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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
What are 5 signs of Cushing's syndrome
``` Bufalo hump Central Obesity Osteoporosis Moon Face Muscle wasting Purple striae Hyperglycaemia HTN Infection ```
26
explain investigation for cushing's disease
- Investigations are first to distinguish someone has cushings - THEN to localise the lesion
27
what is first test for cushing's disease
Random plasma cortisol
28
how will random plasma cortisol present
High
29
what is the main test for Cushing's syndrome
Over-night dexamethasone suppression test
30
explain overnight dexamethasone supression test
1mg dexamethasone is given at midnight and cortisol measured at 8am the next morning
31
what is a normal result in overnight dexamethasone suppression test
< 50
32
what does a cortisol >50 in overnight dexamethasone suppression test indicate
Cushing's syndrome
33
what is the overnight dexamethasone suppression test used for
Identify if someone has Cushing's syndrome and distinguish this from pseudocushing's
34
what is pseudocushing's
Cushing disease appearance due to: obesity. depression, chronic alcoholism, enzyme inducers
35
what is an alternative test to the overnight dexamethasone suppression test
24h urinary cortisol
36
what is third-line test
High-dose dexamethasone suppression test
37
explain high-dose dexamethasone suppression test
2mg/6h dexmethaosne is given for 2 days and cortisol measures at 0 and 48h
38
if cortisol is suppressed what does it indicate
cushing's disease (pituitary adenoma)
39
if cortisol is not suppressed what does it indicate
ectopic ACTH
40
what is an alternative test
midnight cortisol
41
how will midnight cortisol present and why
high - as diurnal rhythm is altered
42
when is a localisation test performed
if overnight dexamethasone suppression test is positive (and it is cushing's syndrome)
43
what is the first-line localisation test
Plasma ACTH
44
if plasma ACTH is undetectable what does it likely indicate
If plasma ACTH is undetectable it indicates an adrenal lesion
45
if plasma ACTH is undetectable, what is next line investigation
next line is CT-MRI Adrenal Glands
46
if no mass is seen on CT/MRI- adrenal glands what is performed
Adrenal vein sampling
47
If ACTH is detectable what is done and why
High dose dexamethasone suppression test or CRH test to distinguish cushing's disease from ectopic ACTH
48
what is CRH test
Give 100mcg CRH and measure cortisol 2h later
49
explain results of CRH test in cushing's disease
CRH will cause an increase in cortisol in cushing's disease (due to acting on pituitary)
50
explain results of CRH test in ectopic ACTH production
No increase in cortisol
51
if CRH test indicates pituitary lesion what should be done
Bilateral inferior petrosal sinus sampling
52
if CRH test indicates ectopic ACTH production what is ordered
CT CAP | MRI Neck, Thorax, Abdomen
53
How is exogenous cushing's syndrome managed
Gradually reduce corticosteroids
54
Why should exogenous corticosteroids not be suddenly stopped
This will precipitate addisonian crisis
55
How is cushing's disease managed
Transphenoidal removal pituitary adenoma
56
If unable to find the source of excess glucocorticoid hormone, what is done
Bilateral adrenalectomy
57
what is a complication of bilateral adrenalectomy
Nelson's syndrome
58
what is nelson's syndrome and why does it occur
Increased pigmentation of the skin | Due to removal adrenal glands removes negative feedback and increases ACTH
59
how are adrenal adenomas managed
Adrenalectomy
60
how are adrenal carcinomas managed
Adrenalectomy and radiotherapy
61
how is ectopic ACTH managed
Surgical removal tumour | Use medication to suppress cortisol in mean-time

Endocrine (20 decks)

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