Adrenals II Flashcards
(45 cards)
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
