98% specificity
-VMA is the MOST specific |
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Term
Clonidine suppression test
-role in pheo dx? |
|
Definition
tumor does not respond to clonidine suppression
-still have high catacholamines |
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Term
| work-up for localizing pheo? |
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Definition
1. CT-->if fails...
2.MIBG scan (131 meta iodobenzylguanidine) = NE analogue
3.-->if still cant find it==> exlap |
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Term
| What test should not be done to locate a pheo? |
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Definition
| No venography-->causes HTN crisis |
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Term
|
Definition
1. alpha block (phenoxybenzamine or prazosin) 2 wk pre-op
-->titrate up dose until juuuust orthostatic.
2. give vol replacement during alpha block (should gain weight)
3. can now beta block |
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|
Term
Pheo resection
1. approach?
2. first major step? |
|
Definition
-Abdominal approach if malignant (10% are!)
-Ligate adrenal v first to avoid spilling catachol. w/manipulation of tumor |
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|
Term
| extra-adrenal sites of pheo? |
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Definition
MC = aortic bifurcation
Vert bodies, paravertebral symp ganglion, contralat adrenal, bladder |
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|
Term
inra-op trouble shooting vs pheo resection
1. hypertension
2. persistent htn after rsxn
3. hypotension after removal
4. bronchospasm after removal |
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Definition
1. esmolol, nipride
2. check other sites (10% are bilateral)
3. volume first-->then phenylephrine
4. albuterol, epinephrine |
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Term
| Drug that inhibits Tyr OHylase? |
|
Definition
Metyrosine
= dec catachol production |
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Term
| what causes false positive VMA ? |
|
Definition
| caffeine, fruits, vanilla, iodine, alpha/beta blockers |
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Term
Conn syndrome
1. =
2. general lab trends? |
|
Definition
primary hyper-aldosteronism
-inc aldost, dec renin |
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Term
|
Definition
1. Adenoma (80% = MCC)
2. adrenal hyperplasia
3. adrenocortical CA (<1 %)
4.ovarian tumors |
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|
Term
secondary hyper-aldosteronism
-frequency vs primary hyper-ald
-causes? |
|
Definition
-more frequent
-primary reninism (renin tumor), 2ndary reninism (several causes) |
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Term
| sxs primary hyperaldosteronism |
|
Definition
HTN (2/2 Na retention but WITHOUT edema)
HypoK+, polydipsia, polyuria, weakness
--HTN WITH HYPOKALEMIA--- |
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Term
primary hyperaldosteronism
dx |
|
Definition
-plasma aldost:renin ratio > 20
-salt suppress test: 3 days of salt load-->24 hr urine aldost
--> aldost. > 14 mcg , UNa > 200
(all BP meds interfere with test EXCEPT alpha blk) |
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Term
| Localizing studies for Conn syndrome lesion |
|
Definition
1. Abd CT/MRI
2. selective adrenal venous sampling for aldosterone |
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Term
|
Definition
Adenoma = adrenalectomy
Diffuse hyperplasia:
-spironolactone, CCBlk, eplerenone (aldost antag), anti-HTNs
-K supp
Last resort = bilat adrenalectomy |
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|
Term
| MC indication for bilateral adrenalectomy @ Conn? |
|
Definition
refractory hypokalemia
-need fludro and hydrocortisone post op |
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Term
|
Definition
| Renal dysfunction 2/2 DM nephropathy |
|
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Term
|
Definition
1. hypo-reninemic hypoaldosteronism (hyperK, HTN, EDEMA)
2. primary aldosterone def (hyperK, HYPOtn 2/2 salt wasting)
3. Aldost resistance (hyperK, HYPOtn 2/2 volume loss) |
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Term
|
Definition
1. hypo-reninemic type-->fludro, antiHTN, lasix
2. primary aldost deficiency-->fludro, hydrocort if adrenal insuf, volume if salt wasting (ie CAH)
3. aldost resistance -->stop diuretic |
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Term
| Sxs acute adrenal insuff? |
|
Definition
| fever, N/V, abd pain, HYPOtn unresponsive to resusc, AMS, hypoglycemia, diarrhea |
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Term
| MCC of acute adrenal insuff? |
|
Definition
-MCC = withdrawal of exog. roids
-bilateral adrenal hem (MCC = sepsis (waterhouse-fried synd)
-adrenalectomy
-Severe stress (trauma, sepsis) |
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|
Term
|
Definition
= chronic adrenal insuff
sx: weakness, weight loss, abd pain, HYPERPIG 2/2 MSH (ACTH breakdown product) |
|
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Term
|
Definition
primary adrenal DZ : autoimmune MC
-TB, fungal, adrenal mets, pituitary dz |
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Term
|
Definition
Inc ACTH.
Dec cortison, dec aldosterone
Lytes: HyperK, hyperGlycemic, hypoNa |
|
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Term
|
Definition
Hydrocort, fludrocort
(use dexamethasone while doing ACTH stim test->measure cortisol) |
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|
Term
| Waterhoue freidrichsen synd MCC org |
|
Definition
|
|
Term
|
Definition
Hemangioblastoma (cerebellar, spinal cord, retina)
Renal angioma, RCCarcinoma
Pheo |
|
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Term
|
Definition
1. Myxoma @ heart and skin + hyperpig (lentiginosis)
2. Endocrine over-activity (ex: cushings)
(cause = PR-KAR tumor suppressor) |
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