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21 Adrenal
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93
Religious Studies
10th Grade
11/15/2017

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Term
Describe adrenal blood supply?
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
Term
difference between left vs right adrenal vein drainage?
Definition
left adrenal vein goes to left renal vein
right adrenal v goes direct to IVC
Term
Adrenal cortex
layers and secretion products?
Definition
Glomerulosa -->aldosterone
Fasciculata ----> glucorcorticoids
Reticularis -----> androgens + estrogens (minor role)
Term
from where is adrenal cortex derived?
Definition
mesoderm
Term
enzyme common to all layers of adrenal cortex = ?
Definition
21 and 11 beta OHylase
Term
basic steroid hormone production pathway = ?
Definition
Cholesterol->progesterone-->androgens-->cortisol-->aldosterone
Term
Adrenal cortex vs medulla innervation source?
Definition
cortex has none
medulla is from splanchnic nerve
Term
Adrenal medulla basic lymphatic drainage?
Definition
-->subdiaphragmatic and renal LN
Term
Hypothal-pit-adrenal axis
-basic physio
Definition
CRH from hypothal-->ant pit, releases ACTH-->stims adrenal cortex--> cortisol
Term
cortisol effects?
Definition
aa breakdown, lipolysis, gluconeogenesis
-inc blood glucose 2/2 production and insulin resistance
-inotropic effect
Term
general pattern of cortisol blood levels?
Definition
Dinural
high am, low pm (peak = 4-6 a.m.
-also inc with stress
Term
origin of adrena medulla?
Definition
neural crest cells (ectoderm)
Term
basic catacholamine synth pathway?
Definition
TYR-->dopa-->dopamine-->NE-->Epi
(TYR OHylase = Rate lim step @ TYR->Dopa)
Term
PNMT function?
Definition
only enzyme that converts NE -->Epi
ONLY found at adrenal medulla
Term
MAO function
Definition
NE-->Normetanephrine
epi-->Normetanephrine
--->Normeta and metanephrine produce VMA
Term
MC site of extra-adrena neural crest tissue?
Definition
Organ of Zuckerkandl (aortic bifurcation)
also retroP
Term
what needs supplementation after bilateral adrenalectomy?
Definition
glucorcorticoids and mineralocorticoids
-prevents addisons dz (adrenal insuff)
-prevents Nelsons syndrome
Term
CAH
what are the three types?
Definition
21 OHylase deficiency
11 OHylase deficiency
17 OHylase deficiency
Term
MCC of CAH?
Definition
21 OHylase def
Term
MCC ambigious genitalia?
Definition
21 OHylase
Term
PPhys of 21 OHylase deficiency?
Definition
Inc 17-OH progesterone = inc testosterone
Term
Sxs 21 OHylase def?
Definition
1. HypoTN (salt wasting crisis @ 2nd-3rd week of life)
2. Virilization in females. Precocious puberty in males
Term
21 OHylase def tx?
Definition
1. 20 cc/kg bolus, hydrocort, glucose vs Salt wasting
2. long term: hydrocort, fludrocort, +/-genitoplasty for female
Term
11 OHylase def
-hormone level effect?
Definition
increase 11-deoxycortisone
inc testosterone
Term
11 OHylase deficiency
-sxs
Definition
HTN (salt saving, since deoxycort is increased and acts as mineralocorticoid)
virilization of females, precocious puberty in males
Term
Tx 11OHylase def
Definition
hydrocort, possible genitoplasty for females
NO Fludrocortisone
Term
17 OHylase def hormone level effect?
Definition
Inc progesterone, pregnenolone
Term
Sx 17 OHylase def?
Definition
HTN (salt saving)
Males: ambiguous genitalia at birth
Females: miss puberty (tx w/estrogen to induce puberty)
Term
Tx 17 OHylase def?
Definition
Hydrocortisone, possible genitoplasty for MALES
NO Fludrocortisone
Term
CAH
-which subtypes should not receive a specific corticosteroid?
-which subtypes are amenable to genitoplasty in M vs F?
Definition
-11 and 17OHylase def==>NO FLUDROCORT
-21 and 11 ==>genitoplasty for FEMALES
-17 ==>Genitoplasty for males
Term
basic labs/imaging for pt with malignant HTN?
