Hyperadrenal disorders Flashcards

1
Q

what are the clinical features of Cushing’s Syndrome?

A
  • hypernatraemia
  • hypokalaemia
  • high cortisol

fat:
- centripetal obesity
- moon face
- buffalo hump
- proximal myopathy
skin:
- red striae
- thin skin and bruising
metabolic:
- osteoporosis
- diabetes
- hypertension

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

what are the causes of Cushing’s Syndrome?

A

ACTH dependent:

  • pituitary dependent Cushing’s Disease
  • ectopic ACTH (lung cancer) secretion

ACTH independent:

  • too many steroids (exogenous)
  • adrenal adenoma producing cortisol
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

what are the investigations to determine the cause of Cushing’s?

A

1) urinary free cortisol (24 hr)
2) blood diurnal (depending on time of day) cortisol analysis
3) low-dose dexamethasone suppression test

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

what results can you get in the blood diurnal cortisol test?

A

normal= cortisol is high in the morning and low in the night

Cushings= cortisol remains high all the time

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

what is the dosage given for the dexamethasone suppression test?

A

0.5 mg in 6 hours periods over 48 hours

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

what results can you getting the dexamethasone supression test?

A

normal= dexamethasone suppresses cortisol and it falls to 0 due to feedback inhibition

Cushings= any cause of Cushing’s will fail to suppress the cortisol

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

what drugs can be given to treat Cushing’s?

A

enzyme inhibitors
receptor blocking drugs

e.g metyrapone, ketoconazole

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

what type of surgery can be done to treat Cushing’s

A

pit. surgery
bilateral adrenalectomy
unilateral adrenalectomy

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

what is the mechanism of action of metyrapone

A

reduces cortisol production: inhibits 11 alpha hydroxylase

prevents the production of corticosterone from deoxycorticosterone and therefore cortisol

raises ACTH secretion due to feedback
process has been arrested at 11-deoxycortisol (precursor to cortisol) which has no feedback effect

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

what are the uses of metyrapone?

A
  • prior to surgery
  • dose is adjusted to cortisol
  • improve patients symptoms
  • promotes post-op recovery
  • used to control Cushing’s after radiotherapy
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

what are the two unwanted actions of metyrapone

A

1) hypertension on long term administration

2) hirsutism with androgen production

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

why does metyrapone cause hypertension

A

deoxycortisol accumulates in the zona glomerulosa
it has aldosterone-like activity
leads to salt retention and hypertension

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

how is ketoconazole used to treat Cushing’s

A

in high concentrations it inhibits steroidogenesis

controls symptoms prior to surgery and is orally active

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

what is the unwanted effect of ketoconazole?

A

liver damage

SEP too significant to be used anymore

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

what is Conn’s Syndrome?

A

benign adrenal (Z.glomerulosa) tumour

producing excess aldosterone

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

what are the consequences of Conn’s?

A

hypertension

hypernatraemia and hypokalaemia

17
Q

what causes hypertension and therefore hypernatraemia and hypokalaemia in Conn’s?

A

due to water retention caused by aldosterone

enhancing sodium reabsorption (in exchange for potassium which is then excreted)

18
Q

what are the diagnoses of Conns?

A
  • young person with hypertension
  • primary hyperaldosteronism (high steroids)
  • RAS should be suppressed to exclude secondary hyperaldosteronism
  • high sodium
  • low potassium
19
Q

what is the treatment of Conn’s?

A
  • aldosterone receptor antagonists e.g. spironolactone

- surgical resection

20
Q

what is the mechanism of action of spironolactone?

A

converted to several metabolites e.g. canrenone
becomes a competitive inhibitor of MR

blocks Na reabsorption and K excretion (potassium sparing diuretic)

21
Q

describe the pharmacokinetics of spironolactone

A

aldosterone receptor antagonist
orally active
highly protein bound
metabolised in the liver

22
Q

what are the unwanted actions of spironolactone?

A

menstrual irregularities (progesterone receptor)

gynaecomastia (androgen receptor)

23
Q

why is eplerone a better alternative to spironolactone?

A

MR antagonists

similar affinity to MR as spironolactone
less binding to androgen and progesterone receptors therefore better tolerated than spironolactone

24
Q

what is a phaeochromocytoma?

A

tumour of the adrenal medulla
secretion of catecholamines (adrenaline and noradrenaline)

medical emergency
more common in certain inherited conditions

25
Q

what are the clinical features of phaeochromocytoma?

A
  • hypertension in young people
  • episodic severe hypertension in older people (after abdominal palpitation to squeeze out more adrenaline)
  • double vision
  • chest pain, palpitations
  • sweating
26
Q

how is Conn’s hypertension different to that in phaechromocytoma

A

Conn’s hypertension is not episodic

27
Q

what are the consequences of severe hypertension in phaec

A

MI
stroke
ventricular fibrillation
death if not treated

28
Q

precautions when treating phaec

A

the patient requires surgery but needs careful prep as anaesthesia can precipitate a hypertensive crisis

29
Q

what is the first step treatment of phaechromocytoma?

A

1) alpha blockade (alpha receptors) –> prevent reflex tachycardia

IV fluids needed for the associated BP drop
beta blockade to prevent tachycardia

30
Q

what are the 4 different origins of phaechromocytoma?

A

10% extra-adrenal (in sympathetic chain)
10% malignant
10% bilateral
10% familial

but very rare

31
Q

Most common variety of congenital adrenal hyperplasia

A

21 hydroxylase deficiency

Congenital adrenal hyperplasia is generally caused by a deficiency in adrenal enzymes

32
Q

when is surgery for phaecochromocytoma done?

A

Surgery is only done after the alpha and beta blockers are in full effect

alpha blocker for reflexive tachycardia

33
Q

First therapeutic step in phaeochromocytoma

A

Administration of alpha blockade

34
Q

Aldosterone receptor antagonists and agonist examples

A

Antagonist- spironolactone

Agonist- fludrocortisone

35
Q

what are the main symptoms of Cushing’s?

A
proximal obesity
hirsutism
early menopause
hyperglycaemia
hypertensive.
36
Q

what tests would confirm a diagnosis of Cushing’s syndrome?

A

1) Urinary free cortisol
– over 24 hours and carried out twice.

2) Synthetic steroid administration (dexamethasone) – administered at night and then in the morning, cortisol levels measured
– cortisol should be LOW as you’ve administered a large dose of ACTH analogue (should negatively influence cortisol production

37
Q

what will a young person with multi drug resistant hypertension most likely be presenting with?

A

Conn’s

38
Q

what are there symptoms of phaechromocytoma?

A
  • sudden episodic headaches
  • double vision
  • malignant hypertension
  • chest pain
  • palpitations
  • sweating
39
Q

what are the investigations for phaec?

A
  • urinary catecholamines

- CT for adrenal tumour