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LCRS Endocrinology > Hyperadrenal disorders > Flashcards

Hyperadrenal disorders Flashcards

1
Q

What is the main problem in Cushing’s

A

Too much cortisol

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2
Q

What can too much cortisol caused by?

A

Tumour in pituitary that’s making ACTH which makes adrenal grow
Rarely, benign tumour of the adrenal gland

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3
Q

What does cortisol do?

A

Switches protein synthesis off and fat synthesis on

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4
Q

What is Cushings DISEASE caused by?

A

Tumour of the pituitary

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5
Q

What are the clinical features of Cushings?

A 
Too much cortisol
Centripetal obesity
Buffalo hump and moon face
Proximal myopathy
Hypertension and hypokalaemia
Red striae, thin skin and easy bruising
Osteoporosis
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6
Q

What are the causes of Cushing’s syndrome?

A

Taking too many oral steroids (cortisol like steroids- glucocorticoids)
Ectopic ACTH from lung cancer
Adrenal adenoma

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7
Q

What is the first thing you do if you see a patient presenting with all the symptoms of cushings?

A

Measure their cortisol

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8
Q

What is the problem with measuring cortisol?

A

It varies throughout the day- it is high in the morning and low in the evening (midnight it is very low)
Also pain and stress of blood test will increase cortisol

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9
Q

What would you see if someone has Cushing’s and you measure their cortisol at midnight?

A

It wouldn’t be low

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10
Q

What is another test to see if someone has Cushing’s?

A

Use the negative feedback system- if you gave a normal person dexamethasone your pituitary will detect extra steroid and it will turn off the ACTH and within hours you will have zero cortisol

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11
Q

What are the three tests you can do to identify the cause of Cushings?

A

24 hour urine collection for urinary free cortisol
Blood diurnal cortisol levels
Low dose dexamethasone suppression test (Gold standard)

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12
Q

What is the treatment of Cushing’s syndrome?

A

Depends on cause
Pituitary surgery (transphenoidal hypophysectomy)
Bilateral adrenalectomy- can’t make anymore cortisol
Unilateral adrenalectomy for adrenal mass

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13
Q

What is the main problem in Conn’s?

A

Too much aldosterone

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14
Q

Where are catecholamines produced?

A

Adrenal medulla

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15
Q

What is a tumour of the adrenal medulla that leads to lots of catecholamines called?

A

Phaeochromocytoma

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16
Q

What is the first step to block the effects of the excess catecholamines?

A

Give an alpha blocker

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17
Q

Why do you give an alpha blocker first when treating phaeochromocytoma?

A

The urgent issue is really high blood pressure caused by catecholamines binding to alpha receptors in vasculature leading to vasoconstriction

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18
Q

What do you give once the blood pressure has decreased when treating phaeochromocytoma?

A

Beta blocker

19
Q

What does metyrapone inhibit?

A

11beta-hydroxylase

20
Q

What does inhibiting 11beta-hydroxylase do?

A

Slows down the cortisol synthesis pathway by inhibiting the conversion of 11-deoxycorticosterone to corticosterone and conversion of 11-deoxycortisol to cortisol so it decreases levels of corticosterone and cortisol

21
Q

What happens due to the decrease in cortisol when using metyrapone?

A

The levels of ACTH rise

22
Q

When is metyrapone used for Cushing’s patients? (2)

A

Prior to surgery- Cushings patients are not good surgical candidates as predisposed to infection and do not heal easily (thin skin)
After radiotherapy- effects of radiotherapy are slow to be seen so metyrapone is used till then

23
Q

What are the two negative things about metyrapone?

A

Leads to accumulation of 11-deoxycorticosterone and 11-deoxycortisol- 11-deoxycorticosterone has mineralocorticoid properties, it leads to retention of sodium and excretion of potassium in the kidneys so you get salt retention and hypertension
Second problem- Because two limbs of pathway are blocked, all the precursors are funnelled towards sex steroid synthesis so there is an increase in adrenal androgens which can cause hirsuitism and is particularly unpleasant in women

24
Q

What is hirsuitisim ?

A

Excess body hair

25
Q

What are the unwanted aspects of metyrapone?

A 
Nausea, vomiting
Sedation, hypoadrenalism
Impaired performance of skilled tasks
Hypertension
Hirsutism
26
Q

What is the main use of ketoconazole?

A

Anti-fungal (withdrawn in 2013 due to high risk of hepatotoxicity)

27
Q

What else can ketoconazole be used for?

A

Inhibit cortisol production

28
Q

How does ketoconazole inhibit cortisol production?

A

It inhibits cytochrome p450 SCC enzymes- blocks the production of glucocorticoids, mineralocorticoids and androgens

29
Q

When is ketoconazole used?

A

In a similar way to metyrapone, before surgery

30
Q

What are unwanted actions of ketoconazole?

A 
Nausea, vomiting, abdominal pain
Alopecia
Gynaecomastia, oligospermia, impotence
Ventricular tachycardia
Liver damage
31
Q

What is the cause of Conn’s syndrome?

A

Benign adrenocortical tumour

32
Q

Where is aldosterone made?

A

Zona glomerulosa

33
Q

What does excess aldosterone in Conns cause?

A

Sodium retention and potassium loss- hypertension and hypokalaemia

34
Q

How is Conns treated?

A

To stop effects of excess aldosterone you give a mineralocorticoid receptor antagonist such as spironolactone
Surgery - image and remove tumour

35
Q

What are the side effects of spironolactone?

A

It is very non-specific so there are lots:
Menstrual irregularities (progesterone receptor antagonist)
Gynaecomastia in men (androgen receptor antagonist)
GI tract disease
Can cause renal/hepatic disease

36
Q

What is eplerenone?

A

Mineralocorticoid receptor antagonist

37
Q

Why eplerenone used more frequently than spironolactone?

A

Less side effects (more specific)

38
Q

What is the main effect of a phaeochromocytoma?

A

Large release of adrenaline will cause a large increase in blood pressure which could lead to a stroke. There is a sudden onset of pain, fever, tachycardia and hypertension

39
Q

When would you especially expect pheochromocytoma?

A

Episodic severe hypertension in young people

40
Q

How do you manage a phaeochromocytoma?

A

Eventually they need surgery but patient has to be very carefully prepared for surgery as anaesthetic can precipitate a hypertensive crisis

41
Q

How do you prepare a patient with phaeochromocytoma for surgery?

A

First you give a drug to block the receptor to which adrenaline binds- alpha blocker (patients may need IV fluid as this commences)
Once you’ve done this you give them a beta blocker to prevent tachycardia

42
Q

What percentage of phaeochromocytomas are inside the adrenals?

A

90%

43
Q

What percentage of phaeochromocytomas are malignant?

A

10%

44
Q

What percentage of phaeochromocytomas are curable by operation?

A

90%

LCRS Endocrinology (18 decks)

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