-
arterial supply to adrenal
-
all zones have what enzymes
21 and 11 beta hydoxylase
-
glomerulosa secretes what
aldosterone
-
steroid pathway
cholesterol-progesterone-androgens/cortisol/aldosterone
-
lymphatics of adrenal gland drain where
subdiaphragmatic and renal
-
adrenal cortex derived from what embryonic layer
mesoderm
-
enzyme only found in medulla
phenylethanolamine
-
VMA is a biproduct of
normetanephrine and metanephrine
-
mcc of ambiguous genitalia
21 hydroxylase def
-
21 hydroxylase def results in an increase in what hormones
17-OH Progesterone and testosterone
-
11 hydroxylase def results in an increase in
11 deoxycortisone and testosterone
-
hormones increased in 17 hydroxylase def
progesterone and pregnenolone
-
sxs/characteristics of 17 hydroxylase def
- HTN
- ambiguous genitalia in males at birth
- females miss puberty if estrogens not given to induce
-
diagnostic w/u for malignant HTN
- bmp (bun/cr)
- 24 hr urine of epi, ne, vma meta/normetanephrine and cortisol
- serum nephrines, cortisol, aldosterone, renin
- u/s kidneys and carotids
- CT chest/abd/pelvis
-
common mets to adrenal
lung breast melanoma renal
-
chararcteristics which make an adrenal incidentaloma benign appearing
- < 10 hounsfield, lipid based
- well circumscribed
-
FNA indicated for which incidentalomas
- >10 HU
- CT washout < 50% at 10min
- young age
-
surgery indicated for what adrenal masses
- > 5cm
- ominous characteristics
- functional tumor
- enlarging
-
what are some ominous characteristics
- complex
- hemorrhagic areas, irregular margins, heterogeneous
- dense
- vascular
-
most sensitive for cushing syndrome
24hr urine free cortisol
-
next step if 24hr urine cortisol is high but ACTH is low
get abd ct
-
how can you differentiate hyperplasia from adenoma
NP-59 scintigraphy
-
#2 noniatrogenic cause of cushings
ectopic
-
sxs of adrenocortical CA
virilization and precocious puberty in children
feminization and masculinization, HTN abd pain, weight loss in adults
-
inhibits cholesterol formation
aminogluethimide
-
medical agents used in adrenal pathology
- metyrapone
- aminoglutethimide
- ketoconazole
- mitotane
-
-
10% rule
- extraadrenal
- familial or MEN
- children
- cancer
- bilateral
-
genetic syndromes with pheo
- MEN 2A/B
- vHL
- NF 1
- familial paraganglioma
- tuberous sclerosis
- Sturge-Weber
-
MIBG is an analogue of
norepinephrine
-
metabolite that is most specific
VMA
-
other sites to look for pheo
- aortic bifurcation
- sympathetic chain
- vertebral bodies
- bladder
- opposite adrenal
-
tx HTN during removal
esmolol or nipride
-
syndrome associated with increase secretion of renin
Bartters
-
next step once lab values characteristic of hyperaldosteronism
- aldosterone to renin ratio
- if 15-20:1 then proceed with
- salt suppression test
-
mcc of hypoaldosteronism
diabetic neuropathy causing hypo-reninemic hypolaldosteronism
-
what sign distinguishes chronic versus acute adrenal insufficiency
hyperpigmentation
-
characteristics of vHL
- hemangioblastomas of cerebellum/spinal cord or retina
- renal angioma or renal cell CA
- pheo
-
what characterizes the carney complex
- PR-KAR tumor suppressor
- myxomas
- skin hyperpigmentation
- endocrine overactivity
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