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eLCRS - 엔도 > Endo 7: Hypoadrenal Disorders > Flashcards

Endo 7: Hypoadrenal Disorders Flashcards

1
Q

What does P450 enzyme do?

A

performs Side chain cleavage

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2
Q

how is cholesterol converted to cortisol + aldosterone ?

A
  • start with cholesterol
  • (in adrenal cortex) –> pregnenolone
  • put OH group onto 17/21/11
    –> forms cortisol
    OR
  • if you put OH group onto 21/11/18 –> forms aldosterone
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3
Q

glucocorticoid synthesis enzymes + sex steroid synthesis enzymes = turned on by _____

A

glucocorticoid synthesis enzymes + sex steroid synthesis enzymes = turned on by ACTH

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4
Q

if pituitary detects stress –> _____ is produced

which turns on enzymes so you produce more ______

A

if pituitary detects stress –> ACTH is produced

which turns on enzymes so you produce more cortisol

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5
Q

how does cortisol production differ In the aldosterone pathway?

A
  • renin + ATII turns on enzymes in the mineralocorticoid pathway –> to produced more cortisol
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6
Q

List common causes of adrenocortical failure

A
  • Tuberculosis Addison’s Disease (Most common)
    (TB grows in adrenal glands)
  • Autoimmune addison’s disease (most common in uk)
  • congenital adrenal hyperplasia
    (missing enzymes, lower level of cortisol –> so adrenal glands grow)
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7
Q

What happens in autoimmune addison’s disease?

A
  • the immune system makes a mistake –> and wipes out the adrenal gland
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8
Q

What happens in congenital adrenal hyperplasia?

A
  • can be complete/ partial
  • caused by enzyme deficiency (commonly 21-hydroxylase deficiency) –> so adrenals can’t make hormones properly
  • born with big adrenals
  • adrenals can still make sex steroids due to overflow of 17 hydroxyprogesterone –> but can’t make anything else
  • so cortisol + aldosterone = deficient
  • testosterone = in excess (So if female, they would have abnormalities of the genitals)
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9
Q

List features/ symptoms of addison’s disease.

A
  • pigmentation in mouth
  • darker hair
  • patches of vitiligo
  • hypotension
  • loss of weight
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10
Q

What are some consequences of adrenocortical failure?

A
  • fall in BP
  • loss of salt in urine
  • increased plasma potassium
  • fall in glucose due to glucorticoid deficiency
  • high ACTH –> results in increased pigmentation (MSH)
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11
Q

Why does high ACTH result in increased pigmentation ?

A

POMC –> ACTH + MSH

- MSH = causes increase in pigmentation

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12
Q

POMC = broken down to:

A

POMC –> ACTH + MSH + endorphines + enkephalins + other peptides

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13
Q

how would you treat someone who is unconscious due to addison’s ?

A

inject hydrocortisone

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14
Q

What are some tests for addison’s

What would the results be like in a patient tie addison’s disease?

A
  • cortisol at 9am
  • test ACTH level
  • short aynACTHen test
  • cortisol at 9am = LOW e.g 100
  • short aynACTHen test = LOW e.g 150
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15
Q

Describe the presentation of congenital adrenal hyperplasia

A
  • hypotension

- virilisation (development of male physical characteristics in a female - AMBIGUOUS GENITALIA)

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16
Q

21 hydroxylase deficiency = recessive / dominant

A

21 hydroxylase deficiency = recessive

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17
Q

what is the difference between partial and complete 21- hydroxylase deficiency?

A
  • same hormones will be low/high but not quite so severely.
  • e.g low cortisol –> but only a little bit hypotensive
  • long period of slightly raised testosterone
18
Q

What happens in 11-beta hydroxylase deficiency ?

A
  • you get build up of 11 deoxycorticosterone
  • -> which is an active aldosterone receptor agonist
  • so child is hypertensive, hypokalemic and virilised
19
Q

What happens in 17-hydroxylase deficiency ?

A
  • you get high levels of aldosterone
  • so they are hypertensive + hypokalaemic
    BUT
  • they are missing cortisol + sex steroids
    –> so they never go through puberty
  • has borderline hypoglycemia

note: they don’t get addisonian crisis because they have aldosterone

20
Q

Which hormones are deficient in 17-hydroxylase deficiency?

A
  • cortisol + sex steroids
21
Q

Which hormones are in excess in 17-hydroxylase deficiency?

A
  • 11 deoxycorticosterone + aldosterone
22
Q

What problems occur in 17 hydroxylase deficiency ?

A
  • hypertension
  • low K
  • sex steroid deficiency
  • glucocorticoid deficiency

(no cortisol, no sex steroids)

23
Q

11-deoxycorticosterone behaves similarly to ________

in excess, it can cause ______ + ________

A

11-deoxycorticosterone behaves similarly to aldosterone

in excess, it can cause hypertension + hypokalemia

24
Q

All steroids come from ________

A

cholesterol

25
Q

note: cholesterol –> pregnenolone –> progesterone

A

-

26
Q

pregnenolone –> progesterone via enzyme _______

A

dehydrogenase

27
Q

How would you treat someone who comes in with falling BP + tan, lethargy?

A
  • give them saline IV
28
Q

What is synACTHen test ?

A
  • take blood sample
  • give 250 ug synacthen IM
    then measure cortisol response
29
Q

What is a normal cortisol level at 9am and 9.30am?

A

at 9am : 270-900

at 9.30 am: >600

30
Q

congenital adrenal hyperplasia is most commonly due to =

A
  • most commonly due to 21-hydroxylase deficiency

note: may be complete/ partial

31
Q

in partial 21 hydroxylase deficiency, which hormones are deficient?

A

cortisol+ aldosterone

32
Q

In martial 21 hydroxylase deficiency, which hormone are in excess

A

sex steroid + testosterone

33
Q

how would you treat 21 hydroxylase deficiency?

A

give them cortisol

34
Q

AT what age might 21 hydroxylase deficinecy present?

A

at any age

35
Q

what is the effect of 21 hydroxylase deficiency on males + females?

A

in males = precocious puberty

in females = hirsutism, clitorial enlargement, small breasts, hyperplasia of adrenal cortex

36
Q

what is the effect of 11 hydroxylase deficiency?

A

high bp
high level of sex steroids
no aldosterone
no cortisol

37
Q

11 deoxycorticosterone acts similarly to _____

A

aldosterone

38
Q

in 11 hydroxylase deficiency, hormone deficient =

A

cortisol

aldosterone

39
Q

in 11 hydroxylase deficiency, hormone in excess=

A

sex steroid, testosterone

40
Q

what is the main problem in 17 hydroxylase deficiency?

A
  • no growth spurt
  • no puberty
  • and also presents with hypertension
41
Q

how would you treat a patient with 11 hydroxylase deficiency?

A
  • give them hormone replacement

e. g testosterone, oestrogen etc.

42
Q

Draw a diagram showing the processes involved in the conversion of cholesterol to

a) aldosterone
b) cortisol
c) sex steroids

A

-

eLCRS - 엔도 (20 decks)

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