Pathology of the Adrenal I Flashcards
glucocorticoid production
zona fasciculata
cortisol
mineralocorticoid production
zona glomerulosa
aldosterone
sex steroid production
zona reticularis
estrogens and androgens
chromaffin cells
in adrenal medulla
secrete catecholamines
epinephrine
cushings syndrome
hypercortisol
central obesity, moon facies, abdominal striae
cushings
elevated glucocorticoid levels
cushing
exogenous and endogenous
exogenous cushings
iatrogenic
admin of exogenous glucocorticoids
endogenous cushings
ACTH dependent
ACTH independent
ACTH dependent cushings
pituitary adenoma - producing ACTH
ectopic ACTH
small cell lung cancer
but rare
ACTH independent cushings
adrenal adenoma or carcinoma
elevated cortisol and low ACTH
majority of endogenous hypercortisolism
ACTH secreting pituitary adenoma
cushing disease
ACTH producing pituitary adenoma
more in women - young adults
most common cause of ACTH-indepenent cushing syndome
primary adrenal neopalsms
adenomas and carcinomas
crooke hyaline change
pituitary adenoma
-producing ACTH
cushings disease
adrenal cortex color
yellow
HTN and weight gain
seen early with cushings syndrome
buffalo hump
cushings
hypercortisol and muscle
atrophy of type 2 myofibers
decreased muscle mass and proximal limb weakness
striae in cushings
glucocorticoids induce gluconeogenesis - hyperglycemia, glucosuria, polydipsia - secondary diabetes
loss of collagen and bone resorption
skin thin fragile and easily bruised - striae occurs
lady stops menstruating
overweight, HTN, striae, increased cortisol
cushings
decreased libido
in cushings
lab diagnosis of cushings
24 hour urine free cortisol increased
loss of normal diurnal (high morning/low afternoon) pattern of cortisol release
dexamethasone suppression test
dexamethasone - suppresses ACTH secretion
in pituitary adenoma - low dose will not work, but high dose will
in ectopic ACTH or adrenal tumor low and high dose will not work
ectopic - high ACTH
adrenal tumor - low ACTH
ectopic ACTH vs. adrenal tumor
ectopic - high ACTH
adrenal tumor - low ACTH
primary hyperaldosteronism
excess aldosterone secretion
may be primary or secondary
decreased renin activity
common symptom in primary hyperaldosteronism
BP elevation
due to too much Na
conns syndrome
primary hyperaldosteronism
adrenal aldosteronoma
solitary aldosterone secreting adenoma
-surgery
bilateral adrenal hyperplasia
primary adrenal hyperaldosteronism
tx - spironolactone
CYP11B2
chromosome 8
altered in glucocorticoid remediable hyperaldosteronism
most common primary hyperaldosteronism
idiopathic - bilateral enlargement
tx - medical - spironolactone
secondary hyperaldosteronism
with increased plasma renin
1 - renal artery stenosis
2 - arterial hypovolemia and edema
3 - pregnancy
renal artery stenosis
decreased renal perfusion
- increased renin
- secondary hyperaldosteronism
spironolactone bodies
in aldosterone producing adenoma
hyperaldosteronism clinical
hypertension
long term - left ventricular hypertrophy and reduced diastolic volume
strokes and MI
diagnosis of primary hyperaldosteronism
elevated ratio of plasma aldosterone to plasma renin
confirmatory aldosterone suppression test must be performed**
virilization
development of sex differences in male and female
adrenal causes of androgen excess
adrenocortical neoplasms
congenital adrenal hyperplasia
two compounds from adrenal cortex that can be converted to testosterone
dehydroepiandrosterone
androstenedione
21 hydroxylase deficiency
too much testosterone formation
female gets hairy
congenital adrenal hyperplasia
auto recessive
deficiency or total lack of enzymes involved in biosynthesis of cortical steroids
mutation in CYP21A2
21 hydroxylase deficiency
main cause of congenital adrenal hyperplasia**
3 adrenogenitalism syndromes:
1 - salt wasting - classic
2 - virilizing
3 - nonclassic
salt wasting syndrome
congenital adrenal hyperplasia
-total lack of 21 hydroxylase
no synthesis of mineralocorticoids
also deficient cortisol
excess androgens - virilization occurs
male - unrecognized
a birth - see a salt wasting crisis**
higher K/Na ratio
severe tetany
simple virilizing adrenogenital syndrome
genital ambiguity
1/3 pt with 21-hydroxylase deficiency
enough mineralocorticoids to prevent salt wasting crisis
increased testosterone - progressive virilization
salt wasting crisis at birth**
21 hydroxylase deficiency
- congeintal adrenal hyperplasia
- salt wasting syndrome
produce no mineralocorticoids
late onset adrenal virilism
nonclassic congenital adrenal hyperplasia
partial 21 hydroxylase deficiency
hirsutism, acne, menstrual irregularity
nonclassic - adrenal virilism
partial deficiency of 21 hydroxylase
all cases of congenital adrenal hyperplasia
adrenals are bilaterally hyperplastic
congeintal adrenal hyerplasia in males
21 hydroxylase deficiency
-excessive androgens
enlarged external genitalia and other evidence of precocious puberty
older males - oligospermia
ambiguous genitalia
suspect congneital adrenal hyperplasia
