Endo 6 - Hyperadrenal disorders Flashcards

1
Q

Cushings syndrome is caused by too much?

A

Cortisol

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2
Q

What are 6 clinical features of Cushings?

A
  1. Centripetal obesity
  2. Moon face and buffalo hump
  3. Proximal myopathy
  4. Hypertension and hypokalaemia
  5. Red striae/thin skin / bruising
  6. Osteoporosis / diabetes
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3
Q

What are 4 causes of Cushings?

A
  1. Taking too many steroids (exogenous)
  2. Pituitary dependent Cushings
  3. Ectopic ACTH from lung cancer
  4. Adrenal adenoma secreting cortisol
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4
Q

Name 3 investigations to determine the cause of Cushings syndrome

A
  1. 24h urine collection measuring urinary free cortisol
  2. Blood diurnal cortisol levels
  3. Low dose dexamethasone suppression test
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5
Q

In someone with Cushings, what is the diurnal cortisol rhythm like?

A

It is high all the time

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6
Q

What are the results of giving low dose dexamethasone ?

A

If normal = cortisol suppressed to 0

Any cause of Cushings = failure to suppress cortisol to 0

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7
Q

What is Conns syndrome?

A

Excess aldosterone

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8
Q

What medication is given to treat patients with Cushings syndrome?

A

Steroid biosynthesis inhibitors -

Ketoconazole, metyrapone

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9
Q

Where in the adrenal cortex are aldosterone, cortisol and oestradiol produced?

A

Aldosterone - zona glomerulosa

Cortisol - zona fasciculata

Oestradiol - zona reticularis

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10
Q

How does metyrapone work?

A

It inhibits 11-B-hydroxylase

Which causes an accumulation of 11-deoxycortisol

Also causes increased ACTH secretion

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11
Q

What are the uses of metyrapone?

A
  1. Control Cushings syndrome before surgery

2. Control Cushings syndrome after radiotherapy

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12
Q

What are 2 side effects of metyrapone?

A
  1. Increased salt retention and hypertension due to 11-deoxycortisone (Arrested) accumulating in z.glomerulosa
  2. Increased adrenal androgen production - hirsutism in women
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13
Q

What is the mechanism of action of ketoconazole

A

Ketoconazole = anti fungal

Blocks production of glucocorticoids, mineralocorticoids and sex steroids

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14
Q

What are the uses of ketoconazole

A

Treatment and management of Cushings Syndrome prior to surgery

It is a tablet

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15
Q

What is the unwanted action of ketoconazole

A

Liver damage - could be fatal.

MUST CHECK LIVER FUNCTION REGULARLY

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16
Q

What surgical treatments are there for Cushings?

A
  1. Pituitary surgery
  2. Bilateral adrenalectomy
  3. Unilateral adrenalectomy
17
Q

Conns syndrome is due to XS aldosterone. What does this cause

A
  1. Hypertension and hypokalaemia
18
Q

If primary hyperaldosteronism (Conns), how is it diagnosed?

A

High aldosterone, low renin = Conns

RAS system suppression = exclude secondary hyperaldosteronism

19
Q

How is Conns syndrome treated?

A

Block Mineralocorticoid (MR) receptor - MR antagonist

e.g. Spironolactone, epleronone

20
Q

Spironolactone - mechanism of action

A

Converted into canrenone = competitive MR antagonist

Blocks Na resorption and K excretion in kidney tubules (K sparing diuretic) - combats effects of increased aldosterone

21
Q

What are the unwanted action of spironolactone?

A
  1. Menstrual irregularities (progesterone agonist)

2. Gynaecomastia (androgen receptor antagonist)

22
Q

Epleronone - mechanism of action

A
  1. MR antagonist

2. Less binding to progesterone/androgen receptors = less side effects and better tolerated

23
Q

What are phaeochromocytomas?

A

Adrenal medulla tumours - which secrete catecholamines

24
Q

What are the clinical features of a phaeo?

A
  1. Hypertension in young people
  2. Episodic severe hypertension attacks
  3. More common in certain inherited conditions
25
Q

What are the problems with phaeo?

A
  1. Severe hypertension - can cause MI or stroke

2. High Adrenaline - can cause VF / death

26
Q

How are phaeos managed?

A
  1. Alpha blockade
  2. Give IV as alpha blockade commences
  3. Beta-blocker to prevent tachycardia
  4. Surgery
27
Q

What is the 10% rule of phaeochromocytomas?

A
  1. 10% extra-adrenal
  2. 10% malignant
  3. 10% bilateral

Phaeos = very very rare