Adrenals II Flashcards

1
Q

what compounds does the adrenal Cx make that can be converted to testosterone

A

dehydroepiandrosterone and adrostenedione

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2
Q

what occurs with a 21-hydroxylase deficiency

A

increased secretion ACTH–> adrenal hyperplasia and increased testosterone

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3
Q

mutation in CYP21A2 causes what

A

21-hydroxylase deficiency

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4
Q

what syndromes wil a 21-hydroxylase deficiency cause

A
  • salt wasting, classic, adrenogenitalism
  • simple virilizing adrenogenitalism
  • nonclassic adrenogenitalism
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5
Q

in complete deficiency of 21 hydroxylase there is no synthesis of what

A

mineralocorticoids and cortisol synthesis

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6
Q

when is complete deficiency for salt wasting syndrome diagnosed

A

soon after birth

in utero the electrolytes and fluids can be maintained by maternal kidneys

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7
Q

what are Sx of salt wasting syndrome

A

hyponatremia, hyperkalemia which induce acidosis , hypotension, CV collapse and maybe even death

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8
Q

what is simple virilizing androgenital syndrome without salt wasting

A

hace enough mineralocorticoids
but lowered glucocorticoid fail to inhibit ACTH secretion
increased testosterone

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9
Q

14 y.o with hirsuitism acne and menstrual irregularities

A

nonclassic, late onset adrenal virilism

partial deficiency in 21 hyrdoxylase

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10
Q

adrenals b/l hyperplastic with brown Cx with no lipid and hyperplasia in anterior pituitary

A

congenital adrenal hyperplasia

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11
Q

what are the Sx of 21 hydroxylase deficiency in infants

A

clitoral hypertrophy, pseudohermaprhoditism

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12
Q

what are the sx of 21 hydroxylase deficiency in postpubertal females

A

oligomenorrhea, hirsuitism and acne

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13
Q

how does 21 hydroxylase deficiency present in males

A

enlargement of the external genitalia and other evidence of precosious pubertal patients
oligoseprmia in males

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14
Q

neonate with ambiguous genitalia, what must be on ddx?

A

CAH

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15
Q

CAH are what type inhertiance disorders

A

auto recessive

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16
Q

reduction cortisol causes what compensatory mech

A

increase ACTH which stimulates androgens

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17
Q

what are they types of adrenocortical insufficiency

A

primary acute adrenocortical insufficiency
primary chronic adrenocortical insuffciency (addisons)
secondary adrenocortical insufficiency

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18
Q

what are main causes of loss of cortical cells in adrenal glands

A

autoimmune
autoimmune polyendocrinopathy syndrome 1 and 2
infection : TB and fungi and AIDS
metastatic carcinoma

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19
Q

what is an adrenal crisis

A

immediate increase in steroid output from glands

20
Q

what can cause adrenal crisis

A
  • someone with chronic insufficiency precipitated by stress
  • rapid withdrawl steroids
  • failure to increase steroid doses
  • massive adrenal hemorrhage (newborns with difficult delivery)
21
Q

what is waterhouse-friderichsen syndrome

A

DIC with hemorrhagic infarction of adrenals

22
Q

what bacteria can cause waterhouse friderichesen syndrome

A

N meningitidis, pseudomonas species, pneumococci, haemophilus influenzae or even staphylococci

23
Q

What is a clincal sign of DIC

A

widespread purpura

24
Q

what is the most common cause of primary adrenal insufficiency

A

autoimmune adrenalitis

25
Q

What are the causes of autoimmune adrenalitis

A

APS1 and aPS2

26
Q

What infections cause chronic adrenocortical insufficiency

A

TB and fungi

27
Q

what cancers commonly mets to adrenals

A

carcinomas of lung and breast

28
Q

granulomatoud inflammation in adrenals

A

TB or fungi caused adrenalitis

29
Q

how would you expect adrenals to look in case of metastatic carcinoma causing hypoadrenalism

A

enlarged and normal architecture obscured by infiltrating neoplasm

30
Q

when does addisons come to attention

A

when circulating glucocorticoids and mineralocorticoids are significantly decreased

31
Q

initial cc of addisons

A

progressive weakness and easy fatigability

32
Q

GI disturbances in addisons

A

anorexia, nausea, vomiting, weight loss and diarrhea

33
Q

hyperpigmentation of neck, elbows, knees, knuckles

A

primary adrenal disease

increased POMC/ACTH

34
Q

electrolytes of someone with primary adrenal insufficiency

A

potassium retention and Na loss

hyperkalemia, hyponatremia, volume depleion and hypotension

35
Q

APS1 characterized by

A

autoimmune attack against endocrin organs and autoAb against IL-17

36
Q

reduced output ACTH leads to a syndrome that mimics what

A

syndrome of hypoadrenalism that mimics addisons

37
Q

characterizations of secondary hypoadrenalism

A

deficient cortisol and androgen output, normal or near normal aldosterone synthesis (aka no electrolyte disturbances)

38
Q

adrenals in secondary hypoadrenalism

A

markedly decreased in size
flattened glands that are yellow
medulla unaffected

39
Q

in children what are the more common adrenocortical neoplasms

A

carcinomas

40
Q

what are the familial cancer syndromes associated with adrenocortical carcinomas

A

Li-Fraumeni synfrome germiline TP53 mutation

Beckwith-Wiedemann syndrome- defect in epigenetic imprinting

41
Q

Virilizing neoplasms are most likley

A

carcinomas

42
Q

fucntional adrenal adenomas are assoc with

A

hyperaldosteronism and cushing syndrome

43
Q

incindentalomas

A

adrenocortical adenomas

yellow from lipids

44
Q

what carcinomas mets to adrenals

A

bronchogenic origin(smokers)

45
Q

outcome adrenal incindentalomas

A

no clinical importance

follow every 6 mo