Definition
24 hour urine studies
serum studies
renal/carotid US
CT CAP
Term
% of people with adrenal incidentaloma?
Definition
1%, inc w/age
size > 1 cm
Term
MCC adrenal incidentaloma
bengin vs malig
Definition
benign = non-functional adrenal adenoma
malig = met from separate primary (MC = lung CA)
Term
Adrenal and mets
-MC mets to adrenal?
Definition
lung (MC), breast, melanoma, renal
(adrenal is MC site for mets)?
Term
CT findings suggestive of benign adrenal mass?
Definition
Hypodense (< 10 Hounsfield units, lipid based)
well circumscribed
Term
FNA indications for asymptomatic adrenal mass?
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)
Term
Surgery indications for asympt adrenal mass?
Definition
-greater than 5 cm
-Ominous: complex, hemorrhagic, irregular, heterogen, dense vascular
-functional, enlarging, +FNA
Term
schedule for following asympt adrenal mass?
Definition
CT abd q3 months x 1 year-->then yearly
Term
adrenal cyst mgmt?
Definition
aspirate
-->clear = follow it and resect if it recurs
-->bloody = resection
Term
FNA shows agniomyolipoma
-->mgmt?
Definition
benign..leave it alone
Term
Dx of cushings syndrome?
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
Term
most sensitive test for cushing?
Definition
24 hr urine free cortisol
Term
use of NP-59 scintigraphy in cushing dx?
Definition
is taken up by adrenal adenomas, but not CA
-ddx hyperplasia vs adenoma
Term
MCC Cushing?
Definition
iatrogenic = MCC overall
Cushing disease = pituitary adenoma = MCC non-iatrogenic cushings syndrome
Term
tx of cushing DISEASE?
Definition
-trans-sphen rsxn-->XRT for residual tumor
-Possible bilateral adrenalectomy (medically with mitotane) if cant find pit adenoma
Term
pt with cushings disease but cant find pit source?
Definition
consider bilateral adrenalectomy
-surgically vs medically w/mitotane
Term
what needs supplementation after trans-spehnoidal cushings disease surgery vs bilateral adrenalectomy?
Definition
-trans sphen: 3 years of glucorcorticouds
-bilat adrenal: life long glucocort = mineralocort
Term
MC source of extra-pit cushing syndrome?
Definition
small cell lung CA
(not suppressed with high dose dexameth test)
Term
Imaging options to localize Ectopic ACTH secreting tumor?
Definition
CT
Somatostatin scan
Term
Top 3 causes of non-iatrogenic cushing syndrome
Definition
1 = pit adenoma
2 = ectopic tumor (MCC Small cell lung)
3 = adrenal adenoma (low ACTH)
Term
Adrenocortical carcinoma
-MC age?
Definition
bimodal (before age 5, after 5th decade)
Term
Adrenocortical carcinoma
-% functional
-% advanced at dx/
Definition
50% functional
80% advanced
Term
Adrenocortical carcinoma
-sxs
Definition
-90% virilization of females /precocc puberty @ males,
-feminization in men, masculization in women can occur
-HTN, abd pain, weight loss, weakness
Term
adrenocortical Ca
-tx
Definition
Radical adrenalectomy (takes kidney)
-debulking can inc survival
-mitotane post op = inc disease free survival
Term
adrenocortical Ca 5Ysr
Definition
20%
Term
Medical tx options for cushing syndrome?
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)
Term
indications for bilateral adrenalectomy?
Definition
Unresectable ectopic ACTH tumor (if slow growing)
Pit adenoma that cant be found
bilateral adrenal hyperplasia (medical first)
Term
difference between gluco and mineralocorticoid
Definition
?
Term
origin of pheo cells?
Definition
ectopic NCC or sympathetic ganglia
Term
MC location of pheo?
Definition
MC right adrenal medulla
Term
10% rule of pheo = ?
Definition
10% are.. malignant, bilateral, pediatric, familial, extra-adrenal, a/w MEN
Term
what type of pheo is most likely malignant?
Definition
extra-adrenal pheo (25%)
Term
What genetic syndromes have pheos?
