2: Cushing's syndrome Flashcards

1
Q

What is mnemonic to remember three layers of adrenal cortex

A

GFR

Remember substances produced get better from outside in: water, food, sex

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2
Q

What are 3 layers of the adrenal medulla

A

Zona Glomerulosa
Zona fasiculata
Zona reticularis

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3
Q

What is produced by the zone glomerulosa

A

Glucocorticoids

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4
Q

What is produced by the zone fasciulata

A

Mineralocorticoids

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5
Q

What is produced by the zone reticularis

A

Androgens (sex steroids)

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6
Q

What cells are contained in adrenal medulla

A

Chromaffin cells

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7
Q

Define cushings syndrome

A

Symptoms due to excess circulating glucocorticoid

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8
Q

Define cushings disease

A

Excess circulating glucocorticoid due to pituitary tumour

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9
Q

What is peak age of cushings disease

A

30-50 years-old

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10
Q

What is the commonest cause of Cushing syndrome

A

Exogenous steroids

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11
Q

How is the aetiology of Cushing’ syndrome divided

A

ACTH-dependent

ACTH-independent

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12
Q

What are the two ACTH dependent causes of cushings syndrome

A

Pituitary disease

Ectopic ACTH

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13
Q

How could cushings disease be identified on abdominal CT

A

Bilateral adrenal hyperplasia

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14
Q

What causes ectopic ACTH

A

ACTH released by cancer - such as small cell lung cancer

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15
Q

How will ACTH dependent causes present and why

A

Increased pigmentation due to conversion of surplus ACTH to melanin

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16
Q

What abnormality will be seen on ABG and why

A

Hypokalaemia metabolic alkalosis

  • Due to excess mineralocorticoid
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17
Q

What won’t be present in ectopic ACTH production

A

Typical cushing’s disease symptoms

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18
Q

What are 5 ACTH-independent causes of Cushing’s syndrome

A
  1. Carney Complex
  2. Exogenous steroids
  3. McCune Albright
  4. Adrenal adenoma
  5. Adrenal Nodular Hyperplasia
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19
Q

What is McCune Albright Syndrome

A

Non inheritable genetic condition caused by somatic mutation in GNAS

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20
Q

How will McCune-Alrbight syndrome present

A
  • Cafe au Lait Pigmentation
  • Precocious Puberty
  • Short stature
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21
Q

What are 5 symptoms of cushing’s disease

A
  • Weight Gain
  • Acne
  • Proximal muscle weakness
  • Change in mood
  • Gonadal dysfunction
  • Achilles tendon rupture
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22
Q

How may patients present with proximal muscle weakness

A

Difficultly getting out of a chair

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23
Q

what gonadal dysfunction happens in males

A

Decrease libido

ED

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24
Q

what gonadal function happens in females

A

Decrease libido

Virillisation

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25
Q

What are 5 signs of Cushing’s syndrome

A
Bufalo hump 
Central Obesity 
Osteoporosis 
Moon Face 
Muscle wasting 
Purple striae 
Hyperglycaemia 
HTN 
Infection
26
Q

explain investigation for cushing’s disease

A
  • Investigations are first to distinguish someone has cushings
  • THEN to localise the lesion
27
Q

what is first test for cushing’s disease

A

Random plasma cortisol

28
Q

how will random plasma cortisol present

A

High

29
Q

what is the main test for Cushing’s syndrome

A

Over-night dexamethasone suppression test

30
Q

explain overnight dexamethasone supression test

A

1mg dexamethasone is given at midnight and cortisol measured at 8am the next morning

31
Q

what is a normal result in overnight dexamethasone suppression test

A

< 50

32
Q

what does a cortisol >50 in overnight dexamethasone suppression test indicate

A

Cushing’s syndrome

33
Q

what is the overnight dexamethasone suppression test used for

A

Identify if someone has Cushing’s syndrome and distinguish this from pseudocushing’s

34
Q

what is pseudocushing’s

A

Cushing disease appearance due to: obesity. depression, chronic alcoholism, enzyme inducers

35
Q

what is an alternative test to the overnight dexamethasone suppression test

A

24h urinary cortisol

36
Q

what is third-line test

A

High-dose dexamethasone suppression test

37
Q

explain high-dose dexamethasone suppression test

A

2mg/6h dexmethaosne is given for 2 days and cortisol measures at 0 and 48h

38
Q

if cortisol is suppressed what does it indicate

A

cushing’s disease (pituitary adenoma)

39
Q

if cortisol is not suppressed what does it indicate

A

ectopic ACTH

40
Q

what is an alternative test

A

midnight cortisol

41
Q

how will midnight cortisol present and why

A

high - as diurnal rhythm is altered

42
Q

when is a localisation test performed

A

if overnight dexamethasone suppression test is positive (and it is cushing’s syndrome)

43
Q

what is the first-line localisation test

A

Plasma ACTH

44
Q

if plasma ACTH is undetectable what does it likely indicate

A

If plasma ACTH is undetectable it indicates an adrenal lesion

45
Q

if plasma ACTH is undetectable, what is next line investigation

A

next line is CT-MRI Adrenal Glands

46
Q

if no mass is seen on CT/MRI- adrenal glands what is performed

A

Adrenal vein sampling

47
Q

If ACTH is detectable what is done and why

A

High dose dexamethasone suppression test or CRH test to distinguish cushing’s disease from ectopic ACTH

48
Q

what is CRH test

A

Give 100mcg CRH and measure cortisol 2h later

49
Q

explain results of CRH test in cushing’s disease

A

CRH will cause an increase in cortisol in cushing’s disease (due to acting on pituitary)

50
Q

explain results of CRH test in ectopic ACTH production

A

No increase in cortisol

51
Q

if CRH test indicates pituitary lesion what should be done

A

Bilateral inferior petrosal sinus sampling

52
Q

if CRH test indicates ectopic ACTH production what is ordered

A

CT CAP

MRI Neck, Thorax, Abdomen

53
Q

How is exogenous cushing’s syndrome managed

A

Gradually reduce corticosteroids

54
Q

Why should exogenous corticosteroids not be suddenly stopped

A

This will precipitate addisonian crisis

55
Q

How is cushing’s disease managed

A

Transphenoidal removal pituitary adenoma

56
Q

If unable to find the source of excess glucocorticoid hormone, what is done

A

Bilateral adrenalectomy

57
Q

what is a complication of bilateral adrenalectomy

A

Nelson’s syndrome

58
Q

what is nelson’s syndrome and why does it occur

A

Increased pigmentation of the skin

Due to removal adrenal glands removes negative feedback and increases ACTH

59
Q

how are adrenal adenomas managed

A

Adrenalectomy

60
Q

how are adrenal carcinomas managed

A

Adrenalectomy and radiotherapy

61
Q

how is ectopic ACTH managed

A

Surgical removal tumour

Use medication to suppress cortisol in mean-time