Adrenals II Flashcards
what compounds does the adrenal Cx make that can be converted to testosterone
dehydroepiandrosterone and adrostenedione
what occurs with a 21-hydroxylase deficiency
increased secretion ACTH–> adrenal hyperplasia and increased testosterone
mutation in CYP21A2 causes what
21-hydroxylase deficiency
what syndromes wil a 21-hydroxylase deficiency cause
- salt wasting, classic, adrenogenitalism
- simple virilizing adrenogenitalism
- nonclassic adrenogenitalism
in complete deficiency of 21 hydroxylase there is no synthesis of what
mineralocorticoids and cortisol synthesis
when is complete deficiency for salt wasting syndrome diagnosed
soon after birth
in utero the electrolytes and fluids can be maintained by maternal kidneys
what are Sx of salt wasting syndrome
hyponatremia, hyperkalemia which induce acidosis , hypotension, CV collapse and maybe even death
what is simple virilizing androgenital syndrome without salt wasting
hace enough mineralocorticoids
but lowered glucocorticoid fail to inhibit ACTH secretion
increased testosterone
14 y.o with hirsuitism acne and menstrual irregularities
nonclassic, late onset adrenal virilism
partial deficiency in 21 hyrdoxylase
adrenals b/l hyperplastic with brown Cx with no lipid and hyperplasia in anterior pituitary
congenital adrenal hyperplasia
what are the Sx of 21 hydroxylase deficiency in infants
clitoral hypertrophy, pseudohermaprhoditism
what are the sx of 21 hydroxylase deficiency in postpubertal females
oligomenorrhea, hirsuitism and acne
how does 21 hydroxylase deficiency present in males
enlargement of the external genitalia and other evidence of precosious pubertal patients
oligoseprmia in males
neonate with ambiguous genitalia, what must be on ddx?
CAH
CAH are what type inhertiance disorders
auto recessive
reduction cortisol causes what compensatory mech
increase ACTH which stimulates androgens
what are they types of adrenocortical insufficiency
primary acute adrenocortical insufficiency
primary chronic adrenocortical insuffciency (addisons)
secondary adrenocortical insufficiency
what are main causes of loss of cortical cells in adrenal glands
autoimmune
autoimmune polyendocrinopathy syndrome 1 and 2
infection : TB and fungi and AIDS
metastatic carcinoma
what is an adrenal crisis
immediate increase in steroid output from glands
what can cause adrenal crisis
- someone with chronic insufficiency precipitated by stress
- rapid withdrawl steroids
- failure to increase steroid doses
- massive adrenal hemorrhage (newborns with difficult delivery)
what is waterhouse-friderichsen syndrome
DIC with hemorrhagic infarction of adrenals
what bacteria can cause waterhouse friderichesen syndrome
N meningitidis, pseudomonas species, pneumococci, haemophilus influenzae or even staphylococci
What is a clincal sign of DIC
widespread purpura
what is the most common cause of primary adrenal insufficiency
autoimmune adrenalitis
What are the causes of autoimmune adrenalitis
APS1 and aPS2
What infections cause chronic adrenocortical insufficiency
TB and fungi
what cancers commonly mets to adrenals
carcinomas of lung and breast
granulomatoud inflammation in adrenals
TB or fungi caused adrenalitis
how would you expect adrenals to look in case of metastatic carcinoma causing hypoadrenalism
enlarged and normal architecture obscured by infiltrating neoplasm
when does addisons come to attention
when circulating glucocorticoids and mineralocorticoids are significantly decreased
initial cc of addisons
progressive weakness and easy fatigability
GI disturbances in addisons
anorexia, nausea, vomiting, weight loss and diarrhea
hyperpigmentation of neck, elbows, knees, knuckles
primary adrenal disease
increased POMC/ACTH
electrolytes of someone with primary adrenal insufficiency
potassium retention and Na loss
hyperkalemia, hyponatremia, volume depleion and hypotension
APS1 characterized by
autoimmune attack against endocrin organs and autoAb against IL-17
reduced output ACTH leads to a syndrome that mimics what
syndrome of hypoadrenalism that mimics addisons
characterizations of secondary hypoadrenalism
deficient cortisol and androgen output, normal or near normal aldosterone synthesis (aka no electrolyte disturbances)
adrenals in secondary hypoadrenalism
markedly decreased in size
flattened glands that are yellow
medulla unaffected
in children what are the more common adrenocortical neoplasms
carcinomas
what are the familial cancer syndromes associated with adrenocortical carcinomas
Li-Fraumeni synfrome germiline TP53 mutation
Beckwith-Wiedemann syndrome- defect in epigenetic imprinting
Virilizing neoplasms are most likley
carcinomas
fucntional adrenal adenomas are assoc with
hyperaldosteronism and cushing syndrome
incindentalomas
adrenocortical adenomas
yellow from lipids
what carcinomas mets to adrenals
bronchogenic origin(smokers)
outcome adrenal incindentalomas
no clinical importance
follow every 6 mo