Definition
MEN 2A/B
VHL, NF type 1, Tuberous sclerosis, Sturge Weber, familial paragnanlioma
Term
Pheo dx
-highest specificity test?
Definition
24 hour urine- Epi, NE, VMA, metanephrines/normeta
-98% specificity
-VMA is the MOST specific
Term
Clonidine suppression test
-role in pheo dx?
Definition
tumor does not respond to clonidine suppression
-still have high catacholamines
Term
work-up for localizing pheo?
Definition
1. CT-->if fails...
2.MIBG scan (131 meta iodobenzylguanidine) = NE analogue
3.-->if still cant find it==> exlap
Term
What test should not be done to locate a pheo?
Definition
No venography-->causes HTN crisis
Term
pre-op prep for pheo?
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
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
Term
extra-adrenal sites of pheo?
Definition
MC = aortic bifurcation
Vert bodies, paravertebral symp ganglion, contralat adrenal, bladder
Term
inra-op trouble shooting vs pheo resection
1. hypertension
2. persistent htn after rsxn
3. hypotension after removal
4. bronchospasm after removal
Definition
1. esmolol, nipride
2. check other sites (10% are bilateral)
3. volume first-->then phenylephrine
4. albuterol, epinephrine
Term
Drug that inhibits Tyr OHylase?
Definition
Metyrosine
= dec catachol production
Term
what causes false positive VMA ?
Definition
caffeine, fruits, vanilla, iodine, alpha/beta blockers
Term
Conn syndrome
1. =
2. general lab trends?
Definition
primary hyper-aldosteronism
-inc aldost, dec renin
Term
MCC Conn syndrome?
Definition
1. Adenoma (80% = MCC)
2. adrenal hyperplasia
3. adrenocortical CA (<1 %)
4.ovarian tumors
Term
secondary hyper-aldosteronism
-frequency vs primary hyper-ald
-causes?
Definition
-more frequent
-primary reninism (renin tumor), 2ndary reninism (several causes)
Term
sxs primary hyperaldosteronism
Definition
HTN (2/2 Na retention but WITHOUT edema)
HypoK+, polydipsia, polyuria, weakness
--HTN WITH HYPOKALEMIA---
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)
Term
Localizing studies for Conn syndrome lesion
Definition
1. Abd CT/MRI
2. selective adrenal venous sampling for aldosterone
Term
Primary hyperaldost
tx
Definition
Adenoma = adrenalectomy
Diffuse hyperplasia:
-spironolactone, CCBlk, eplerenone (aldost antag), anti-HTNs
-K supp
Last resort = bilat adrenalectomy
Term
MC indication for bilateral adrenalectomy @ Conn?
Definition
refractory hypokalemia
-need fludro and hydrocortisone post op
Term
MCC hypoaldosteronism
Definition
Renal dysfunction 2/2 DM nephropathy
Term
3 types of HYPOaldost?
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)
Term
Tx of hypoaldosterone ?
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
Term
Sxs acute adrenal insuff?
Definition
fever, N/V, abd pain, HYPOtn unresponsive to resusc, AMS, hypoglycemia, diarrhea
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)
Term
Addisons dz
1. =
2. sx
Definition
= chronic adrenal insuff
sx: weakness, weight loss, abd pain, HYPERPIG 2/2 MSH (ACTH breakdown product)
Term
MCC addisons dz?
Definition
primary adrenal DZ : autoimmune MC
-TB, fungal, adrenal mets, pituitary dz
Term
Dx of addisons?
Definition
Inc ACTH.
Dec cortison, dec aldosterone
Lytes: HyperK, hyperGlycemic, hypoNa
Term
Tx addisons
Definition
Hydrocort, fludrocort
(use dexamethasone while doing ACTH stim test->measure cortisol)
Term
Waterhoue freidrichsen synd MCC org
Definition
n meningitides
Term
VHL findings
Definition
Hemangioblastoma (cerebellar, spinal cord, retina)
Renal angioma, RCCarcinoma
Pheo
Term
Carney Complex
-findings
Definition
1. Myxoma @ heart and skin + hyperpig (lentiginosis)
2. Endocrine over-activity (ex: cushings)
(cause = PR-KAR tumor suppressor)
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