Adrenal Disorders Flashcards

1
Q

where are corticosteroids synthesised?

A

adrenal cortex

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

examples of corticosteroids

A
mineralcorticoids (aldosterone)
glucocorticoids (cortisol)
sex steroids (androgens, oestrogen)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

what is the precursor of a steroid?

A

cholesterol

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

what is the effect of angiotensin II on the adrenals?

A

activates:
3-hydroxysteroid dehydrogenase
11, 21, 18-hydroxylase

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

what is the effect of ACTH on the adrenals?

A

activates: 3-hydroxysteroid dehydrogenase

11, 21 and 17-hydroxylase

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

what is addisons disease?

A

primary adrenal failure

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

what is the most common cause of Addisons worldwide?

A

tuberculosis of the adrenal glands

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

what is the most common cause of Addisons in the UK?

A

autoimmune destruction of the adrenal glands

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

what are the main signs of Addisons disease?

A

skin and mucous membrane pigmentation
weight loss
vomiting, diarrhoea
low blood pressure

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

what are the physiological consequences of addisons?

A
low BP
loss of salt in urine
increased plasma potassium 
low blood glucose
increased skin pigmentation
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

why does high ACTH lead to increased pigmentation?

A

POM-C is cleaved to form ACTH and MSH (and endorphins) so pathologically high ACTH may lead to tanned skin

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

what tests should be performed if Addisons is suspected?

A

9am cortisol

short synACTHen test

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

what is a short synACTHen test?

A

give 250ug synACTHen IM

measure cortisol response (should increase)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

how is Addisons treated?

A

fludrocortisone 50-100mg

and either hydrocortisone 3x daily(10, 5, 2.5mg) OR prednisolone 3mg daily

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

what is the most common congenital adrenal hyperplasia?

A

21-hydroxylase deficiency

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

what is complete 21-hydroxylase deficiency?

A

missing 21-hydroxylase from birth

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

what hormones are absent in complete 21-hydroxylase deficiency?

A

aldosterone and cortisol

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

which hormones are in EXCESS in complete 21-hydroxylase deficiency?

A

sex steroids and testosterone

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

what are the signs of someone with complete 21-hydroxylase deficiency?

A

neonate with salt-losing addisonian crisis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

what do girls with complete 21-hydroxylase deficiency develop?

A

ambiguous genitalia, virilised by adrenal testosterone (males usually okay)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

how do partial and complete 21-hydroxylase deficiency present differently?

A

partial may present much later in life as is not fatal without treatment. males may present with precocious puberty and females with hirsutism and virilisation

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

what is virilisation?

A

development of male physical characteristics in females/precocious boys

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

what hormones are deficient in 11-hydroxylase deficiency?

A

cortisol and aldosterone

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

what hormones are in excess in 11-hydroxylase deficiency?

A

sex steroids - testosterone

11-deoxycortisterone

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
Q

what do individuals with 11-hydroxylase deficiency present with?

A

virilisation,
HYPERtension
HYPOkalaemia

26
Q

what does 11-deoxycorticosterone act like?

A

aldosterone - excess can cause hypertension and hypokalaemia

27
Q

what hormones are deficient in 17-hydroxylase deficiency?

A

cortisol and sex steroids

28
Q

what hormones are in excess in 17-hydroxylase deficiency?

A

11-deoxycorticosterone and aldosterone (mineralocorticoids)

29
Q

what do individuals with 17-hydroxylase deficiency present with?

A
hypertension, 
hypokalaemia, 
infertility, 
low libido, 
erectile dysfunction 
low glucose
30
Q

what is cushings syndrome?

A

high cortisol

31
Q

what are the main signs of cushings disease?

A
centripedal obesity, moon face, buffalo hump
proximal myopathy
hypertension and hypokalaemia
red striae, thin skin, bruising
osteoporosis, diabetes
32
Q

what is the main cause of cushings syndrome?

A

taking steroids
pituitary adenoma
ectopic ACTH lung cancer
adrenal adenoma

33
Q

what is cushings disease?

A

cushings syndrome caused by pituitary adenoma

34
Q

how is cushings syndrome diagnosed?

A

24hr urine collection for urinary free cortisol
blood diurnal cortisol levels
low dose dexamethasone suppression test

35
Q

what is a low dose dexamethasone test?

A

0.5mg 6 hourly doses of artificial steroid for 48hrs

any cause of cushings will fail to suppress cortisol (normal should go to 0)

36
Q

how do you treat cushings?

A

metyrapone or ketoconazol medication
pituitary surgery
bilateral or unilateral adrenalectomy

37
Q

what is metyrapone used for?

A

control of cushings prior to surgery (promotes better wound healing)
control of cushings after radiotherapy (while waiting for it to work)

38
Q

what are the side effects of metyrapone?

A

hirsutism

hypertension if long term

39
Q

what is ketoconazole used for?

A

mainly an antifungal,

at high doses - control and treatment of cushings syndrome

40
Q

what is ketoconazoles mechanism of action?

A

inhibits steroidogenesis by 17a-hydroxylase

41
Q

when is a bilateral adrenalectomy typically performed?

A

for ectopic ACTH lung cancer

42
Q

when is a unilateral adrenalectomy typically performed?

A

for adrenal masses

43
Q

what is Conns syndrome?

A

benign adrenal cortical tumour of zona glomerulosa
with hyperaldosteronaemia
aka primary hyperaldosteronism

44
Q

how does Conns syndrome present?

A
hypertension
muscle cramps
heart palpitations
hypokalaemia
anxiety
headaches
45
Q

how is conns syndrome diagnosed?

A
measuring renin (low) and aldosterone levels (high)
low potassium (hypokalaemia)
46
Q

what is spironolactone used for?

A

primary hyperaldosteronism (conns syndrome)

47
Q

how does metyrapone work

A

blocks 11-hydroxylase
therefore no production of cortisol or corticosterone
side effects casued by buildup of 11-deoxycorticosterone in zona glomerulosa - hypertension

48
Q

treatment of conns syndrome

A

spironolactone (or epleronone)

and removal of tumour

49
Q

spironolactone side effects

A

gynaecomastia

menstrual abnormalities

50
Q

what is a phaeochromocytoma?

A
tumour of adrenal medulla
neuroendocrine cells
secretes catecholamines (nor/adrenaline)
51
Q

how is phaeochromocytoma different to other adrenal tumours?

A

neuroendocrine cells
it stores the adrenaline then suddenly releases it unlike a steady release of hormones
therefore symptoms can come on very suddenly - blood pressure rises etc

52
Q

signs of phaeochromocytoma

A

intermittent severe hypertension - especially after abdominal palpation
hypertension in young

53
Q

risks due to phaeochromocytomas

A
myocardial infarction
stroke
ventricular fibrillation
sudden death
medical emergency!
54
Q

management of phaeochromacytoma

A

alpha adrenoreceptor blocker and give fluids to prevent a bp crash
beta blocker to prevent tachycardia
then removal of tumour surgically

55
Q

types of phaeochromocytoma

A

extra-adrenal (somewhere in sympathetic chain)
bilateral
malignant
heritable

56
Q

what is a salt losing addisonian crisis

A

body unable to retain salt (sodium) as its lost in urine

crash in blood pressure

57
Q

what should you suspect if a baby is born with ambiguous genitalia

A

21 hydroxylase deficiency - give NaCl fluids just in case of crisis

58
Q

practice drawing the adrenal steroid pathway on paper RN

A

you better have done it

59
Q

treatment of 21-hydroxylase deficiency

A

replacement for aldosterone and cortisol so

single morning dose of prednisolone 3mg

60
Q

symptoms of phaechromocytoma

A
severe intermittent hypertension
heavy sweating
palpitations and tachycardia
shortness of breath 
panic-attack like symptoms
61
Q

easy ways to remember CAHs

A

If the enzyme deficiency starts with 1 (eg. 11 or 17 hydroxylase) → it causes hypertension.

If the enzyme deficiency ends with 1 (eg. 11 or 21 hydroxylase) → it causes virilisation in females and precocious puberty in males

  • In each of these conditions, the absence of an enzyme results in decreased cortisol, which causes:
    • Hyperpigmentation (due to decreased negative feedback → elevated ACTH → increased MSH from POMC breakdown)
    • Hypoglycaemia (because one of the functions of cortisol is to promote gluconeogenesis)
    • Nausea and vomiting (mechanism unclear but this is a classic symptom of adrenal insufficiency)